ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Neutrophilic dermatoses

Neutrophilic dermatoses
Literature review current through: Aug 2023.
This topic last updated: Aug 11, 2021.

INTRODUCTION — The neutrophilic dermatoses are a group of disorders characterized by skin lesions for which histologic examination reveals intense epidermal, dermal, or hypodermal infiltrates composed primarily of neutrophils with no evidence of infection or true vasculitis [1]. Classification of the neutrophilic dermatoses is based upon the recognition of clinical and pathologic features, as well as the identification of associated diseases [1,2].

Cutaneous findings in neutrophilic dermatoses are variable, and can include vesiculopustules, plaques, nodules, or ulcerations (table 1) [1,3,4]. Depending on the disorder, lesions may be localized or widespread. Extracutaneous involvement may be present in some cases [5].

The pathogenic mechanisms of the various neutrophilic dermatoses are not well understood. The occurrence of neutrophilic dermatoses in autoinflammatory diseases and observed similarities in the clinical and histologic features, cytokine profiles, and therapeutic approaches between neutrophilic dermatoses and autoinflammatory diseases have led some authors to propose that neutrophilic dermatoses should be considered autoinflammatory diseases [6]. (See "The autoinflammatory diseases: An overview".)

SWEET SYNDROME — The prototype of the neutrophilic dermatoses is Sweet syndrome (acute febrile neutrophilic dermatosis). There are four primary features of Sweet syndrome: a cutaneous eruption consisting of erythematous papules and plaques, a dermal nonvasculitic neutrophilic infiltration on biopsy, fever, and peripheral neutrophilia [7,8]. Rare clinical variants include cellulitis-like, subcutaneous, and necrotizing fasciitis-like presentations [9-11].

The cutaneous eruption consists of erythematous to violaceous tender papules which enlarge to form plaques with irregular pseudovesicular surfaces (picture 1A-D). True pustulation and blistering also can occur. The plaques are usually a few centimeters in diameter, and may have central yellowish discoloration, creating a target-like appearance. The plaques may cause pain and a burning sensation, but are not pruritic.

The skin eruption is often accompanied by constitutional signs of fever, malaise, and arthralgia, as well as leukocytosis and an elevated erythrocyte sedimentation rate. Although the skin lesions can occur in any site, they are most often found on the face, neck, and upper extremities, especially the dorsum of hands. A variety of other systemic manifestations can also occur, depending on the organs involved (eg, eyes, oropharynx, lung, heart, musculoskeletal and nervous systems, spleen) [7,8,11-14]. Examples of associated diseases and conditions are malignancies, infections, drugs, autoimmune diseases, inflammatory bowel disease, pregnancy, and pathergy.

Sweet syndrome is reviewed in greater detail separately. (See "Sweet syndrome (acute febrile neutrophilic dermatosis): Pathogenesis, clinical manifestations, and diagnosis" and "Sweet syndrome (acute febrile neutrophilic dermatosis): Management and prognosis".)

PYODERMA GANGRENOSUM — Pyoderma gangrenosum is a painful ulcerative neutrophilic disease of the skin of unknown origin. There are multiple clinical variants; examples include classic, bullous, pustular, vegetative, peristomal, and postoperative PG. The earliest clinical lesion is a pustule with an inflammatory base, an erythematous nodule, or a hemorrhagic bulla on a violaceous base (picture 2). These lesions evolve to form shallow or painful deep ulcers, and can expose underlying tendons or muscle. The ulcers have a purulent base and a ragged, undermined, violaceous gunmetal-colored border that spreads peripherally (picture 3A-B). If there are several ulcers, they may coalesce to form larger lesions that heal with atrophic, pigmented cribriform scarring (picture 4). PG is discussed in greater detail separately. (See "Pyoderma gangrenosum: Pathogenesis, clinical features, and diagnosis" and "Pyoderma gangrenosum: Treatment and prognosis".)

NECROTIZING NEUTROPHILIC DERMATOSES — The term necrotizing neutrophilic dermatosis refers to presentations of Sweet syndrome or pyoderma gangrenosum that resemble necrotizing fasciitis. Patients have ulcers or erythematous plaques, often with erythematous-violaceous borders and associated purpura, purulence, pustules, or edema (picture 5A-B). Features suggestive of severe infection accompany these findings, such as fever, shock, and laboratory evidence of leukemoid reactions or leukocytosis [15].

Misdiagnosis of necrotizing neutrophilic dermatosis as necrotizing fasciitis is common, leading to delayed or ineffective treatment. Tissue biopsies and cultures are critical for differentiating necrotizing neutrophilic dermatosis and necrotizing fasciitis. Patients in whom necrotizing neutrophilic dermatosis is suspected may benefit from early introduction of immunosuppression, particularly when clear evidence of infection is absent and improvement has not occurred with antimicrobial therapy [15].

GENERALIZED PUSTULAR PSORIASIS — Generalized pustular psoriasis (GPP) is a rare acute, sterile, pustular dermatosis, occurring in patients with overt plaque or latent psoriasis [16-19]. Erythroderma may occur and the condition can be life threatening. GPP is accompanied by constitutional signs and symptoms, including fever, chills, malaise, myalgias, and arthralgias. The disorder may occur in any age group, including children [18,20,21]. Generalized pustular psoriasis may also occur in association with pregnancy; the term impetigo herpetiformis has been used to describe this condition. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis" and "Dermatoses of pregnancy", section on 'Pustular psoriasis of pregnancy'.)

Most patients present with large areas of tender erythema affecting the flexural, crural, and acral sites in which multiple, discrete, small pustules appear in groups and coalesce to form lakes of pus (picture 6A-F). Inherent complicating problems are infection, volume depletion, and a capillary leak syndrome possibly triggered by interleukin 1 and tumor necrosis factor from keratinocytes [22,23]. Systemic involvement can occur, with manifestations such as arthritis and kidney, liver, and heart failure.

A number of precipitating factors have been identified [17-19]. Examples include pregnancy, infections, hypocalcemia, medications such as salicylates, iodides, gold therapy, lithium, hydroxychloroquine, biologic tumor necrosis factor inhibitors [24], and reduction or withdrawal of systemic or local potent glucocorticoid therapy.

The differential diagnosis of GPP includes acute generalized eruptive pustulosis (AGEP) (table 2) [25]. Like GPP, AGEP can be precipitated by drugs (eg, beta-lactam and macrolide antibiotics, hydroxychloroquine, sulfonamides, terbinafine, and diltiazem) and enteroviral infections. Clinical features of AGEP include high fever, an erythematous eruption with nonfollicular pustulosis (picture 7A-B). The onset is typically rapid and skin lesions heal with supportive therapy in two to three weeks. (See "Acute generalized exanthematous pustulosis (AGEP)".)

Data on the treatment of GPP are limited, consisting primarily of individual case reports and retrospective studies. Treatment options for GPP include systemic therapies, topical therapies (eg, baths, emollients, adjunctive topical medications), and photochemotherapy. (See "Pustular psoriasis: Management".)

DEFICIENCY OF THE INTERLEUKIN 1 RECEPTOR ANTAGONIST — A genetic autoinflammatory syndrome characterized by the neonatal onset of sterile multifocal osteomyelitis, periostitis, and cutaneous pustulosis as well as the absence of evidence of infection, high-titer autoantibodies, and autoreactive T cells has been described in several patients [26-28]. The cutaneous eruption manifests as discrete crops of pustules, generalized pustules, or ichthyosiform lesions and may be accompanied by nail changes. Histopathologic examination of the skin lesions in affected patients demonstrates extensive epidermal and dermal neutrophilic infiltrates. Deficiency of the interleukin 1 receptor antagonist is discussed in greater detail separately. (See "Cryopyrin-associated periodic syndromes and related disorders", section on 'Deficiency of the IL-1-receptor antagonist (DIRA)'.)

REACTIVE ARTHRITIS — The triad of arthritis, conjunctivitis, and urethritis occurring after urethral or enteric infection is referred to as reactive arthritis. Genital lesions are also common. Twenty-three to fifty percent of patients may have balanitis. Isolated balanitis may be a forme fruste or may precede the development of other features of reactive arthritis [29]. (See "Reactive arthritis".)

Balanitis most often occurs at the coronal margin of the prepuce and the nearby glans, especially at the perimeatal area. The eruption is characterized by erythema that develops pinpoint erosive papules and pustules. The lesions can coalesce and have jagged scalloped borders, especially about the meatus, and creates the so-called "balanitis circinata" or circinate balanitis (picture 8). These lesions are painless in uncircumcised patients and retraction of the foreskin is necessary to detect the lesions. In circumcised patients, the lesions may harden to a crust and can then cause scarring or pain (picture 9). The differential diagnosis of balanitis is erythema multiforme, fixed drug eruption, psoriasis, herpes simplex, candidiasis, and balanitis plasmacellularis (Zoon's disease). Ulcerative vulvitis can occur, but it is less common than balanitis [30]. The arthritis is an autoimmune arthropathy that occurs in response to a urogenital or enteric infection.

A variety of mucocutaneous expressions of the disease may be seen:

The "keratoderma blenorrhagicum" cutaneous eruption of reactive arthritis, which occurs in 10 to 30 percent of cases, is usually found on the soles and palms, but can be seen in other parts of the body. Keratoderma blenorrhagicum can be clinically indistinguishable from pustular psoriasis (picture 10A-B). The cutaneous manifestations begin as clear vesicles on erythematous bases and progress to pustular keratotic lesions that coalesce to form plaques. There is subungual abscess formation with accumulation of debris and onycholysis (picture 11).

A self-limited stomatitis characterized by superficial ulcerations or erythematous grayish plaques involving the buccal mucosa, palate, and tongue (picture 12).

Ostraceous psoriasiform plaques may appear on the body.

Treatment of the cutaneous manifestations with topical glucocorticoids, keratolytics, or systemic agents (methotrexate [2.5 to 20 mg per week], mycophenolate mofetil [1 g twice daily], or acitretin [25 mg/day] with psoralen plus UVA [RePUVA] or without psoralen plus UVA) may be helpful. Infliximab therapy was associated with improvement in the cutaneous and nail manifestations of reactive arthritis in a patient with reactive arthritis and HIV infection [31]. Other anti-tumor necrosis factor-alpha therapies that may be effective are etanercept (25 mg twice weekly), adalimumab (40 mg every other week), and thalidomide (100 mg/day). Therapy of the systemic manifestations is discussed separately. (See "Reactive arthritis".)

SAPHO SYNDROME — Recognition of patients with clinical features that include synovitis, acne, pustulosis, hyperostosis, and osteomyelitis led to the acronym SAPHO [32]. The SAPHO syndrome consists of a wide spectrum of aseptic neutrophilic dermatoses associated with aseptic osteoarticular lesions [33-35]. The disorder usually affects children and young or middle-aged adults. Cutaneous disorders associated with SAPHO include palmar and plantar pustulosis (picture 13A-C), follicular occlusion tetrad (acne conglobata, hidradenitis suppurativa, dissecting cellulitis of the scalp, pilonidal sinus), acne fulminans, pustular psoriasis, Behçet syndrome, Sweet syndrome, Sneddon-Wilkinson disease, pyoderma gangrenosum, linear IgA bullous dermatosis, and Lyme disease [36]. (See "Major causes of musculoskeletal chest pain in adults", section on 'Sternocostoclavicular hyperostosis (SAPHO syndrome)'.)

PALMOPLANTAR PUSTULOSIS — Palmoplantar pustulosis presents as a sterile vesiculopustulosis in shiny, erythematous, scaling areas of the palms and soles (picture 14A-C). As the lesions "dry up," they develop dry brown hemorrhagic scales. The eruption is usually chronic and symmetric and primarily affects women, especially smokers, between the ages of 40 and 60 years of age. Associated nail dystrophy may be present [37]. (See "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis", section on 'Clinical manifestations'.)

The disorder may develop or exacerbate following infections such as tonsillitis, odontogenic infection, and sinus infections [38]. Palmoplantar pustulosis tends to have a prolonged course consisting of periods of exacerbations and remissions. The disease eventually clears spontaneously. A possible association between this disorder and the use of tumor necrosis factor-alpha inhibitors has been noted. (See "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis".)

The exact nosologic position of palmoplantar pustulosis is uncertain. It is associated with psoriasis in some patients and rarely with the SAPHO syndrome [39-41]. (See "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis", section on 'Classification' and "SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome".)

Palmoplantar pustulosis is reviewed in detail separately. (See "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis" and "Palmoplantar pustulosis: Treatment".)

SUBCORNEAL PUSTULAR DERMATOSIS — Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) was first described in 1956 [42]. This rare disorder has been observed in patients with rheumatoid arthritis, inflammatory bowel disease, and solid tumors, most often affecting women between the ages of 40 and 60. (See "Subcorneal pustular dermatosis".)

The eruption is characterized by flaccid pustules with visible fluid levels grouped in a circinate pattern on normal or erythematous skin in the inguinal and axillary folds, on the trunk, and the proximal parts of extremities (picture 15A-C). Patients do not usually have mucosal involvement. Histopathology reveals a subcorneal accumulation of neutrophils and the absence of acantholysis (picture 16).

There are usually no constitutional signs or symptoms and the eruption is asymptomatic. A variety of disease associations have been described, such as a monoclonal immunoglobulin A (IgA) and immunoglobulin M (IgM) gammopathies, myelomas, cryoglobulinemia, Raynaud phenomenon [43], pyoderma gangrenosum followed by subcorneal pustular dermatosis [44], amicrobial lymph node suppuration, and aseptic spleen abscesses [45], in addition to infections and certain medications.

Subcorneal pustular dermatosis does not scar, but transient postinflammatory hyperpigmentation may occur. The disease is relatively benign but often has a chronic relapsing course. The clinical differential diagnosis is impetigo, pustular psoriasis, pemphigus foliaceus, linear IgA bullous dermatosis, exanthematous pustulosis, necrolytic migratory erythema, dermatophytosis, and amicrobial pustulosis of the folds.

The subcorneal pustular dermatosis type of IgA pemphigus is clinically and histologically indistinguishable from classic subcorneal pustular dermatosis [42]. IgA pemphigus can be differentiated from classic subcorneal pustular dermatosis through immunofluorescence studies. Specimens from patients with IgA pemphigus demonstrate the presence of IgA autoantibodies binding to desmosomal components of the epidermis (desmogleins or desmocollins). (See "Pathogenesis, clinical manifestations, and diagnosis of pemphigus".)

Subcorneal pustular dermatosis is reviewed in detail separately. (See "Subcorneal pustular dermatosis".)

AMICROBIAL PUSTULOSIS OF THE FOLDS — Amicrobial pustulosis of the (cutaneous) folds (APF) is a neutrophilic dermatosis that can resemble subcorneal pustular dermatosis and variants of pustular psoriasis. APF is rare and has a predilection for females, with a mean age of onset of 30 years [46]. It is associated with a spectrum of autoimmune diseases, especially systemic lupus erythematosus. The disease is characterized by a relapsing primary follicular and nonfollicular pustular dermatosis, which favors the cutaneous folds of the skin, the scalp, and the periorificial areas of the head, the mouth, nostrils, and the external ear canals. Histologically, there is a superficial spongiform pustulation in the epidermis with primarily periadnexal and perivascular neutrophilic infiltration of the dermis and no evidence of infection or vasculitis. Systemic glucocorticoids appear to be the most effective treatment, and improvement with dapsone, colchicine, cyclosporine, hydroxychloroquine, or methotrexate as monotherapy or adjunctive treatment has been reported in individual patients [46,47].

BOWEL-ASSOCIATED DERMATOSIS-ARTHRITIS SYNDROME — During the 1960s and 1970s, jejunoileal bypass surgery was performed for obesity. Approximately 20 percent of these patients developed an influenza-like syndrome with an episodic and recurrent cutaneous eruption [48]. A similar syndrome has been described in patients with inflammatory bowel disease, a Billroth II procedure for peptic ulcer disease, biliopancreatic diversion, or blind loops of bowel following surgery [49]. (See "Clinical manifestations and diagnosis of arthritis associated with inflammatory bowel disease and other gastrointestinal diseases" and "Bariatric procedures for the management of severe obesity: Descriptions", section on 'Jejunoileal bypass'.)

These patients have now been given the diagnosis of bowel-associated dermatitis-arthritis syndrome. Affected patients present with a serum sickness-like syndrome characterized by fever, chills, malaise, arthralgia, arthritis, and myalgia [50,51], which frequently precede the skin eruption. The aseptic nonerosive polyarthritis is asymmetric, episodic, affects both small and large joints, and may be associated with tenosynovitis. Diarrhea and malabsorption are additional features.

The most characteristic cutaneous skin lesions are erythematous macules up to 1 cm in diameter that develop a central papulovesicle or pustule (picture 17A-C). The eruption most often occurs on the upper extremities and trunk but can occur anywhere, except the face and genitalia. Lesions resembling erythema nodosum may occur, and a liquefying panniculitis has been described.

The pathogenesis is uncertain. Bowel bacterial overgrowth with subsequent immune complex formation within the circulation may be the initial event, followed by deposition of these complexes in tissue target sites and activation of the alternate pathway of complement.

Treatment options are reanastomosis in patients with bypass surgery, prednisone (10 to 60 mg/day), dapsone (100 mg/day), and antibiotics. The antibiotics that may be effective include tetracycline (1 to 2 g/day), minocycline (100 to 400 mg/day), clindamycin (600 mg/day), trimethoprim-sulfamethoxazole (160/800 mg/day), erythromycin (500 to 2000 mg/day), and metronidazole (500 to 1000 mg/day) [48,52,53]. Immunosuppressants, including cyclosporine and mycophenolate mofetil, and treatment of the underlying inflammatory bowel disease have also appeared effective in individual patients [54-57].

BEHÇET SYNDROME — Behçet syndrome is a persistent, relapsing multisystem disease with prominent mucocutaneous findings (picture 18A-B) [58]. It is characterized by recurrent oral ulcerations in virtually every patient plus, in many patients, recurrent genital lesions, eye lesions (such as posterior uveitis and retinal vasculitis), skin lesions, and pathergy (table 3) [59]. There may be vascular, neurologic, musculoskeletal, cardiac, renal, and gastrointestinal manifestations. The prevalence of Behçet syndrome is higher in the Middle East and Asia than in the Western countries. (See "Clinical manifestations and diagnosis of Behçet syndrome".)

The cutaneous manifestations of Behçet syndrome have been divided into two categories: a nonspecific group (folliculitis, acne, and dermatographism) and the so-called sterile neutrophilic group (vesiculopustular lesions, pustules, hemorrhagic pustular [vasculitic] lesions, plaques, and erythema nodosum-like nodules) [60]. The papules and plaques are edematous erythematous to violaceous lesions similar to those seen in Sweet syndrome. The papules can be purpuric and become pustular.

The erythematous nodular lesions, which resemble erythema nodosum, involve the legs, buttocks, arms, and neck. These lesions differ from erythema nodosum by their shorter duration (one to three weeks), less induration, and a different clinical distribution and histopathologic picture. However, erythema nodosum and cutaneous vasculitis have been seen in Behçet syndrome.

Pathergy refers to an erythematous papular or pustular response to local skin injury. It is defined as a greater than 5 mm lesion that appears 24 to 48 hours after skin prick by a needle. Pathergy is less common in North American and North European patients with Behçet syndrome (10 to 20 percent), than in Eastern patients (50 to 75 percent).

The characteristic histopathologic finding in early lesions is a neutrophilic angiocentric infiltrate with leukocytoclastic vasculitis. Late lesions demonstrate a lymphocytic infiltrate. A variety of medications have been used for the manifestations of Behçet syndrome. The treatment of Behçet syndrome is reviewed separately. (See "Treatment of Behçet syndrome".)

ERYSIPELAS-LIKE ERYTHEMA OF FAMILIAL MEDITERRANEAN FEVER — Familial Mediterranean fever (FMF) is characterized by recurrent fever; polyserositis involving the peritoneum, pleura, and synovium; and a pathognomonic cutaneous finding, erysipelas-like erythema (ELE). ELE, which presents as tender, erythematous patches or plaques, typically involves the lower extremities (picture 19A-B). The condition tends to be unilateral, but can be bilateral and symmetrical and tends to recur in the same sites.

Histologically, there is predominately a neutrophilic infiltrate and nuclear debris in the reticular dermis without evidence of vasculitis. During acute FMF attacks, neutrophils are seen in serosal fluid. Colchicine remains the mainstay of the treatment of FMF [61,62]. Children with ELE may have more severe disease activity requiring higher doses of colchicine [63]. However, a small percentage of patients may require additional therapy with interleukin 1 inhibition. (See "Clinical manifestations and diagnosis of familial Mediterranean fever" and "Management of familial Mediterranean fever".)

NEUTROPHILIC ECCRINE HIDRADENITIS — Neutrophilic eccrine hidradenitis is a reactive disorder that may occur in association with malignancy (with or without chemotherapy), infections, and/or certain medications [64,65]. The clinical lesions are typically erythematous edematous plaques that may be purpuric and painful (picture 20A-B). They are located on the extremities, trunk, face, and palms, and may mimic cellulitis when present near the eyes. Fever and neutropenia can be concurrent features.

Histopathology reveals neutrophils surrounding and infiltrating the eccrine gland with occasional intraductal abscess formation and necrosis of secretory cells. Syringosquamous metaplasia of sweat glands and fibrosis of adjacent dermis occurs.

Associated malignancies include myelogenous leukemia, other leukemias, Hodgkin lymphoma, and solid tumors. Sweat gland involvement may occur in the absence of exposure to chemotherapy, but the use of certain chemotherapy agents has been temporally associated with development of skin lesions (table 4) (see "Cutaneous adverse effects of conventional chemotherapy agents", section on 'Neutrophilic eccrine hidradenitis'). Among the infectious agents reported in association with this skin disorder are Serratia marcescens, Enterobacter cloacae, Staphylococcus aureus, Mycobacterium chelonae, Nocardia species, and HIV. In addition to the chemotherapeutic agents already mentioned, other possibly associated drugs are acetaminophen (paracetamol), zidovudine, stavudine, granulocyte colony-stimulating factor, cetuximab, minocycline, and BRAF inhibitors (dabrafenib, vemurafenib) [66]. Tissue culture is useful for distinguishing infectious and noninfectious neutrophilic eccrine hidradenitis [67].

The lesions usually spontaneously resolve within a month but can recur. Neutrophilic eccrine hidradenitis is reviewed in detail separately. (See "Cutaneous adverse effects of conventional chemotherapy agents", section on 'Neutrophilic eccrine hidradenitis'.)

NEUTROPHILIC DERMATOSIS OF THE DORSAL HANDS — Neutrophilic dermatosis of the dorsal hands is considered by some authors to be a variant of Sweet syndrome, but the presence of leukocytoclastic vasculitis in some otherwise similar cases has led others to classify it as a "pustular vasculitis" [68]. Whether this disorder is best classified with the neutrophilic dermatoses or the cutaneous vasculitides is uncertain, but the preponderance of available evidence suggests the dermal infiltration by neutrophils is the predominant pathogenic mechanism and that vasculitis may represent an epiphenomenon [69].

The clinical hallmarks are painful nodules and plaques located on the dorsal aspect of the hands; these may proceed to form pustules and/or ulcerate (picture 21) [68,70-72]. While the initial presentation is suggestive of dermal infection, the lesions are not responsive to antibiotic treatment, and microbiologic cultures and histologic examination with stains for bacteria, mycobacteria, and fungi have not supported an infectious etiology in reported cases. Women may be more often affected than men.

The initial report of this entity noted leukocytoclastic vasculitis in affected skin [68], but a subsequent description of patients with similar physical findings had only neutrophilic dermal infiltrates and pustules upon microscopic examination [70]. In a series of 17 patients with this disorder, 4 had evidence of vasculitis [73].

Treatment with oral glucocorticoids and with dapsone have each been reported to be effective in small uncontrolled series [70,72,73]. Minocycline did not appear to provide long-term control in two patients in whom its use was associated with initial improvement [72].

NEUTROPHILIC DERMATOSES ASSOCIATED WITH RHEUMATOID ARTHRITIS — Sweet syndrome, pyoderma gangrenosum, rheumatoid neutrophilic dermatitis, and palisaded neutrophilic granulomatous dermatitis may develop in patients with rheumatoid arthritis. Other types of skin lesions that are not classified among the neutrophilic dermatoses may also be associated with rheumatoid arthritis (RA). (See "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Skin disease'.)

Rheumatoid neutrophilic dermatitis — Rheumatoid neutrophilic dermatitis (RND) was first described in 1978 and is a rare skin manifestation most frequently described in middle-aged women with severe seropositive RA. It has also been reported in seronegative RA [74,75].

The eruption typically consists of erythematous, urticarial-like papules, plaques, nodules, bullae, or annular lesions less than 2 cm in diameter (picture 22) [76-78], which sometimes develop in clusters [74]. The lesions are usually symmetrical and favor the extremities, neck, and trunk; they are usually not pruritic and may be tender.

Histopathologically, there is a dense dermal neutrophilic infiltrate without vasculitis. Leukocytoclasis may occur, and the infiltrate can extend in the panniculus. The dermal papilla may have microabscesses [79].

Unlike Sweet syndrome, the lesions of RND are usually asymptomatic, tend to resolve spontaneously or with improvement of the rheumatoid arthritis after one to three weeks, lack the constitutional signs and symptoms of fever and malaise, and ocular disease [80]. Therapy consists of topical or systemic glucocorticoids, dapsone (100 to 200 mg/day), colchicine (0.6 mg twice daily) [81] and antimalarials, such as hydroxychloroquine (200 mg twice daily) [80].

Palisaded neutrophilic granulomatous dermatitis and interstitial granulomatous dermatitis — Granulomatous dermatitis in the context of autoimmune disease presents in two major clinical and histopathologic patterns: palisaded neutrophilic granulomatous dermatitis (PNGD) and interstitial granulomatous dermatitis (IGD). Some clinicians categorize these disorders within the same clinicopathologic spectrum [82,83], proposing the broad term "reactive granulomatous dermatitis" [84], while others have maintained that these conditions are separate entities [85]. Additional names that have been used to describe PNGD in the literature include rheumatoid papules, Churg-Strauss granuloma, cutaneous extravascular necrotizing granuloma, and superficial ulcerating rheumatoid necrobiosis.

PNGD may present as symmetrically distributed umbilicated papules that favor the extensor surfaces of the extremities, particularly the elbows and fingers. Other morphologies include nodules and indurated plaques with clinical overlap with IGD and can occur on the face and trunk [83,84,86]. The most frequent disease association is rheumatoid arthritis, but PNGD may also appear in systemic lupus erythematosus, systemic vasculitides, other immune-mediated disorders, lymphoproliferative disease, sarcoidosis, and with certain medications [83,84,87]. Rare cases have been reported without an identifiable underlying systemic disease [88,89]. It is hypothesized that immune complex deposition in blood vessels may be the triggering factor for PNGD [90]. The histopathologic findings of early lesions include a small-vessel leukocytoclastic vasculitis and a dense neutrophilic infiltrate [90]. Mature lesions exhibit palisaded granulomas and dermal fibrosis. (See "Palisaded neutrophilic and granulomatous dermatitis".)

The term IGD describes a form of granulomatous dermatitis in which annular plaques or "rope-like" cords favor the lateral trunk and skin folds in patients with rheumatoid arthritis (picture 23) [91,92]. Coexistence of both the crusted papules, described more frequently in PNGD, and annular plaques of IGD have been described within the same patient, supporting the notion of a clinicopathologic spectrum that incorporates the two conditions [82,84,86]. IGD has been associated with a variety of factors, including autoimmune disease, malignancy, infections, and drugs [83,84,93]. The histopathologic findings include foci of degenerated collagen with palisading granulomas composed of histiocytes. This is accompanied by a dermal interstitial lymphohistiocytic infiltrate with varying numbers of neutrophils and eosinophils [36,85]. Interstitial mucin may or may not be present [94]. Vasculitis is absent, described as a distinguishing factor from PNGD.

The clinical differential diagnosis of papular PNGD includes papular granuloma annulare, rheumatoid nodules, and perforating disorders [36]. In cases with clinical features of IGD, patch type of granuloma annulare, inflammatory morphea, erythema migrans, and mycosis fungoides should be considered. (See "Granuloma annulare: Epidemiology, clinical manifestations, and diagnosis", section on 'Clinical features'.)

Treatment of the underlying disease may cause remission and prevent recurrence of PNGD [87,95]. The management of PNGD is discussed separately. (See "Palisaded neutrophilic and granulomatous dermatitis", section on 'Management'.)

Most treatments in the literature for IGD are also aimed at treating the underlying systemic disease. High-potency topical corticosteroids, intralesional corticosteroids, dapsone, and hydroxychloroquine have also been utilized for management [96-99]. Although PNGD and IGD have occurred after the initiation of anti-tumor necrosis factor (TNF) therapy [94,100], IGD also has improved with anti-TNF drugs [101,102]. In addition, there are case reports describing improvement in IGD with ustekinumab (interleukin 12/23 inhibition) [103,104].

NEUTROPHILIC URTICARIAL DERMATOSIS — Neutrophilic urticarial dermatosis is a condition that has been described primarily in patients with cryopyrin-associated periodic syndromes, Schnitzler syndrome, adult-onset Still's disease, and systemic lupus erythematosus [105-107]. (See "Cryopyrin-associated periodic syndromes and related disorders" and "Clinical manifestations and diagnosis of adult-onset Still's disease" and "Clinical manifestations and diagnosis of systemic lupus erythematosus in adults", section on 'Clinical manifestations'.)

Affected patients develop erythematous macules or thin plaques on the skin that resolve within 24 hours (picture 24). Trunk and/or extremity involvement is most common.

Histopathologic examination of lesional tissue reveals an intense neutrophilic infiltrate in the dermis with leukocytoclasia but without significant edema or fibrinoid necrosis of vessel walls seen in vasculitis. Isolated necrobiotic collagen bundles may be present. Treatment with dapsone, colchicine, or interleukin 1 blockade with anakinra has been associated with improvement in some patients [105,106,108]. Additional treatment options for disorders associated with the development of neutrophilic urticarial dermatosis (eg, cryopyrin-associated periodic syndromes, Schnitzler syndrome, Still's disease) are reviewed separately. (See "Cryopyrin-associated periodic syndromes and related disorders", section on 'Treatment of cryopyrinopathies' and "Urticarial vasculitis", section on 'Differential diagnosis' and "Treatment of adult-onset Still's disease".)

PALMOPLANTAR ECCRINE HIDRADENITIS — Palmoplantar eccrine hidradenitis is an uncommon condition that presents as an abrupt onset of erythematous, tender papules and nodules on soles of the feet and less commonly on the palms (picture 25A-B). The condition primarily occurs in healthy children and typically lasts between one and four weeks [109]. The associated pain can be significant and can impair ambulation. The etiology is uncertain; a relationship to strenuous physical activity and hyperhidrosis is speculated, potentially resulting in eccrine duct obstruction and rupture leading to inflammation [110].

The diagnosis is made based upon clinical appearance and history; a biopsy is not usually necessary. If performed, a biopsy reveals a neutrophilic peri-eccrine infiltrate. A superficial and deep perivascular infiltrate containing neutrophils, lymphocytes, and histiocytes, as well as septal panniculitis may be present [109]. Treatment consists of avoidance of physical activity and bed rest [110].

NEUTROPHILIC DERMATOSES ASSOCIATED WITH ERYTHEMA NODOSUM LEPROSUM — Erythema nodosum leprosum is an acute, inflammatory, immune complex-mediated reaction affecting skin, nerves, and other organs that occurs in patients with lepromatous or borderline lepromatous leprosy. The histologic finding of an inflammatory infiltrate containing neutrophils, lymphocytes, and macrophages with or without vasculitis is characteristic. The clinical manifestations vary based upon the histopathologic location of the inflammatory infiltrate and can vary from pustular or vesicular eruptions (epidermal involvement) to plaques (dermal involvement) or erythema nodosum-like nodules (subcutaneous involvement) (picture 26). Nodules on the upper extremities were the most common cutaneous findings in one series of 46 patients [111]. Erythema nodosum leprosum with clinical features and histologic features that closely mimic Sweet syndrome have been reported [112]. (See "Leprosy: Epidemiology, microbiology, clinical manifestations, and diagnosis", section on 'Type 2 reaction (T2R, erythema nodosum leprosum, ENL)'.)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topics (see "Patient education: Pyoderma gangrenosum (The Basics)" and "Patient education: Behçet syndrome (The Basics)" and "Patient education: Reactive arthritis (The Basics)")

Beyond the Basics topics (see "Patient education: Reactive arthritis (Beyond the Basics)")

SUMMARY

The neutrophilic dermatoses are a clinically diverse group of disorders characterized by the histologic presence of abundant neutrophils in the absence of infection. Select examples of neutrophilic dermatoses include Sweet syndrome and pyoderma gangrenosum. (See 'Introduction' above.)

Immunomodulatory therapies are the mainstays of treatment for neutrophilic dermatoses. Systemic and topical corticosteroids are often utilized in the management of these diseases. (See 'Introduction' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Samuel L Moschella, MD, FAAD, FACP, who contributed to an earlier version of this topic review.

  1. Callen JP. Neutrophilic dermatoses. Dermatol Clin 2002; 20:409.
  2. James WD. Newer neutrophilic dermatoses. Arch Dermatol 2003; 139:101.
  3. Jorizzo JL, Solomon AR, Zanolli MD, Leshin B. Neutrophilic vascular reactions. J Am Acad Dermatol 1988; 19:983.
  4. Moschella SL. Review of so-called aseptic neutrophilic dermatoses. Australas J Dermatol 1983; 24:55.
  5. Wallach D, Vignon-Pennamen MD. From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research. J Am Acad Dermatol 2006; 55:1066.
  6. Satoh TK, Mellett M, Contassot E, French LE. Are neutrophilic dermatoses autoinflammatory disorders? Br J Dermatol 2018; 178:603.
  7. Kemmett D, Hunter JA. Sweet's syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990; 23:503.
  8. von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31:535.
  9. Surovy AM, Pelivani N, Hegyi I, et al. Giant cellulitis-like Sweet Syndrome, a new variant of neutrophilic dermatosis. JAMA Dermatol 2013; 149:79.
  10. Chan MP, Duncan LM, Nazarian RM. Subcutaneous Sweet syndrome in the setting of myeloid disorders: a case series and review of the literature. J Am Acad Dermatol 2013; 68:1006.
  11. Kroshinsky D, Alloo A, Rothschild B, et al. Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis. J Am Acad Dermatol 2012; 67:945.
  12. Moreland LW, Brick JE, Kovach RE, et al. Acute febrile neutrophilic dermatosis (Sweet syndrome): a review of the literature with emphasis on musculoskeletal manifestations. Semin Arthritis Rheum 1988; 17:143.
  13. Hisanaga K, Iwasaki Y, Itoyama Y, Neuro-Sweet Disease Study Group. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology 2005; 64:1756.
  14. Baartman B, Kosari P, Warren CC, et al. Sight-threatening ocular manifestations of acute febrile neutrophilic dermatosis (Sweet's syndrome). Dermatology 2014; 228:193.
  15. Sanchez IM, Lowenstein S, Johnson KA, et al. Clinical Features of Neutrophilic Dermatosis Variants Resembling Necrotizing Fasciitis. JAMA Dermatol 2019; 155:79.
  16. Baker H, Ryan TJ. Generalized pustular psoriasis. A clinical and epidemiological study of 104 cases. Br J Dermatol 1968; 80:771.
  17. Baker H. Generalized pustular psoriasis. In: Psoriasis, Roenigk HH Jr, Maibach HI (Eds), Marcel Dekker, 1985. p.15.
  18. Zelickson BD, Muller SA. Generalized pustular psoriasis. A review of 63 cases. Arch Dermatol 1991; 127:1339.
  19. Farber EM, Nall L. Pustular psoriasis. Cutis 1993; 51:29.
  20. de Oliveira ST, Maragno L, Arnone M, et al. Generalized pustular psoriasis in childhood. Pediatr Dermatol 2010; 27:349.
  21. Zelickson BD, Muller SA. Generalized pustular psoriasis in childhood. Report of thirteen cases. J Am Acad Dermatol 1991; 24:186.
  22. McGregor JM, Barker JN, MacDonald DM. Pulmonary capillary leak syndrome complicating generalized pustular psoriasis: possible role of cytokines. Br J Dermatol 1991; 125:472.
  23. Handfield-Jones SE, Garvey M, McGibbon DH, Black MM. Capillary leak syndrome in generalized pustular psoriasis. Br J Dermatol 1992; 127:64.
  24. Shmidt E, Wetter DA, Ferguson SB, Pittelkow MR. Psoriasis and palmoplantar pustulosis associated with tumor necrosis factor-α inhibitors: the Mayo Clinic experience, 1998 to 2010. J Am Acad Dermatol 2012; 67:e179.
  25. Beylot C, Bioulac P, Doutre MS. [Acute generalized exanthematic pustuloses (four cases) (author's transl)]. Ann Dermatol Venereol 1980; 107:37.
  26. Aksentijevich I, Masters SL, Ferguson PJ, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med 2009; 360:2426.
  27. Reddy S, Jia S, Geoffrey R, et al. An autoinflammatory disease due to homozygous deletion of the IL1RN locus. N Engl J Med 2009; 360:2438.
  28. Minkis K, Aksentijevich I, Goldbach-Mansky R, et al. Interleukin 1 receptor antagonist deficiency presenting as infantile pustulosis mimicking infantile pustular psoriasis. Arch Dermatol 2012; 148:747.
  29. Calin A. Workshop III. Management of Reiter's syndrome. Ann Rheum Dis 1979; 38 Suppl 1:suppl 96.
  30. Wu IB, Schwartz RA. Reiter's syndrome: the classic triad and more. J Am Acad Dermatol 2008; 59:113.
  31. Gaylis N. Infliximab in the treatment of an HIV positive patient with Reiter's syndrome. J Rheumatol 2003; 30:407.
  32. Chamot AM, Benhamou CL, Kahn MF, et al. [Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases]. Rev Rhum Mal Osteoartic 1987; 54:187.
  33. Kahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol 1994; 8:333.
  34. Beretta-Piccoli BC, Sauvain MJ, Gal I, et al. Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in childhood: a report of ten cases and review of the literature. Eur J Pediatr 2000; 159:594.
  35. Van Doornum S, Barraclough D, McColl G, Wicks I. SAPHO: rare or just not recognized? Semin Arthritis Rheum 2000; 30:70.
  36. Rencic A, Nousari CH. Other rheumatologic diseases. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo JL, Rapini RP, et al (Eds), Elsevier Limited, 2008. p.597.
  37. Olazagasti JM, Ma JE, Wetter DA. Clinical Features, Etiologic Factors, Associated Disorders, and Treatment of Palmoplantar Pustulosis: The Mayo Clinic Experience, 1996-2013. Mayo Clin Proc 2017; 92:1351.
  38. Yamamoto T. Pustulotic arthro-osteitis associated with palmoplantar pustulosis. J Dermatol 2013; 40:857.
  39. Saghafi M, Henderson MJ, Buchanan WW. Sternocostoclavicular hyperostosis. Semin Arthritis Rheum 1993; 22:215.
  40. Kahn MF, Chamot AM. SAPHO syndrome. Rheum Dis Clin North Am 1992; 18:225.
  41. Paller AS, Pachman L, Rich K, et al. Pustulosis palmaris et plantaris: its association with chronic recurrent multifocal osteomyelitis. J Am Acad Dermatol 1985; 12:927.
  42. Sneddon IB, Wilkinson DS. Subcorneal pustular dermatosis. Br J Dermatol 1979; 100:61.
  43. Kasha EE Jr, Epinette WW. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literature. J Am Acad Dermatol 1988; 19:854.
  44. Kohl PK, Hartschuh W, Tilgen W, Frosch PJ. Pyoderma gangrenosum followed by subcorneal pustular dermatosis in a patient with IgA paraproteinemia. J Am Acad Dermatol 1991; 24:325.
  45. Dallot A, Decazes JM, Drouault Y, et al. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with amicrobial lymph node suppuration and aseptic spleen abscesses. Br J Dermatol 1988; 119:803.
  46. Schissler C, Velter C, Lipsker D. Amicrobial pustulosis of the folds: Where have we gone 25years after its original description? Ann Dermatol Venereol 2017; 144:169.
  47. Boms S, Gambichler T. Review of literature on amicrobial pustulosis of the folds associated with autoimmune disorders. Am J Clin Dermatol 2006; 7:369.
  48. Kennedy C. The spectrum of inflammatory skin disease following jejuno-ileal bypass for morbid obesity. Br J Dermatol 1981; 105:425.
  49. Dicken CH. Bowel-associated dermatosis-arthritis syndrome: bowel bypass syndrome without bowel bypass. J Am Acad Dermatol 1986; 14:792.
  50. Truchuelo MT, Alcántara J, Vano-Galván S, et al. Bowel-associated dermatosis-arthritis syndrome: another cutaneous manifestation of inflammatory intestinal disease. Int J Dermatol 2013; 52:1596.
  51. Thrash B, Patel M, Shah KR, et al. Cutaneous manifestations of gastrointestinal disease: part II. J Am Acad Dermatol 2013; 68:211.e1.
  52. Stein HB, Schlappner OL, Boyko W, et al. The intestinal bypass: arthritis-dermatitis syndrome. Arthritis Rheum 1981; 24:684.
  53. Ely PH. The bowel bypass syndrome: a response to bacterial peptidoglycans. J Am Acad Dermatol 1980; 2:473.
  54. Cox NH, Palmer JG. Bowel-associated dermatitis-arthritis syndrome associated with ileo-anal pouch anastomosis, and treatment with mycophenolate mofetil. Br J Dermatol 2003; 149:1296.
  55. Kinyó A, Farkas K, Molnár T, et al. Bowel-associated dermatosis-arthritis syndrome - a relapsing, extremely rare extraintestinal sign of acute flare-up in Crohn's disease. Eur J Dermatol 2011; 21:443.
  56. DeFilippis EM, Magro C, Jorizzo JL. Bowel-associated dermatosis - arthritis syndrome in a patient with ulcerative colitis: an extraintestinal manifestation of inflammatory bowel disease. Clin J Gastroenterol 2014; 7:410.
  57. Heard M, Zhang M, Jorizzo JL. A case of bowel-associated dermatosis-arthritis syndrome treated with ustekinumab: The importance of targeting underlying gastrointestinal disease. JAAD Case Rep 2020; 6:506.
  58. Chajek T, Fainaru M. Behçet's disease. Report of 41 cases and a review of the literature. Medicine (Baltimore) 1975; 54:179.
  59. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990; 335:1078.
  60. Arbesfeld SJ, Kurban AK. Behçet's disease. New perspectives on an enigmatic syndrome. J Am Acad Dermatol 1988; 19:767.
  61. Kolivras A, Provost P, Thompson CT. Erysipelas-like erythema of familial Mediterranean fever syndrome: a case report with emphasis on histopathologic diagnostic clues. J Cutan Pathol 2013; 40:585.
  62. Grattagliano I, Bonfrate L, Ruggiero V, et al. Novel therapeutics for the treatment of familial Mediterranean fever: from colchicine to biologics. Clin Pharmacol Ther 2014; 95:89.
  63. Gezgin Yildirim D, Seven MB, Gönen S, Söylemezoğlu O. Erysipelas-like erythema in children with familial Mediterranean fever. Clin Exp Rheumatol 2020; 38 Suppl 127:101.
  64. Harrist TJ, Fine JD, Berman RS, et al. Neutrophilic eccrine hidradenitis. A distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy. Arch Dermatol 1982; 118:263.
  65. Roustan G, Salas C, Cabrera R, Simón A. Neutrophilic eccrine hidradenitis unassociated with chemotherapy in a patient with acute myelogenous leukemia. Int J Dermatol 2001; 40:144.
  66. Herms F, Franck N, Kramkimel N, et al. Neutrophilic eccrine hidradenitis in two patients treated with BRAF inhibitors: a new cutaneous adverse event. Br J Dermatol 2017; 176:1645.
  67. Bassas-Vila J, Fernández-Figueras MT, Romaní J, Ferrándiz C. Infectious eccrine hidradenitis: a report of 3 cases and a review of the literature. Actas Dermosifiliogr 2014; 105:e7.
  68. Strutton G, Weedon D, Robertson I. Pustular vasculitis of the hands. J Am Acad Dermatol 1995; 32:192.
  69. Cohen PR. Skin lesions of Sweet syndrome and its dorsal hand variant contain vasculitis: an oxymoron or an epiphenomenon? Arch Dermatol 2002; 138:400.
  70. Galaria NA, Junkins-Hopkins JM, Kligman D, James WD. Neutrophilic dermatosis of the dorsal hands: pustular vasculitis revisited. J Am Acad Dermatol 2000; 43:870.
  71. Curcó N, Pagerols X, Tarroch X, Vives P. Pustular vasculitis of the hands. Report of two men. Dermatology 1998; 196:346.
  72. DiCaudo DJ, Connolly SM. Neutrophilic dermatosis (pustular vasculitis) of the dorsal hands: a report of 7 cases and review of the literature. Arch Dermatol 2002; 138:361.
  73. Cheng AMY, Cheng HS, Smith BJ, Stewart DA. Neutrophilic Dermatosis of the Hands: A Review of 17 Cases. J Hand Surg Am 2018; 43:185.e1.
  74. Brown TS, Fearneyhough PK, Burruss JB, Callen JP. Rheumatoid neutrophilic dermatitis in a woman with seronegative rheumatoid arthritis. J Am Acad Dermatol 2001; 45:596.
  75. Yamamoto T, Matsunaga T, Nishioka K. Rheumatoid neutrophilic dermatitis, rheumatoid papules, and rheumatoid nodules in a patient with seronegative rheumatoid arthritis. J Am Acad Dermatol 2003; 48:634.
  76. Scherbenske JM, Benson PM, Lupton GP, Samlaska CP. Rheumatoid neutrophilic dermatitis. Arch Dermatol 1989; 125:1105.
  77. Mashek HA, Pham CT, Helm TN, Klaus M. Rheumatoid neutrophilic dermatitis. Arch Dermatol 1997; 133:757.
  78. Fujio Y, Funakoshi T, Nakayama K, et al. Rheumatoid neutrophilic dermatosis with tense blister formation: a case report and review of the literature. Australas J Dermatol 2014; 55:e12.
  79. Lowe L, Kornfeld B, Clayman J, Golitz LE. Rheumatoid neutrophilic dermatitis. J Cutan Pathol 1992; 19:48.
  80. Sánchez JL, Cruz A. Rheumatoid neutrophilic dermatitis. J Am Acad Dermatol 1990; 22:922.
  81. Youn CS, Hwang JH, Cho KH, Yoon TY. Colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis. J Dermatol 2000; 27:782.
  82. Coutinho I, Pereira N, Gouveia M, et al. Interstitial Granulomatous Dermatitis: A Clinicopathological Study. Am J Dermatopathol 2015; 37:614.
  83. Rosenbach M, English JC 3rd. Reactive Granulomatous Dermatitis: A Review of Palisaded Neutrophilic and Granulomatous Dermatitis, Interstitial Granulomatous Dermatitis, Interstitial Granulomatous Drug Reaction, and a Proposed Reclassification. Dermatol Clin 2015; 33:373.
  84. Rodríguez-Garijo N, Bielsa I, Mascaró JM Jr, et al. Reactive granulomatous dermatitis as a histological pattern including manifestations of interstitial granulomatous dermatitis and palisaded neutrophilic and granulomatous dermatitis: a study of 52 patients. J Eur Acad Dermatol Venereol 2021; 35:988.
  85. Long D, Thiboutot DM, Majeski JT, et al. Interstitial granulomatous dermatitis with arthritis. J Am Acad Dermatol 1996; 34:957.
  86. Sangueza OP, Caudell MD, Mengesha YM, et al. Palisaded neutrophilic granulomatous dermatitis in rheumatoid arthritis. J Am Acad Dermatol 2002; 47:251.
  87. Gulati A, Paige D, Yaqoob M, et al. Palisaded neutrophilic granulomatous dermatitis associated with systemic lupus erythematosus presenting with the burning rope sign. J Am Acad Dermatol 2009; 61:711.
  88. Fett N, Kovarik C, Bennett D. Palisaded neutrophilic granulomatous dermatitis without a definable underlying disorder treated with dapsone. J Am Acad Dermatol 2011; 65:e92.
  89. Misago N, Shinoda Y, Tago M, Narisawa Y. Palisaded neutrophilic granulomatous dermatitis with leukocytoclastic vasculitis in a patient without any underlying systemic disease detected to date. J Cutan Pathol 2010; 37:1092.
  90. Chu P, Connolly MK, LeBoit PE. The histopathologic spectrum of palisaded neutrophilic and granulomatous dermatitis in patients with collagen vascular disease. Arch Dermatol 1994; 130:1278.
  91. Tomasini C, Pippione M. Interstitial granulomatous dermatitis with plaques. J Am Acad Dermatol 2002; 46:892.
  92. Verneuil L, Dompmartin A, Comoz F, et al. Interstitial granulomatous dermatitis with cutaneous cords and arthritis: a disorder associated with autoantibodies. J Am Acad Dermatol 2001; 45:286.
  93. Heidary N, Mengden S, Pomeranz MK. Palisaded neutrophilic and granulomatous dermatosis. Dermatol Online J 2008; 14:17.
  94. Deng A, Harvey V, Sina B, et al. Interstitial granulomatous dermatitis associated with the use of tumor necrosis factor alpha inhibitors. Arch Dermatol 2006; 142:198.
  95. Germanas JP, Mehrabi D, Carder KR. Palisaded neutrophilic granulomatous dermatitis in a 12-year-old girl with systemic lupus erythematosus. J Am Acad Dermatol 2006; 55:S60.
  96. Gerbing EK, Metze D, Luger TA, Ständer S. [Interstitial granulomatous dermatitis without arthritis: successful therapy with hydroxychloroquine]. J Dtsch Dermatol Ges 2003; 1:137.
  97. Peroni A, Colato C, Schena D, et al. Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern. Br J Dermatol 2012; 166:775.
  98. Kroesen S, Itin PH, Hasler P. Arthritis and interstitial granulomatous dermatitis (Ackerman syndrome) with pulmonary silicosis. Semin Arthritis Rheum 2003; 32:334.
  99. Walling HW, Swick BL. Interstitial granulomatous dermatitis associated with chronic inflammatory demyelinating polyneuropathy. Cutis 2012; 90:30.
  100. Bremner R, Simpson E, White CR, et al. Palisaded neutrophilic and granulomatous dermatitis: an unusual cutaneous manifestation of immune-mediated disorders. Semin Arthritis Rheum 2004; 34:610.
  101. Zoli A, Massi G, Pinnelli M, et al. Interstitial granulomatous dermatitis in rheumatoid arthritis responsive to etanercept. Clin Rheumatol 2010; 29:99.
  102. Kreuter A, Gambichler T, Altmeyer P. Infliximab therapy for interstitial granulomatous dermatitis. J Eur Acad Dermatol Venereol 2007; 21:251.
  103. Cornillier H, Kervarrec T, Tabareau-Delalande F, et al. Interstitial granulomatous dermatitis occurring in a patient with SAPHO syndrome one month after starting leflunomide, and subsequently disappearing with ustekinumab. Eur J Dermatol 2016; 26:614.
  104. Leloup P, Aubert H, Causse S, et al. Ustekinumab therapy for severe interstitial granulomatous dermatitis with arthritis. JAMA Dermatol 2013; 149:626.
  105. Kieffer C, Cribier B, Lipsker D. Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore) 2009; 88:23.
  106. Gusdorf L, Bessis D, Lipsker D. Lupus erythematosus and neutrophilic urticarial dermatosis: a retrospective study of 7 patients. Medicine (Baltimore) 2014; 93:e351.
  107. Lee WJ, Kang HJ, Shin HJ, et al. Neutrophilic urticarial dermatosis and Sweet-like neutrophilic dermatosis: under-recognized neutrophilic dermatoses in lupus erythematosus. Lupus 2018; 27:628.
  108. Krause K, Tsianakas A, Wagner N, et al. Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study. J Allergy Clin Immunol 2017; 139:1311.
  109. Esler-Brauer L, Rothman I. Tender nodules on the palms and soles: palmoplantar eccrine hidradenitis. Arch Dermatol 2007; 143:1201.
  110. Neri I, Baraldi C, Ricci L, Patrizi A. Painful nodules on the soles in a 9-year-old boy. Arch Dis Child 2014; 99:856.
  111. Negera E, Walker SL, Girma S, et al. Clinico-pathological features of erythema nodosum leprosum: A case-control study at ALERT hospital, Ethiopia. PLoS Negl Trop Dis 2017; 11:e0006011.
  112. Heng YK, Chiam YT, Giam YC, Chong WS. Lepromatous leprosy in erythema nodosum leprosum reaction mimicking Sweet's syndrome. Int J Dermatol 2011; 50:1124.
Topic 5571 Version 36.0

References

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟