Linear IgA bullous dermatosis: Pathogenesis, clinical features, and diagnosis

Authors:: Enno Schmidt, MD, PhD; Adela Rambi G Cardones, MD
Section Editor:: John J Zone, MD
Deputy Editor:: Abena O Ofori, MD

Literature review current through: May 2025. | This topic last updated: Jul 03, 2025.

INTRODUCTION —

Linear IgA (immunoglobulin A) bullous dermatosis (LABD), also known as linear IgA disease, is a rare, idiopathic or drug-induced autoimmune blistering disease characterized by the linear deposition of IgA at the dermoepidermal junction.

LABD occurs in both adults and children, and since the late 1980s, the disorder designated as chronic bullous disease of childhood has been recognized as the childhood form of LABD [1]. Children classically present with widespread annular blisters that exhibit a predilection for the lower abdomen, thighs, and groin (picture 1A-D). In adults, annular lesions are less common, and blisters often involve the extensor extremities, face, and trunk (picture 2A-D). Mucosal lesions, manifesting as inflammation, erosions, or scarring, may also occur in LABD.

The pathogenesis, clinical features, and diagnosis of LABD will be reviewed here. The management of LABD and the general approach to the evaluation of patients with blistering skin lesions are reviewed separately.

●(See "Linear IgA bullous dermatosis: Treatment".)

●(See "Approach to the patient with cutaneous blisters".)

EPIDEMIOLOGY —

LABD is rare. Reports of disease incidence from various countries range from less than 0.5 to 2.3 cases per million individuals per year [2,3].

●Age – LABD may occur more frequently in children than in adults and is considered the most common autoimmune blistering disease in children [4-6]. In a study that analyzed 2014 data from a health insurance database that included approximately 9.5 million individuals in Germany, the estimated prevalence of LABD was 24.3 per million for patients less than 18 years of age compared with 10.3 per million for patients 18 years or older [5].

Adults frequently develop LABD later in life, with many cases occurring after the age of 60 [1,2]. In children, the disease usually presents between the ages of 6 months and 10 years; in a series of 25 affected children, the average age of onset was 4.5 years [1]. Rarely, LABD occurs in neonates [7,8].

●Sex – A predilection for LABD based on sex has not been definitively established [2,9].

PATHOGENESIS —

Although the presence of IgA antibodies bound to the basement membrane zone is accepted as a characteristic feature of LABD, the mechanism of lesion formation is not well understood.

Both humoral and cellular immune responses may be involved in the pathogenesis of LABD. In particular, tissue injury resulting from an antibody-induced local inflammatory response and the release of proteolytic enzymes by neutrophils and other inflammatory cells contributes to the development of skin and mucosal lesions [2].

Major target antigens — Most patients with LABD have IgA1 antibodies that target a 97 kDa antigen (LABD97) and a 120 kDa antigen (LAD-1) within the basement membrane zone. Both of these antigens are proteolytic fragments of the extracellular portion of bullous pemphigoid antigen 180 (BP180; also referred to as bullous pemphigoid antigen 2 or type XVII collagen), a transmembrane protein that plays a key role in epidermal-dermal adhesion (figure 1) [10,11].

Cleavage of the BP180 antigen exposes neoepitopes on the 15^th collagenous domain that react with IgA autoantibodies [12]. Less frequently, the NC16A epitope on BP180 has been associated with LABD [13-15].

Patients with LABD may also have IgA antibodies that are directed against other basement membrane antigens. (See 'Patients with nonclassic immunopathologic or serologic findings' below.)

Autoantibodies — In vivo and in vitro studies provide experimental evidence for the pathogenicity of IgA against BP180, including the role of IgA interaction with neutrophils [16,17]. Antibodies from patients with LABD that bind to the NC16A domain of BP180 produce blisters in experimental mice but require the presence of neutrophils to do so [16]. In an experimental model, mice that produced human IgA antibodies against mouse BP180/type XVII collagen developed neutrophil accumulation and blisters [17]. In addition, blocking the IgA Fc receptor (Fc-alpha RI [CD89]), the receptor that binds the heavy chain-constant region of IgA, eliminates neutrophil accumulation and blister formation.

RISK FACTORS —

In most patients with LABD, the inciting factor for the disease is unknown.

Genetic factors — Genetic factors may contribute to the development of LABD. Associations of LABD with human leukocyte antigen (HLA) B8, HLA Cw7, HLA DR3, HLA DQ2, HLA-DQB102:01, and the tumor necrosis factor-2 allele have been reported [18,19]. Further study is necessary to determine the role of both drugs and genetics in LABD.

Drug exposure — Multiple case reports have documented drug exposure as a precipitating factor [20,21]. Most reported cases of drug-induced LABD have occurred in adults, but LABD in association with drug exposure and routine vaccinations has also occurred in children [1,20,22,23].

Vancomycin is the pharmacologic agent most frequently reported as a potential inciting factor and is associated with a majority of drug-induced LABD cases [20,24,25]. A variety of other medication classes have been reported to induce LABD. A pharmacovigilance analysis has identified ampicillin-sulbactam and ketoprofen as having strong links to LABD [25]. Examples of other drugs that may be linked to LABD include a variety of antibiotics, nonsteroidal anti-inflammatory agents (eg, diclofenac, naproxen, piroxicam), lithium, captopril, amiodarone, phenytoin, cyclosporine, furosemide, interferon alfa, nemvaleukin alfa, somatostatin, atezolizumab, and spironolactone (table 1) [2,22,26-30]. (See "Vancomycin hypersensitivity".)

Although reports of autoimmune blistering diseases associated with immune checkpoint inhibitor therapy are increasing, immune checkpoint inhibitor-associated LABD appears to be rare [31-34]. In a case series in which 9 of 853 patients treated with an immune checkpoint inhibitor developed an autoimmune blistering disease, the only patient to develop LABD was also receiving vancomycin [31].

Additionally, there are cases of LABD following influenza and coronavirus disease 2019 (COVID-19) vaccination [35-39].

CLINICAL MANIFESTATIONS —

LABD may present with lesions on the skin, the mucous membranes, or both locations. Although both children and adults develop LABD, there are differences in the clinical characteristics of adult and pediatric LABD.

Cutaneous lesions — Since blister formation in LABD occurs subepidermally, the vesicles and bullae that form on the skin of affected patients typically have a tense, rather than flaccid, pemphigus-like quality. (See "Approach to the patient with cutaneous blisters", section on 'Definition'.)

●Children – LABD of childhood, also known as chronic bullous disease of childhood, most often presents with the acute development of vesicles or bullae on sites of inflamed or noninflamed skin (picture 1A and picture 1B-D). New blisters often form at the periphery of resolving lesions, resulting in an arciform or annular appearance (picture 3). Such lesions are frequently described as resembling strings of pearls, crowns of jewels, or rosettes [40].

The distribution of skin lesions is usually widespread, involving the trunk, face (particularly perioral area), genitalia, hands, feet, and other sites. The most intensely involved areas are often the perineum, lower abdomen, and inner thighs [1,9,41].

Affected children may be asymptomatic, but pruritus is common and may be severe. In some patients, intense pruritus heralds recurrences of the disease [10,11].

●Adults – Adult patients with LABD typically experience an abrupt onset of skin lesions; less frequently, the disease develops more slowly [42]. The tense vesicles and bullae may arise on normal skin or within inflammatory plaques (picture 2A-D). Annular lesions demonstrating peripheral vesiculation develop less frequently in adults than in children [42].

The trunk, extensor extremities, buttocks, and face (particularly the perioral area) are common sites for lesion development [2]. This distribution can make it difficult to distinguish LABD from dermatitis herpetiformis, which affects similar sites. Localized variants of LABD presenting as limited blistering eruptions or annular inflammatory plaques have also been reported [43-49].

Pruritus may be intense and can result in the development of excoriated papules or prurigo nodularis-like lesions [50,51].

Mucosal involvement — Mucosal involvement can occur in LABD, but skin involvement is the predominant finding. Patients with mucosal-predominant disease and immunopathologic findings of LABD are generally considered to have other diseases. (See 'Patients with mucous membrane predominant involvement' below.)

●Frequency – Mucosal disease occurs in up to 80 percent of adult patients [1]. Estimates of the incidence of mucosal LABD in childhood disease vary widely. In a series of 25 children with LABD in the United Kingdom, mucosal involvement was present in 64 percent [1]. In contrast, similarly sized retrospective studies in Japan and Tunisia found mucosal disease in only 3 and 8 percent of children, respectively [52,53].

●Clinical findings – Mucosal lesions primarily present as erosions or ulcers; the detection of intact vesicles or bullae is uncommon. Any mucosal surface may be affected, including the oral cavity, conjunctiva, nose, genitalia, pharynx, larynx, anus, and esophagus [1,2,7,42].

The oral and ocular mucosa are the most common mucosal sites [1,2]. In patients with oral disease, lesions are frequently located on the palate, palatine arches, or buccal mucosa [2]. Erosive gingivitis and erosive cheilitis may also occur as manifestations of oral LABD [1,54,55].

Drug-induced disease

●Course – Drug-induced LABD usually begins within one month of drug initiation and often resolves within several weeks following drug cessation [22,56,57]. However, the eruption persists for longer in some patients. Antibiotic-impregnated devices (eg, cement) may contribute to delayed resolution in the setting of antibiotic-induced LABD [58].

Re-exposure to an inciting agent can result in the rapid recurrence of blistering [59].

●Clinical findings – The clinical findings in drug-induced LABD usually do not differ significantly from those of idiopathic disease [20,24]. Occasionally, lesions are localized rather than widespread or resemble morbilliform drug eruptions, erythema multiforme, or toxic epidermal necrolysis (TEN) (picture 4) [60-66]. The presence of mucosal involvement varies [20].

The shared clinical features of idiopathic LABD and drug-induced LABD were demonstrated in a retrospective study of 16 patients considered to have spontaneous LABD and 12 patients considered to have drug-induced LABD that found similar frequencies of erythematous plaques, string of pearls-like configurations, target or target-like lesions, and mucosal involvement in the two groups [66]. Only the atypical features of large erosions and a positive Nikolsky sign were significantly more frequent in the drug-induced group.

Patients with vancomycin-induced disease may have an increased risk for TEN-like presentations. In a retrospective study of 69 patients with drug-induced LABD (including 39 with suspected vancomycin-induced disease), vancomycin was the suspected cause in 11 of 14 patients (79 percent) with TEN-like features [24].

ASSOCIATED DISORDERS —

LABD has occurred in the setting of ulcerative colitis, hematologic or solid organ malignancies, and other disorders.

●Ulcerative colitis – Ulcerative colitis is the most common disorder reported in association with LABD [52,67-71]. In a retrospective study of 70 patients with LABD referred to two dermatology departments in the United Kingdom, five patients (7 percent) had ulcerative colitis, and ulcerative colitis preceded the diagnosis of LABD by an average of six years [68]. Moreover, a Japanese review of 213 reported cases of LABD identified four patients with this disease [52].

The reason for an association between LABD and ulcerative colitis is unclear. Some authors have suggested that abnormal IgA1 production by the inflamed bowel may contribute to the development of LABD [68]. Although improvement in skin disease following surgical intervention for bowel disease has been reported [69-71], in other patients, skin disease has persisted after surgery [68]. Limited data from case reports suggest that persistence of LABD may be more common after colectomy with sparing of the rectal mucosa than after proctocolectomy [72].

●Malignancy – The occurrence of LABD in association with lymphoproliferative and solid organ malignancies has been reported in case reports [48,73-90]. However, further studies are necessary to confirm an association between LABD and malignancy. Thus, in the absence of signs or symptoms suggestive of a malignancy, screening beyond age-appropriate investigation is not warranted in patients with LABD.

●Other – Other disorders reported to occur in association with LABD in a few patients include a variety of infections [45,91-95], IgA nephropathy [96], psoriasis [94,97], and systemic lupus erythematosus [52,98]. The development of LABD has also been reported following exposure to ultraviolet light [45,99,100].

Unlike dermatitis herpetiformis, which may clinically and histologically resemble LABD, gluten sensitivity is not a feature of LABD, and gluten-free diets do not improve LABD [2,101].

DIAGNOSIS —

The clinical signs of LABD may resemble multiple mucocutaneous diseases.

When to suspect linear IgA bullous dermatosis — A diagnosis of LABD is usually suspected when children or adults develop tense bullae in an arciform or annular distribution on the skin. In patients with blistering disorders without these features, pathologic or immunopathologic findings supportive of LABD typically lead to the consideration of LABD. (See 'Patient assessment' below.)

Diagnostic criteria and approach — Confirming a diagnosis of LABD typically involves correlation of the clinical, pathologic, and immunopathologic findings. Key immunopathologic tests include direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) studies.

The gold standard for diagnosis is the detection of linear deposits of IgA along the basement membrane zone on DIF [10]. (See 'Pathology' below.)

Diagnostic criteria and approach — We agree with the diagnostic criteria proposed by the European Academy of Dermatology and Venerology Task Force Autoimmune Bullous Diseases. These include the presence of both of the following [6]:

●Clinical manifestations compatible with LABD. Mucous membrane involvement must not be predominant over skin involvement. (See 'Patients with mucous membrane predominant involvement' below.)

●Positive DIF showing solely linear deposits of IgA along the dermoepidermal junction or of both IgA and immunoglobulin G (IgG). In the latter case, the fluorescence intensity of the IgA deposits must be stronger than that of IgG. (See 'Direct immunofluorescence' below.)

We perform both DIF and IIF routinely for patients with suspected LABD. IIF is performed on salt-split skin and classically shows IgA bound to the epidermal side of split skin in LABD. (See 'Indirect immunofluorescence' below.)

In the event of negative DIF, DIF should generally be repeated [6]. IIF findings compatible with LABD can support the diagnosis when a repeat DIF is negative.

If both DIF and IIF are negative for features of LABD or are not feasible, immunoblot or enzyme-linked immunosorbent assay (ELISA) testing may also be used to assess for findings consistent with LABD [6]. (See 'Indirect immunofluorescence' below and 'Other tests for patients with inconclusive findings' below.)

Patients with mucous membrane predominant involvement — Patients with mucous membrane-predominant involvement are generally not considered to have LABD even in the presence of pathologic or DIF findings consistent with LABD. Guidelines from the European Academy of Dermatology and Venereology support the classification of patients with predominant mucosal involvement as having mucous membrane pemphigoid irrespective of the target antigen and autoantibody isotype [102,103]. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid".)

Patients with nonclassic immunopathologic or serologic findings — Although LABD may present with atypical features, the possibility of an alternative diagnosis should also be considered for patients with nonclassic immunopathologic or serologic findings.

●Concomitant IgG autoantibodies – The presence of both IgA and IgG antibodies against basement membrane zone components has been detected in a subset of patients with features consistent with LABD. In a review of 213 reported cases of LABD in Japan, such findings were present in approximately 20 percent of patients [52]. A separate study from Germany reported dual positivity in 18 percent of 222 patients with LABD [104].

Additional study is necessary to determine whether this presentation represents a distinct disorder or a variant of LABD. The term "linear IgA/IgG dermatosis" has been used to refer to this presentation [105].

●IgA autoantibodies against other antigens – IIF studies demonstrating antibodies bound only to the dermal side of salt-split skin suggest the presence of IgA autoantibodies against basement membrane zone antigens other than the antigens classically associated with LABD. Additionally, immunoblotting or ELISA studies can identify antibodies against alternative antigens, such as type VII collagen, laminin 332, and p200. (See 'Indirect immunofluorescence' below and 'Other tests for patients with inconclusive findings' below.)

Although patients with mucosa-predominant presentations of autoimmune subepidermal blistering diseases are generally classified as having mucous membrane pemphigoid, the diagnostic classification of patients with skin-predominant blistering and IgA autoantibodies against basement membrane zone components other than products of the extracellular domain of the bullous pemphigoid antigen 180 (BP180) antigen is debated [106]. (See 'Patients with mucous membrane predominant involvement' above.)

Experts diverge on whether these entities represent heterogeneous manifestations of LABD or different disease entities.

•Type VII collagen – Some experts, including authors of the 2024 LABD guidelines from the European Academy of Dermatology and Venereology, classify patients with clinical findings of LABD but IgA antibodies to type VII collagen as having IgA epidermolysis bullosa acquisita [6,104]. Other experts support classifying this presentation as anti-type VII collagen sublamina densa-type LABD [107,108].

Type VII collagen appears to be the target antigen in some patients with vancomycin-induced bullous disease that clinically resembles LABD [109]. (See "Epidermolysis bullosa acquisita" and 'Risk factors' above.)

•Laminin 332 or p200 – There are case reports of patients with skin-predominant autoimmune subepidermal blistering disease associated with predominant or exclusive IgA autoantibodies against laminin 332 (a basement membrane zone antigen also associated with mucous membrane pemphigoid) or p200 (a 200-kDa protein in the lower lamina lucida of the basement membrane zone also associated with p200 pemphigoid) (figure 1) [110,111]. We consider these patients to have a subtype of LABD.

The term "anti-laminin gamma-1/p200 sublamina densa-type linear IgA bullous dermatosis" has also been proposed for patients with antibodies against p200 [108]. Some authors have classified these patients as IgA anti-p200 pemphigoid rather than LABD [112,113].

Epitope spreading may play a role when autoantibodies against multiple basement membrane zone antigens are detected in patients with LABD [6].

Patient assessment — The initial assessment of a patient with suspected LABD includes a history, physical examination, and histopathology and immunopathology studies.

History — The patient history should include an assessment for potential inciting medications (table 1).

Established drug algorithms can be helpful for supporting causality [114]. When available, drug-induced lymphocyte stimulation testing may provide additional information when multiple medications are temporally related to the onset of skin disease [115]. (See 'Drug exposure' above and "Drug reaction with eosinophilia and systemic symptoms (DRESS)", section on 'In vitro tests'.)

Physical examination — A full skin examination should be performed to assess for consistent clinical findings and to determine the extent of involvement. (See 'Cutaneous lesions' above.)

The oral, ocular, nasal, and genital mucosa should also be assessed for erosions, ulcers, or blisters suggestive of mucosal involvement. Suggestive symptoms can include eye discomfort, dysphagia, dysphonia, dysuria, and dyspareunia. (See 'Mucosal involvement' above.)

Patients with signs or symptoms of ocular, nasal, pharyngeal, laryngeal, or esophageal disease should be referred to appropriate specialists for further evaluation.

Pathology — The pathologic tests for LABD include routine histopathologic examination, DIF, and IIF. Skin biopsy specimens are necessary for routine histopathologic examination and DIF. IIF involves the assessment for relevant autoantibodies in patient serum.

Routine histopathology

●Specimen collection – Ideal specimens for routine histopathologic examination should include both a portion of blistered skin and adjacent intact skin. A 4 mm punch biopsy taken from the edge of a blister is usually sufficient. (See "Approach to the patient with cutaneous blisters", section on 'Skin biopsy'.)

●Findings – The histopathologic findings of LABD are not diagnostic and often closely resemble dermatitis herpetiformis.

Characteristic findings include a subepidermal blister with an underlying neutrophil-predominant dermal infiltrate, sometimes forming microabscesses (picture 5) [2]. In approximately one-quarter of patients, eosinophils predominate, and lymphocytes are present in approximately 50 percent of patients [1,116].

Similar pathologic findings can be seen in bullous pemphigoid, mucous membrane pemphigoid, and anti-p200 pemphigoid. (See "Approach to the patient with cutaneous blisters", section on 'Skin biopsy'.)

Direct immunofluorescence

●Specimen collection – For DIF, a small biopsy (eg, 4 mm punch) should be taken from perilesional skin (clinically normal-appearing skin immediately adjacent to a lesion). The specimen should be sent to an immunopathology laboratory in a preservative, such as Michel's medium or isotonic sodium chloride (NaCl) solution, or as a snap-frozen specimen. Specimens for DIF should not be placed in formalin.

When the specimen can be delivered to the laboratory within minutes, transport on saline-moistened gauze is an alternative. (See "Approach to the patient with cutaneous blisters", section on 'Direct immunofluorescence'.)

The technique for performing DIF is reviewed separately. (See "Approach to the patient with cutaneous blisters", section on 'Direct immunofluorescence'.)

●Findings – The diagnostic DIF finding in LABD is a linear deposit of IgA (exclusive or predominant over IgG) at the dermoepidermal junction (picture 6) [10]. In LABD, an n-serrated pattern is present. In contrast, IgA epidermolysis bullosa acquisita with antibodies against type VII collagen characteristically exhibits a u-serrated pattern [117]. (See "Approach to the patient with cutaneous blisters", section on 'Basement membrane zone-split skin technique'.)

Indirect immunofluorescence — IIF can detect circulating IgA antibodies against basement membrane zone components and may aid in diagnosis, particularly when a skin biopsy is not feasible.

●Specimen collection – A patient blood sample is collected and centrifuged to separate serum. Subsequently, progressive dilutions of the patient's serum are incubated with a tissue substrate. For best results, IIF should be performed on a substrate of salt-split human skin [11,118].

The technique for performing IIF is reviewed separately. (See "Approach to the patient with cutaneous blisters", section on 'Indirect immunofluorescence' and "Approach to the patient with cutaneous blisters", section on 'Basement membrane zone-split skin technique'.)

●Findings – In many cases, IIF studies reveal IgA bound to the epidermal side of salt-split skin, a finding that is consistent with reactivity against BP180 or its 97 and 120 kDa fragments [111,119,120]. However, negative IIF findings are not uncommon. Circulating IgA autoantibodies are only present in low levels in LABD (with titers of 1:10 to 1:40) and in 50 to 70 percent of patients [6]. (See 'Pathogenesis' above.)

In individual cases, antibody binding to the dermal side of salt-split skin is seen and suggests that type VII collagen, laminin-332, or the p200 antigen may be the target antigens [107]. (See 'Patients with nonclassic immunopathologic or serologic findings' above and "Approach to the patient with cutaneous blisters", section on 'Indirect immunofluorescence'.)

Other tests for patients with inconclusive findings — When DIF and IIF are negative (or not feasible) and suspicion for LABD is high, specialized laboratories can perform immunoblot and/or ELISA tests to assess for findings supportive of LABD. (See 'Diagnostic criteria and approach' above.)

Both immunoblotting and ELISA tests are performed with patient serum.

●Immunoblotting – Immunoblotting involves use of gel electrophoresis to separate proteins from an LABD antigen source (eg, extracts from skin or amniotic membrane, cultured keratinocytes, or recombinant fragments of BP180) by molecular weight. Separated proteins are transferred onto a nitrocellulose membrane, which is then incubated with patient serum, allowing antibodies in patient serum to bind to the proteins. Subsequent incubation with a labeled secondary antibody that binds to the patient's antibodies enables identification of LABD-related antibodies in the patient's serum [121-124].

●ELISA – ELISA testing can support a diagnosis of LABD through the detection of serum antibodies against recombinant proteins of BP180. Various recombinant proteins of BP180 have been studied [125,126]. (See "Approach to the patient with cutaneous blisters", section on 'Antigen-specific serologic testing'.)

DIFFERENTIAL DIAGNOSIS —

The acute onset of widespread annular or arcuate blisters strongly suggests the possibility of LABD (picture 3). However, annular lesions are not always present, and multiple other disorders share clinical features with LABD.

Although the clinical features of LABD can be particularly difficult to distinguish from dermatitis herpetiformis, the distinct immunopathologic findings in LABD distinguish these diseases.

Examples of conditions to consider in the differential diagnosis are provided below:

●Bullous impetigo – Bullous impetigo is a Staphylococcus aureus infection that most commonly occurs in young children. Multiple easily-ruptured bullae with clear or yellow fluid are typically present on the face, trunk, or extremities (picture 7). A collarette of scale often remains at the site of ruptured lesions. A culture of the fluid from an intact blister can be used to detect S. aureus. (See "Impetigo", section on 'Bullous impetigo'.)

●Autoimmune blistering disorders

•Dermatitis herpetiformis – Dermatitis herpetiformis is an autoimmune subepidermal blistering disorder with clinical and histopathologic features that can closely resemble LABD. Patients typically present with grouped vesicles or bullae on the scalp, extensor extremities, or buttocks (picture 8). Direct immunofluorescence (DIF) microscopy is useful for distinguishing these disorders. (See "Dermatitis herpetiformis".)

•Bullous pemphigoid – Bullous pemphigoid is an autoimmune subepidermal blistering disorder that typically affects older adults and manifests as pruritic inflammatory plaques and bullae that are primarily distributed on the trunk and extremities (picture 9A-C). Histopathologic examination usually reveals a higher proportion of eosinophils than are present in LABD. DIF in bullous pemphigoid usually reveals linear IgG and C3 deposits at the basement membrane zone. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid", section on 'Clinical features of bullous pemphigoid'.)

•Pemphigoid gestationis – Pemphigoid gestationis is an autoimmune subepidermal blistering disorder that occurs in pregnant individuals. The pruritic eruption frequently begins around the umbilicus and subsequently spreads elsewhere (picture 10A-C). DIF reveals the deposition of C3 in a linear pattern at the basement membrane zone. (See "Dermatoses of pregnancy", section on 'Pemphigoid gestationis'.)

•Bullous lupus erythematosus – Bullous lupus erythematosus is a rare subepidermal blistering disorder (picture 11A-B). The presence of a bullous disorder in a patient with systemic lupus erythematosus suggests the possibility of this disease. (See "Bullous systemic lupus erythematosus".)

•Epidermolysis bullosa acquisita – Epidermolysis bullosa acquisita is a rare, acquired subepidermal blistering disorder of the skin and mucous membranes (picture 12). Scarring and milia are common associated features. On DIF, deposits of IgG are most commonly detected at the basement membrane zone. Less commonly, other immunoglobulins (including IgA) may be present. The classification of disease with exclusive or predominant IgA reactivity against type VII collagen as IgA epidermolysis bullosa acquisita or a variant of LABD has been debated. (See 'Patients with nonclassic immunopathologic or serologic findings' above and "Epidermolysis bullosa acquisita".)

•Mucous membrane pemphigoid – Mucous membrane pemphigoid is defined as an autoimmune subepidermal blistering disorder in which mucosal surfaces are exclusively or predominantly involved, irrespective of the autoantibody isotype. This includes patients with predominant mucous membrane involvement and exclusive or predominant IgA reactivity against the basement membrane zone [102,103]. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid", section on 'Clinical features of mucous membrane pemphigoid'.)

Infrequently, LABD may resemble prurigo nodularis [50], morbilliform drug eruptions [62], erythema multiforme [63], or toxic epidermal necrolysis (TEN) [60,61]. In addition, LABD in children may be mistaken for child abuse. (See 'Cutaneous lesions' above and 'Drug-induced disease' above.)

SUMMARY AND RECOMMENDATIONS

●Epidemiology – Linear IgA bullous dermatosis (LABD) is a rare, autoimmune subepidermal blistering disease. LABD can occur in both children and adults and is the most common autoimmune blistering disease in children. (See 'Epidemiology' above.)

●Pathogenesis – LABD is characterized by IgA antibodies bound to components of the cutaneous basement membrane zone. The major target antigens include a 97 kDa antigen (LABD97) and a 120 kDa antigen (LAD-1). LABD97 and LAD-1 are fragments of the extracellular portion of bullous pemphigoid antigen 180 (BP180) (figure 1). Occasionally, other target antigens are detected. (See 'Major target antigens' above and 'Patients with nonclassic immunopathologic or serologic findings' above.)

Tissue injury resulting from an antibody-induced local inflammatory response and the release of proteolytic enzymes by neutrophils and other inflammatory cells contribute to the development of skin and mucosal lesions. (See 'Pathogenesis' above.)

●Risk factors – Most patients with LABD have idiopathic LABD. Drug exposure may also contribute to the development of LABD. Vancomycin is the most commonly associated drug. Drug-induced LABD usually begins within one month of drug initiation. (See 'Risk factors' above.)

●Clinical manifestations

•Children – Childhood LABD often presents with the acute onset of vesicles and bullae on normal-appearing or visibly inflamed skin. The blisters tend to appear in an arciform or annular distribution (picture 1A-D). Skin lesions are often widespread, with the most intense involvement on the perineum, lower abdomen, and inner thighs.

•Adults – Adults may have abrupt or slower onset of skin blisters. Common sites include the trunk, extensor extremities, buttocks, and face (picture 2A-D). Arciform and annular lesions are less common in adults than in children.

•Associated findings – Pruritus and concomitant mucous membrane involvement are common.

●Diagnosis – Confirming a diagnosis of LABD involves correlation of the clinical, pathologic, and immunopathologic findings.

•When to suspect LABD – Suspicion for LABD often arises when patients present with tense bullae in an arciform or annular distribution on the skin. (See 'Patient assessment' above.)

•Diagnostic approach – Our typical approach to confirmatory testing includes skin biopsies for routine histopathology examination and direct immunofluorescence (DIF) and a serum specimen for an indirect immunofluorescence (IIF) study on salt-split skin. Classic DIF findings include linear deposits of IgA along the dermoepidermal junction. In most, but not all patients, IIF demonstrates antibodies bound to the epidermal side of salt-split skin. (See 'Pathology' above.)

Clinicians should also assess for possible inciting medications. (See 'History' above.)

●Differential diagnosis – The differential diagnosis of LABD includes multiple other blistering disorders. In particular, the clinical features of LABD can resemble dermatitis herpetiformis.

ACKNOWLEDGMENTS —

The UpToDate editorial staff acknowledges Russell P Hall, III, MD, and Caroline L Rao, MD, who contributed to earlier versions of this topic review.

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