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Extragenital lichen sclerosus

Extragenital lichen sclerosus
Author:
Heidi Jacobe, MD
Section Editor:
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor:
Abena O Ofori, MD
Literature review current through: Jul 2022. | This topic last updated: Jan 15, 2021.

INTRODUCTION — Lichen sclerosus (also known as lichen sclerosus et atrophicus) is a chronic inflammatory disorder characterized by the presence of porcelain-white, atrophic plaques on the skin (picture 1A-C). Most cases of lichen sclerosus involve the female genitalia; extragenital manifestations occur in a minority of patients.

The epidemiology, clinical manifestations, and treatment of extragenital lichen sclerosus will be reviewed here (algorithm 1). Anogenital lichen sclerosus is discussed separately. (See "Vulvar lichen sclerosus" and "Balanitis in adults".)

EPIDEMIOLOGY — Extragenital manifestations are estimated to occur in approximately 15 to 20 percent of patients with lichen sclerosus [1]. Similar to genital lichen sclerosus, extragenital disease appears to be more common in women than in men. In a retrospective review, 17 out of 20 patients with histopathologic findings consistent with extragenital lichen sclerosus were female [2]. The disorder is rare in children.

PATHOGENESIS — The pathogenesis of lichen sclerosus is not well understood, and most information on this subject has been derived from studies in genital disease. Proposed etiologic factors for lichen sclerosus have included immune dysfunction, genetic predisposition, infectious agents, and trauma [3-12]. (See "Vulvar lichen sclerosus", section on 'Etiology'.)

CLINICAL MANIFESTATIONS — Extragenital lichen sclerosus can occur in any location on the skin and infrequently appears in the oral cavity. Concomitant vulvar or penile lichen sclerosus may or may not be present. Conjunctival lesions do not occur.

Cutaneous lesions — Extragenital lesions are most commonly found on the back, shoulder, neck, wrist, thigh, and inframammary areas [1,13]. Disease following the lines of Blaschko (figure 1) also has been reported [14,15].

Early, active skin lesions of extragenital lichen sclerosus often appear as flat-topped and slightly scaly, hypopigmented, white, or mildly erythematous, polygonal papules that may coalesce to form larger plaques with peripheral erythema (picture 2A-C). Over time, as activity subsides, lesions develop a porcelain white color, variable degrees of palpable sclerosis, and a cigarette paper-like, wrinkled appearance that correlates with epidermal atrophy (picture 1A-C). In individuals with dark skin, established lesions may demonstrate hyperpigmentation and hypopigmentation rather than a porcelain white color (picture 3A-B) [16]. Telangiectasias, follicular keratotic plugs, and hemorrhagic or nonhemorrhagic bullae may also be present (picture 4) [17,18].

Extragenital lichen sclerosus may be asymptomatic but may also be severely disabling. Patients can experience pruritus or painful fissuring, particularly when lesions are located in areas subject to friction or tension, such as the inframammary folds, antecubital fossae, axillae, waist, inguinal creases, and popliteal fossae. Bullae may develop, often with hemorrhage resulting in erosions. In addition, trauma to unaffected skin, such as injury from tight clothing, injections, radiation therapy, herpes zoster, or other factors, may precipitate new lesions (Koebner phenomenon) (picture 5) [11,12,19-21].

Oral lesions — Oral lichen sclerosus is a rare manifestation of this disorder that involves the lips, gingiva, palate, tongue, or buccal mucosa in children and adults [22-25]. Lesions often appear as irregular, white or hypopigmented plaques. Most patients are asymptomatic, but occasional patients experience pruritus, burning, or tightness in affected areas [22,23,26]. Oral lesions may present independently or in association with lichen sclerosus in other sites [27].

Concomitant morphea — Clinical findings consistent with genital or extragenital lichen sclerosus may occur in patients with morphea (picture 6A-B) [28-31]. It is unclear whether the appearance of lesions consistent with lichen sclerosus represents the simultaneous occurrence of two separate disorders or the development of clinical findings that simply resemble lichen sclerosus in lesions of morphea. (See "Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults", section on 'Lichen sclerosus' and "Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults", section on 'Generalized morphea'.)

The frequency of extragenital lichen sclerosus in patients with morphea was evaluated in a retrospective study of 381 adults and 91 children with morphea [29]. Extragenital lichen sclerosus was detected in 19 patients (4 percent). Additional studies are necessary to confirm the frequency of extragenital lichen sclerosus in this population.

HISTOPATHOLOGY — The characteristic pathologic features of lichen sclerosus include (picture 7) [2]:

Epidermal hyperkeratosis with follicular plugging

Epidermal atrophy with flattening of rete ridges

Vacuolization of the basal layer of the epidermis

Marked edema in the superficial dermis (early lesions)

Homogenized collagen in the upper dermis (established lesions)

Lymphohistiocytic infiltrate underlying the zone of homogenized collagen

DIAGNOSIS — The diagnosis of extragenital lichen sclerosus can often be made based upon the clinical appearance of lesions. The finding of atrophic, porcelain-white plaques on the skin is consistent with the diagnosis. When the diagnosis is uncertain, such as when features suggestive of other disorders are also present, a punch biopsy is useful for confirming the diagnosis. (See 'Differential diagnosis' below and "Skin biopsy techniques", section on 'Punch biopsy'.)

Patients with skin findings consistent with extragenital lichen sclerosus should undergo complete examination of the skin, oral cavity, and external genitalia. This allows for assessment of the extent of skin disease and identification of patients with associated oral or genital lichen sclerosus or morphea. The recognition of genital lichen sclerosus is particularly important because of an association with increased risk for squamous cell carcinoma. (See "Vulvar lichen sclerosus", section on 'Associated malignancy'.)

The identification of characteristic histopathologic findings in biopsies of lesions suspicious for extragenital lichen sclerosus is almost always indicative of the diagnosis (picture 7). Although similar histopathologic findings occur in the lichen sclerosus-like variant of chronic graft-versus-host disease, the clinical history facilitates the differentiation of these disorders. (See 'Histopathology' above and "Cutaneous manifestations of graft-versus-host disease (GVHD)", section on 'Diagnosis' and 'Differential diagnosis' below.)

DIFFERENTIAL DIAGNOSIS — A variety of disorders may present with clinical features that resemble extragenital lichen sclerosus. Examples include:

Vitiligo – Vitiligo is characterized by the presence of well-demarcated, depigmented patches on the skin (picture 8A-B). In contrast to extragenital lichen sclerosus, skin texture is normal, without signs of atrophy or sclerosis. (See "Vitiligo: Pathogenesis, clinical features, and diagnosis", section on 'Clinical features'.)

Lichen planus – Extragenital lichen sclerosus may present as violaceous, polygonal papules on the wrists or ankles that closely resemble lichen planus (picture 9A-B) [32,33]. Unlike extragenital lichen sclerosus, lichen planus often is pruritic. A skin biopsy can be used to distinguish between these disorders. (See "Lichen planus".)

Tinea versicolor – Tinea versicolor presents with hypopigmented macules and patches primarily located on the trunk and proximal upper extremities (picture 10A-B). Fine scale is often evident, and a potassium hydroxide preparation will reveal the presence of fungal elements. Signs of cutaneous atrophy and sclerosis are absent. (See "Tinea versicolor (pityriasis versicolor)" and "Office-based dermatologic diagnostic procedures", section on 'Potassium hydroxide preparation'.)

Anetoderma – In anetoderma, loss of elastin in the dermis contributes to reduced skin elasticity and the appearance of circumscribed, 1 to 3 cm areas of depressed, flaccid, wrinkled, or bulging skin (picture 11). Anetoderma may occur as a primary disorder or as a result of a preceding inflammatory process or other skin abnormality. The neck, trunk, and upper extremities are most frequently affected. (See "Anetoderma".)

Cutaneous T cell lymphoma – Like extragenital lichen sclerosus, lesions of patch-stage mycosis fungoides may present with epidermal atrophy manifesting as fine, cigarette paper-like wrinkling (picture 12A-B). Lesions of mycosis fungoides are often erythematous, resembling an eczematous dermatitis. (See "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides", section on 'Skin lesions'.)

Chronic graft-versus-host disease – Patients who have received hematopoietic cell transplants may develop lesions that closely resemble extragenital lichen sclerosus as a manifestation of chronic graft-versus-host disease (picture 13). Knowledge of the patient's transplant history is useful for diagnosis. (See "Cutaneous manifestations of graft-versus-host disease (GVHD)", section on 'Sclerotic manifestations'.)

Morphea – Skin lesions that resemble the classic, atrophic, white patches of extragenital lichen sclerosus may develop in individuals with morphea, an idiopathic inflammatory disorder that causes sclerotic changes in the skin. The relationship between morphea and lichen sclerosus remains unclear. (See 'Concomitant morphea' above.)

MANAGEMENT — There are few data on the efficacy of treatments for extragenital lichen sclerosus. Recommendations for the use of specific agents are primarily based upon case reports, small uncontrolled studies, and data from studies in genital disease.

Overview — Topical medications, phototherapy, and systemic agents have been used for treatment. Topical corticosteroids and phototherapy are the mainstays of treatment for limited and extensive disease, respectively. Systemic therapy is rarely utilized due to the paucity of efficacy data and the potential for severe adverse effects. Systemic agents are primarily reserved for severe disease that fails to respond to other treatments (algorithm 1).

Patients with both morphea and extragenital lichen sclerosus are managed similarly to patients with morphea. (See 'Concomitant morphea' above and "Morphea (localized scleroderma) in adults: Management".)

Decision to treat and patient counseling — Lichen sclerosus is a benign condition, and deferring treatment is a reasonable option for limited skin involvement. Common reasons for initiating treatment include symptomatic, cosmetically distressing, extensive, or progressively worsening disease.

Providing patients with realistic expectations for treatment outcomes is important. Although symptoms and the appearance and texture of lesions can improve with treatment, lesions usually do not completely resolve. In our experience, active inflammatory lesions respond best, while inactive lesions with hypopigmentation and atrophy are unlikely to improve.

Inactive skin disease — If lesions are inactive (eg, stable, atrophic plaques without associated erythema), supportive therapy with emollients and wound care for fissures and erosions is indicated. Bland emollients (eg, petrolatum) and nonstick dressings are useful.

Active limited skin disease — Potent topical corticosteroids are typically used as initial therapy for patients with active limited disease (eg, inflammatory but relatively stable skin disease involving less than 10 percent of the body surface area). This is due to their consistent efficacy in genital lichen sclerosus, reports of clinical experience documenting improvement in patients with extragenital lesions, and the ease of administration and relative safety of these agents. Patients who respond poorly to topical corticosteroids are typically treated with phototherapy (algorithm 1).

Topical corticosteroids — Although the efficacy of topical corticosteroids in extragenital lichen sclerosus has not been formally studied, improvement in genital disease during topical corticosteroid therapy has been reported in a randomized treatment comparison trial as well as uncontrolled and retrospective studies [34-37]. Multiple reports of clinical experience documenting successful treatment of extragenital lichen sclerosus with superpotent topical corticosteroids also support use for this disease [13,17,32,38-42]. Still, clinical experience suggests topical corticosteroids are less consistently effective for extragenital lesions than for genital lesions [13]:

Administration – We typically prescribe once-daily application of a superpotent agent, such as clobetasol propionate (table 1) [13]. If there is no improvement after two months (four to six weeks for intertriginous sites), or if adverse effects are noted, we discontinue treatment and consider alternative therapies. For patients who respond sufficiently within this period, we reduce the frequency of application to two days per week for maintenance therapy. If the response is maintained over the subsequent three months, the frequency of application can continue to be tapered as tolerated.

Adverse effects – Potential adverse effects of topical corticosteroid therapy include local cutaneous atrophy and systemic absorption resulting in suppression of the hypothalamic-pituitary axis. Intertriginous skin and facial skin areas are at greatest risk for corticosteroid-induced atrophy. Hypothalamic-pituitary axis suppression is most likely to occur with the use of high-potency agents or during treatment of large areas. (See "Topical corticosteroids: Use and adverse effects", section on 'Adverse effects'.)

Phototherapy — Phototherapy is our preferred second-line treatment option for patients with limited disease that cannot be effectively treated with topical corticosteroids. (See 'Phototherapy' below.)

Active extensive or spreading skin disease — In the setting of widespread active disease (eg, inflammatory skin disease involving more than 10 percent of the body surface area), application of topical corticosteroids to all lesions is often impractical. Phototherapy is typically used for the initial treatment of active disease in these patients (algorithm 1). We also tend to use phototherapy for more limited disease when patients are actively developing multiple new lesions in an attempt to suppress disease progression. Topical corticosteroids can be used as an adjunctive treatment for the more bothersome lesions during phototherapy. (See 'Topical corticosteroids' above.)

Phototherapy — Phototherapy offers the ability to treat large areas of skin, thereby facilitating the treatment of patients with widespread lesions. The mechanism through which phototherapy improves extragenital lichen sclerosus is unknown but may involve factors such as the stimulation of matrix metalloproteinases and the depletion of proinflammatory cytokines [43].

Whether exposure of uninvolved skin during phototherapy prevents the development of new lesions has not been proven. However, we believe this might be a benefit of phototherapy and use this treatment for patients with progressive development of new lesions.

Several forms of phototherapy have been used for extragenital lichen sclerosus. The largest study involved the treatment of 10 patients with 40 sessions of ultraviolet A1 (UVA1) light at a dose of 20 J/cm2 four times per week [44]. All patients exhibited lesion softening and improvement in pigmentation. In addition, a few case reports have documented marked improvements in disease severity following treatment with narrowband ultraviolet B (UVB) light [39,43]. Psoralen plus ultraviolet A light (PUVA) photochemotherapy involves the administration of an oral or topical photosensitizer (psoralen) prior to light treatment. Case reports document responses to oral PUVA used alone or in combination with topical tacrolimus [45,46] (see "UVA1 phototherapy" and "UVB therapy (broadband and narrowband)" and "Psoralen plus ultraviolet A (PUVA) photochemotherapy"):

Administration We favor UVA1 phototherapy. If UVA1 phototherapy is not available, we suggest use of narrowband UVB phototherapy. PUVA photochemotherapy is a less favorable option due to the additional precautions and side effects associated with this therapy. (See "Psoralen plus ultraviolet A (PUVA) photochemotherapy", section on 'Safety measures' and "Psoralen plus ultraviolet A (PUVA) photochemotherapy", section on 'Drug interactions' and "Psoralen plus ultraviolet A (PUVA) photochemotherapy", section on 'Adverse effects'.)

Phototherapy is usually given two to five times per week. Our typical dose range for UVA1 is 60 to 100 J/cm2, and we administer narrowband UVB according to the standard regimen. (See "UVA1 phototherapy", section on 'Dosimetry' and "UVB therapy (broadband and narrowband)", section on 'Dosimetry and treatment protocols'.)

Improvement from phototherapy (transitioning of erythema to postinflammatory hyperpigmentation and reduction in the development of new lesions) is usually evident within approximately 15 treatments. Our usual treatment course is 40 to 50 treatments. We typically stop treatment at this point. Treatment success is characterized by resolution of erythema and the cessation of both lesion expansion and new lesion formation. We continue to follow patients closely after the end of treatment. Responders often exhibit continued improvement in skin texture over the subsequent six months after treatment.

Adverse effects – Adverse effects of phototherapy include burning or blistering of skin and potential increased risks for premature skin aging and cutaneous malignancy. The risk for malignancy appears to be highest with long-term PUVA treatment. (See "UVA1 phototherapy", section on 'Adverse effects' and "UVB therapy (broadband and narrowband)", section on 'Short- and long-term adverse effects' and "Psoralen plus ultraviolet A (PUVA) photochemotherapy", section on 'Adverse effects'.)

Refractory extensive skin disease — Systemic immunosuppressive therapy is rarely used for the management of extragenital lichen sclerosus. It is a treatment option for extensive disease in patients who have failed to respond to topical corticosteroids and phototherapy or who are unable to receive phototherapy (algorithm 1).

We typically treat these patients with a combination of systemic glucocorticoids and methotrexate. Systemic glucocorticoids are given during the first few months of treatment to induce improvement while awaiting the slower onset of action of methotrexate. Following the achievement of a satisfactory response (cessation of disease progression, resolution of erythema, and lesion improvement), the systemic glucocorticoid is tapered and discontinued while methotrexate is continued. (See 'Methotrexate and systemic glucocorticoids' below.)

We generally reserve initial treatment with methotrexate alone for patients who have slow disease progression or who are poor candidates for systemic glucocorticoid therapy. Improvement of extragenital lichen sclerosus with methotrexate alone has been reported in case reports and case series [47,48].

Methotrexate and systemic glucocorticoids — The efficacy of glucocorticoids plus methotrexate is supported by a retrospective study of seven patients with progressively worsening extragenital lichen sclerosus that failed to respond to a potent topical corticosteroid (all patients) and phototherapy (six patients) [49]. Patients were treated with 15 mg per week of oral methotrexate and 1000 mg of intravenous methylprednisolone sodium succinate on three consecutive days per month. Treatment was continued for 6 months in six patients and for 10 months in one patient who had a delayed response to treatment. A significant decrease in a nonvalidated clinical disease severity score was detected at the end of treatment. In most patients, signs of improvement were first noted after three months of therapy:

Administration – Our typical treatment regimen includes 10 to 20 mg of oral methotrexate administered once per week plus oral prednisone (1 mg/kg per day or 40 to 60 mg per day). Folic acid supplementation (1 mg per day) is typically given during methotrexate therapy to reduce risk for some side effects of methotrexate. Because absorption of oral methotrexate may be reduced at higher doses [50], we often give methotrexate subcutaneously rather than orally when doses exceed 15 mg per week or when patients fail to respond adequately to oral therapy. Alternatively, we split the total dose of oral methotrexate into a morning and an evening dose or instruct the patient to take the second half of the dose on the following day. (See "Use of methotrexate in the treatment of rheumatoid arthritis", section on 'Folic acid supplementation' and "Use of methotrexate in the treatment of rheumatoid arthritis", section on 'Pharmacology' and "Use of methotrexate in the treatment of rheumatoid arthritis", section on 'Parenteral therapy'.)

Regimens for initiating methotrexate vary. The author typically begins with a dose of 10 to 15 mg per week; some clinicians begin with a lower dose followed by dose increases every one to two weeks in the absence of evidence of significant toxicity. (See "Methotrexate: Drug information".)

Patients are clinically assessed for a satisfactory response to methotrexate and prednisone (cessation of disease progression, resolution of erythema, and lesion improvement) after receipt of 10 to 15 mg of methotrexate per week for one month. If this endpoint has not been reached, we increase the dose of methotrexate to 17.5 to 20 mg per week, continue 1 mg/kg of prednisone per day, and reassess after an additional month. In our experience, most patients achieve satisfactory improvement within this period and tapering of prednisone can begin. We typically taper and discontinue prednisone slowly over the course of two months while continuing the same dose of methotrexate. The optimum duration of therapy with methotrexate is not established. After cessation of prednisone, we generally maintain patients on the lowest effective dose, tapering over time as tolerated.

Our patients who have not achieved a satisfactory response to methotrexate and prednisone within two months continue treatment for an additional month. If the response remains unsatisfactory, this treatment is unlikely to be effective and can be discontinued. (See "Glucocorticoid withdrawal".)

Adverse effects – A variety of potential adverse effects are associated with treatment with methotrexate and glucocorticoids, and close monitoring for toxicity is needed. Gastrointestinal distress is a common dose-related side effect of oral methotrexate therapy that may occur less frequently when methotrexate is given as parenteral therapy. We typically attempt subcutaneous therapy in patients who develop gastrointestinal side effects during oral therapy. (See "Major side effects of low-dose methotrexate" and "Use of methotrexate in the treatment of rheumatoid arthritis", section on 'Parenteral therapy'.)

Use of methotrexate is contraindicated in pregnancy, and relative contraindications for methotrexate therapy include alcohol abuse and pre-existing liver disease. Dose adjustments of methotrexate are necessary in patients with renal insufficiency. The adverse effects of systemic glucocorticoids are reviewed separately. (See "Major side effects of low-dose methotrexate" and "Major side effects of systemic glucocorticoids".)

Oral disease — Treatment of oral lesions of lichen sclerosus often is not required and is usually only initiated in the setting of cosmetic concerns or symptoms. Treatments that have been associated with improvement in this disorder include superpotent or medium-potency topical corticosteroids [24,51], intralesional corticosteroid injections (10 mg/mL triamcinolone acetonide) [52], topical tacrolimus [27], topical pimecrolimus [26], topical corticosteroids followed by topical testosterone [53], surgical excision [23], oral colchicine [23], and oral griseofulvin [54]. In our opinion, topical or intralesional corticosteroids and topical calcineurin inhibitors are reasonable first-line treatments. We typically involve an oral maxillofacial surgeon or dentist in the care of these patients.

Morphea with lichen sclerosus features — Patients with morphea who present with coexisting lesions consistent with extragenital lichen sclerosus should be managed similarly to other morphea patients (algorithm 1). (See 'Differential diagnosis' above and "Morphea (localized scleroderma) in adults: Management" and "Juvenile localized scleroderma", section on 'Treatment'.)

Other therapies — Although topical tacrolimus is an accepted treatment for genital lichen sclerosus, it appears less likely to be effective in extragenital lichen sclerosus. In a prospective uncontrolled study in which 16 patients with lichen sclerosus applied tacrolimus 0.1% ointment twice daily to affected areas, a partial or complete response was observed in 9 of 10 patients with anogenital lesions but only 1 of 6 patients with extragenital lesions [55]. The solitary patient with extragenital lichen sclerosus who improved achieved only a partial response. Topical pimecrolimus, which has also been used successfully for vulvar disease [34], was not effective for extragenital lichen sclerosus in a case report [56]. We typically do not use topical tacrolimus or pimecrolimus for treatment of extragenital lichen sclerosus. (See "Vulvar lichen sclerosus", section on 'Topical calcineurin inhibitors'.)

Local treatments reported to be effective in small numbers of patients include topical calcipotriol [57], pulsed dye laser [58,59], photodynamic therapy [59], and carbon dioxide laser vaporization [60]. Additional studies are necessary to determine the efficacies of these treatments for extragenital disease.

The efficacy of other systemic therapies used in genital lichen sclerosus (eg, oral retinoids, cyclosporine) for extragenital disease is unknown. Improvement in extragenital lichen sclerosus complicated by bullae and ulceration during hydroxychloroquine therapy was reported in one patient [61].

PROGNOSIS AND FOLLOW-UP — The natural history of extragenital lichen sclerosus has been poorly characterized. In our experience, the disease process often continues over the course of years, with unpredictable periods of exacerbation and stability.

Periodic follow-up is indicated given that some patients who present with isolated plaques progress to extensive disease. Although there is a strong link between genital lichen sclerosus and the development of genital squamous cell carcinoma, reports of the development of squamous cell carcinoma in sites of extragenital lichen sclerosus are rare [62,63]. (See "Vulvar lichen sclerosus", section on 'Associated malignancy'.)

The possibility of increased risk for breast cancer in female patients with lichen sclerosus has been raised based upon the findings of a small, retrospective study [64]. However, more data are necessary to confirm this finding and to determine whether changes to breast cancer screening protocols would be beneficial for patients with lichen sclerosus.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Lichen sclerosus".)

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: Lichen sclerosus (The Basics)")

SUMMARY AND RECOMMENDATIONS

Extragenital lichen sclerosus is an uncommon disorder that may involve the skin or oral cavity. The disorder occurs more frequently in women than in men, and the occurrence in children is rare. (See 'Epidemiology' above.)

Early active skin lesions of extragenital lichen sclerosus often present as flat-topped, polygonal papules that may coalesce into large plaques (picture 2A-C). Inactive lesions frequently develop a porcelain white color, a finely wrinkled surface, and variable degrees of sclerosis (picture 1A-C). In individuals with dark skin, lesions may be hyperpigmented or hypopigmented (picture 3A-B). Oral lichen sclerosus presents as irregular, white or hypopigmented plaques on the lips or in the oral cavity. (See 'Clinical manifestations' above.)

A variety of other skin disorders share clinical features with extragenital lichen sclerosus. When the diagnosis is uncertain based upon clinical examination, a skin biopsy should be performed. (See 'Diagnosis' above and 'Differential diagnosis' above.)

Extragenital lichen sclerosus is a benign condition, and treatment may be deferred by patients who do not desire intervention. Common indications for proceeding with therapy include symptomatic, cosmetically distressing, extensive, or progressively worsening disease. (See 'Decision to treat and patient counseling' above.)

For patients with limited extragenital lichen sclerosus who desire treatment, we suggest treatment with a superpotent topical corticosteroid, such as clobetasol propionate (Grade 2C). (See 'Topical corticosteroids' above.)

For patients with widespread or progressive lesions, in whom application of topical therapy is impractical, or for patients who fail treatment with a superpotent topical corticosteroid, we suggest treatment with ultraviolet A1 (UVA1) phototherapy (algorithm 1) (Grade 2C). If UVA1 phototherapy is not available, patients may be treated with narrowband ultraviolet B (UVB) phototherapy. (See 'Phototherapy' above.)

For patients with severe disease that fails to respond to topical corticosteroids and phototherapy, we suggest treatment with a combination of methotrexate and systemic glucocorticoids (algorithm 1) (Grade 2C). Other systemic treatments that have been reported to be effective in single patients include methotrexate alone and hydroxychloroquine. (See 'Refractory extensive skin disease' above.)

Wound care is important in the presence of fissures and erosions and should include bland emollients (eg, petrolatum) and nonstick dressings. (See 'Inactive skin disease' above.)

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Topic 15838 Version 15.0

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