Vulval cicatricial pemphigoid may mimic lichen sclerosus

Abstract

We report two patients with vulval signs suggestive of lichen sclerosus. This diagnosis was never substantiated histologically and microscopic appearances were non-specific. In both cases the development of lesions at other cutaneous and mucosal sites alerted us to the possibility that the autoimmune bullous disease, cicatricial pemphigoid, accounted for the entire clinical picture. Lichen sclerosus and cicatricial pemphigoid are distinctive conditions which clinically are usually readily differentiated on cutaneous surfaces. When the site of presentation is on the vulva the clinical picture may become obscure and this can lead to diagnostic confusion. It is important to establish the correct diagnosis as this predicts the clinical course and prognosis in each case.

Developing an interdisciplinary consultation service for vulvar disorders

“…Monitoring should be performed at least annually if not more often [5, 6]. Massive erosive changes affecting the vulva should raise suspicion not only of lichen planus or Behçet disease but also of blistering autoimmune skin diseases [7, 8]. An additional biopsy from the unaffected mucosa should be taken for direct immunofluorescence (DIF).…”

Circulating basement membrane zone antibodies are found in lichen sclerosus of the vulva

“…Lichen sclerosus is an interface dermatitis with specific histopathological changes and abnormalities in expression of basement membrane zone (BMZ) antigens 2 . There are similarities clinically and histologically between LS and lichen planus 7 and between LS and mucous membrane pemphigoid 8 . Basement membrane zone antibodies are commonly found in mucous membrane pemphigoid and have been recently shown to occur in women with erosive lichen planus of the vulva (S Cooper, pers.…”

Mucous Membrane Pemphigoid of the Vulva

“…Lichen sclerosus, erosive lichen planus, pemphigus vulgaris, and mucous membrane pemphigoid can have very similar clinical presentations. Although all of these disorders may respond to topical superpotent corticosteroids, 4 this does not obviate the need for a correct diagnosis because these diseases may affect different organ systems and have different prognoses. 3,4,6 In 1999, the First International Conference on Mucous Membrane Pemphigoid established the diagnostic criteria for mucous membrane pemphigoid: blisters on mucous membranes, which must be examined by direct immunofluorescence microscopy to demonstrate continuous deposits of any one or a combination of IgG, IgA, or C3 along the basement membrane zone.…”

“…Although all of these disorders may respond to topical superpotent corticosteroids, 4 this does not obviate the need for a correct diagnosis because these diseases may affect different organ systems and have different prognoses. 3,4,6 In 1999, the First International Conference on Mucous Membrane Pemphigoid established the diagnostic criteria for mucous membrane pemphigoid: blisters on mucous membranes, which must be examined by direct immunofluorescence microscopy to demonstrate continuous deposits of any one or a combination of IgG, IgA, or C3 along the basement membrane zone. 1 Although the histopathologic changes of a biopsy and indirect immunofluorescence of a patient's serum can be useful to help make the diagnosis of mucous membrane pemphigoid, results of these tests can be nonspecific and, therefore, are not part of the diagnostic criteria for mucous membrane pemphigoid.…”

“…3,4 Differentiating between these 2 entities is important because 3-5% of women with vulvar lichen sclerosus will develop carcinoma, 8 whereas vulvar carcinoma associated with mucous membrane pemphigoid has not been reported.…”