General Medical Information

Vesicobullous Diseases

Wilberto Nieves-Neira
Bhagirath Majmudar
Ira R. Horowitz

Vesicles and bullae are the characteristic lesions for this group of dermatologic conditions. Erosions of mucous membranes and the vulva also are common. Immunobullous diseases are caused by autoimmune reactions to antigens in the epidermis and basal membrane leading to acantholysis and blistering. These are rare in childhood and often are misdiagnosed. Diagnosis is based on skin biopsies and direct immunofluorescence.

Aphthous ulcers of the oral and genital mucosa are shallow erosions with a grayish-yellow base that vary in size from 1 to 10 mm. The lesions usually are painful. Recurrence of the ulcers is common, followed by resolution within 7 to 10 days. Vulvar and oral erosions may be treated with topical steroids. Clobetasol 0.05% cream is effective for vulvar lesions in adults.

Chronic bullous disease of childhood (linear IgA disease) is characterized by linear IgA deposition at the basement membrane zone. The condition usually presents by age 10 with bullae on the groin, perineum, and buttocks. Mucosal involvement of the eyes, nose, and vulva occurs in 80% of patients. Clusters of bullae are the most common presentation in the genital area and may be confused with herpes infection or sexual abuse. Skin bullae may resemble traumatic burns. Oral sulfapyridine over a period of 1 to 2 years produces remission in over 60% of children. Resistant cases require the use of systemic antibiotics.

Childhood cicatricial pemphigoid may be considered a more severe form of linear IgA disease because it has the same immunofluorescence findings. The mucosal involvement is extensive. Lesions begin as blisters in the mouth, eyes, nose, and genital area with rapid erosion. Vulvar and ocular scarring are prominent. Anterior fusion of the labia or stenosis of the introitus may occur. Treatment is similar to that for linear IgA disease, but remission is rare. Therefore, persistence into adult life is common.

Bullous pemphigoid is a rare occurrence in children but several cases have been reported. Childhood bullous pemphigoid is characterized by linear deposition of IgG and C3 at the basement membrane. Pruritus may be a presenting feature, but mucosal lesions may be asymptomatic. Initially the lesions are plaque-like but progress to widespread, tense bullae. The spectrum of disease ranges from localized lesions to extensive vulvar scarring. Treatment starts with oral prednisone. Dapsone or sulfapyridine can be added. Most children achieve remission within 1 year. Vulvar involvement may be the only manifestation of the condition. Response to topical steroids is good in patients with isolated vulvar lesions. More recently, IgG autoantibodies targeting the C-terminus of collagen XVII/BP180 have been described. This finding suggests that localized vulvar pemphigoid of childhood is a variant of bullous pemphigoid. However, differentiation between the scarring and the nonscarring course of the disease is not possible with the present diagnostic markers. Cicatricial pemphigoid is another rare condition in children. Scarring of the vulva may be prominent. The condition may be confused with lichen sclerosus. Some authors have proposed that cicatricial pemphigoid is a severe form of chronic bullous disease of childhood.

Pemphigus vulgaris is extremely rare in childhood. Adult females with pemphigus vulgaris have vulvar involvement. Rare cases of pemphigus vulgaris manifesting with oral mucosa and vulvar erosion have been reported in adolescence in association with angiofollicular lymph node hyperplasia (Castleman’s disease).