Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

Abstract

Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.

Figure 1

A-C: Numerous erythemas with papules, vesicles, tense blisters, erosions, and crusts were observed on the entire body. D: Numerous milia during recovery

Figure 2

A - A giant decubitus ulcer was presented on sacrococcygeal area. B - Decubitus ulcer during recovery

Figure 3

Histopathology of the skin lesion revealed subepidermal blisters (Hematoxylin & eosin, X10) and infiltration of numerous eosinophils in the upper dermis (Hematoxylin & eosin, X40)

Figure 4

Direct immunofluorescence showed linear IgG and C3 deposits along the subepidermal basal membrane zone