Vesicular pemphigoid with circulating autoantibodies against 230‐kDa and 180‐kDa proteins, and additional autoantibodies against 97‐kDa and 45‐kDa proteins

Abstract

The vesicular variant of bullous pemphigoid is a clinical entity in which the principal type of lesions are multiple small tense vesicles in a symmetric distribution, instead of the classical large, more randomly distributed large bullae. We describe a 62-year-old female who developed a vesicular variant of bullous pemphigoid, with intensely pruritic vesiculopapular eruptions and erythematous irregularly outlined patches confluent on the face, neck, trunk, extremities and oral mucosa. Direct immunofluorescence revealed a linear deposition of IgG and C3 at the basement membrane zone of the skin, and indirect immunofluorescence detected circulating IgG autoantibodies reacting with antigens located on the epidermal side of skin split with 1 M NaCl. Indirect immunogold electron microscopy revealed IgG deposition at the underneath membrane of the basal cells and in the lamina lucida. In addition to the 230-kDa and 180-kDa bullous pemphigoid antigen, immunoblot analysis also demonstrated the presence of IgG antibodies reactive with 97-kDa and 45-kDa protein. Treatment with systemic corticosteroid, minocycline and antihistamines plus topical steroid resulted in moderate improvement. A few vesicles, however, continued to appear intermittently during the past year of treatment.

Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects

“…2. Patients present with numerous small pruritic tense vesicles often on an urticarial or erythematous base symmetrically distributed on extremities and trunk, as seen in dermatitis herpetiformis [45][46][47]. Cases are reported mostly in elderly people [45] and sometimes in children as well [48,49].…”

“…Cases are reported mostly in elderly people [45] and sometimes in children as well [48,49]. Therapies using systemic corticosteroid combined with minocycline are usually effective [45].…”

The Clinical Diagnosis of Autoimmune Bullous Diseases

“…Göz, burun, farenks, özofagus ve anogenital bölge mukozas› nadiren etkilenir 37 . Klasik BP lezyonlar›n›n yan› s›ra lokalize BP, veziküler BP, polimorfik BP, palmoplantar bölgede dishidroziform BP, dermatitis herpetiformis benzeri BP, prurigo nodülaris benzeri ve eritrodermik BP de tan›mlanm›flt›r [38][39][40][41][42][43] . Bunlar›n d›fl›nda yak›n zamanda tan›mlanan anti-p200 pemfigoid, anti-p105 pemfigoid ve anti-p450 pemfigoid olgular› da bulunmaktad›r 44,45 .…”

Two cases of dyshidrosiform pemphigoid with different presentations

“… 8 as small tense vesicles similar to dermatitis herpetiformis. In the sera of patients with VP and those with DP, autoantibodies to bullous pemphigoid antigen (BPAG)1 and BPAG2 are often identified, but heterogeneous autoantibodies, such as the 45 and 97‐kDa molecules, have been also found in the sera of patients with VP 9 . We used the sera of patient 1 to probe antigenic molecule(s) by Western blotting and it reacted with 60 and 180 kDa antigens (data not shown).…”