IgG BULLOUS PEMPHIGOID WITH ANTIBODIES TO IgD, DERMAL BLOOD VESSELS, ECCRINE GLANDS AND THE ENDOMYSIUM OF MONKEY ESOPHAGUS

Abstract

Context: Bullous pemphigoid is mediated by autoantibodies primarily targeting two structural proteins of basement membrane hemidesmosomes, BP180 (BPAG2; collagen XVII) and BP230 (BPAG1). Case Report: A 70-year-old Caucasian male patient was evaluated for a seven day history of multiple itching, erythematous blisters on his extremities. Biopsies for hematoxylin and eosin examination, direct immunofluorescence and indirect immunofluorescence (including salt split skin analysis) were performed. Results: Hematoxylin and eosin examination demonstrated a subepidermal blister. Within the blister lumen, numerous eosinophils and lymphocytes were noted. Direct and indirect immunofluorescence revealed linear deposits of IgG, Complement/C3 and fibrinogen at the basement membrane zone of the skin and surrounding selected dermal blood vessels and sweat glands. Positive intracytoplasmic staining for anti-human IgD was noted in most of the epidermis, as well as surrounding some dermal blood vessels. Indirect immunofluorescence utilizing monkey esophagus substrate demonstrated strong positivity within the endomysium for IgG antibodies. Conclusion: We report a unique case of bullous pemphigoid with reactivity to eccrine sweat glands, and selected dermal blood vessels. In addition, the observed reactivity of anti-human IgD, and of IgG to monkey esophagus endomysium warrant further investigation.

Keywords

• bullous pemphigoid
• eccrine glands
• IgD
• blood vessels
• autoantibodies