Bullous pemphigoid is an autoimmune disease of the skin characterized by the production of antibodies directed at structures of the basement membrane zone (BMZ) leading to subepidermal blisters. Several causative triggers have been described in the literature, among them UV light. Here, we report on a 73-year-old Caucasian female with disseminated morphea who developed blisters on her extremities after receiving whole-body UVA-1 phototherapy. The initial differential diagnosis of a phototoxic versus photoallergic reaction was ruled out as the lesions continued to spread after discontinuation of phototherapy. Histological and direct immunofluorescence examination showing a subepidermal blister and linear IgG deposits along the BMZ along with detection of circulating anti-BMZ antibodies led to the diagnosis of bullous pemphigoid. Immunosuppressive therapy resulted in regression of all blisters. After ruling out other possible causes, such as neoplasias or drugs, we conclude that UVA-1 has to be regarded as the most likely trigger of the disease.
The specific and reproducible enrichment of PLA2-binding cells provides a new tool for the analysis and monitoring of effector cells in bee-venom-allergic patients with immediate-type hypersensitivity.
Zusammenfassung: Herpes gestationis (Pemphigoid gestationis) ist eine seltene Autoimmundermatose in der Schwangerschaft, die durch eine subepidermale Blasenbildung charakterisiert ist. Die Blasen entstehen durch Bindung von Autoantikörpern, die bei der Mehrzahl der Patienten gegen das Bullöse Pemphigoid Antigen 2 (180 kD) und dessen Domäne NC16A gerichtet sind. Herpes gestationis ist nach der Geburt gewöhnlich eine selbstlimitierte Erkrankung. Die durchschnittliche Dauer beträgt 12 Wochen nach Entbindung bei Erstmanifestation und 31 Wochen bei wiederholter Erkrankung. Wir berichten über eine Patientin mit ungewöhnlich schwerem Verlauf hinsichtlich Symptomatik und Dauer sowie fehlendem Ansprechen auf verschiedene immunsuppressive Therapieregime.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.