Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis - Brazilian Society of Dermatology

Abstract

Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors’ experience, aiming at the treatment orientation of these diseases in Brazil. Dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and can be controlled with a gluten-free diet and dapsone. On the other hand, linear IgA bullous dermatosis arises spontaneously or is triggered by drugs, and can be controlled with dapsone, but often requires the association of systemic corticosteroids and eventually immunosuppressants.

Successful treatment of a case of idiopathic linear IgA bullous dermatosis with oral sulfasalazine

“…LABD is also associated with the expression of continuous linear IgA deposits in the basement membrane zone (BMZ). Here, we present a LABD case successfully treated with oral sulfasalazine (Vale, Dimatos, Porro, & Santi, ) in a 17‐year‐old boy.…”

“…It was further established histologically by identifying linear IgA deposits on the BMZ and the absence of other immunoglobulins on the DIF. The patient received enteric‐coated sulfasalazine tablets with a dosage of 40–60 mg kg −1 day −1 along with oral sodium bicarbonate to prevent the formation of crystals in the urinary tract (Li, Zhang, Xu, Chen, & Li, ; Vale et al, ). Rash and severe pruritus symptoms were controlled in 10 days after oral sulfasalazine treatment.…”

“…Dapsone is often considered as the first‐line therapy for LABD (Ohata, Ishii, Koga, & Nakama, ; Vale et al, ). Unfortunately, this drug is not readily available in China.…”

“…Physical examination suggested annular or polycyclic erythema and urticarial-like plaques, along the edge of the lesions. Normal skin with characteristic blistering often called "string-of-pearls" appearance was noted specifically on his arms and trunk (Figure 1a with oral sodium bicarbonate to prevent the formation of crystals in the urinary tract (Li, Zhang, Xu, Chen, & Li, 2018;Vale et al, 2019). Rash and severe pruritus symptoms were controlled in 10 days after oral sulfasalazine treatment.…”

“…Dapsone is often considered as the first-line therapy for LABD (Ohata, Ishii, Koga, & Nakama, 2017;Vale et al, 2019).…”