Dermatose por IgA e IgG linear: relato de caso com boa resposta terapêutica à dapsona e ao micofenolato mofetil

Abstract

Abstract:A 21-year-old female presenting linear IgA and IgG disease initially responded well to dapsone therapy. However, the treatment with dapsone was withdrawn due to severe anemia induced by malaria, which led to worsening of the clinical picture. Although prednisone and methylprednisolone were tried, the patient responded only to the association of dapsone and mycophenolate mofetil. Keywords: Dapsone; Immunoglobulin A; Immunoglobulin G; Receptors, IgGResumo: Relata-se o caso de pacien te femi ni na, de 21 anos, com der ma to se por IgA e IgG linear. Inicialmente, a res pos ta clí ni ca foi favo rá vel à dap so na. Após a inter rup ção desta medi ca ção, por crise de ane mia sin to má ti ca, pre ci pi ta da por malá ria, houve piora da doen ça, ape sar da uti li za ção da pred ni so na e pul so te ra pia com metilpred ni so lo na. A rein tro du ção da dap so na, asso cia da ao mico fe no la to mofe til, pos si bi li tou o con tro le da enfermi da de. ©2011 by Anais Brasileiros de Dermatologia INTRO DUC TIONLinear IgA der ma to sis (LAD) is defi ned as an acqui red autoim mu ne bul lous disea se, cha rac te ri zed by linear depo si tion of IgA along the base ment membra ne zone (BMZ). 1,2 Despite the dif fi cul ties in dis tinguis hing sube pi der mal bul lous disea ses, as of 1979 LAD has been dif fe ren tia ted from her pe ti form der mati tis. Since then, bul lous disea ses with sube pi der mal clea va ge and linear depo si tion of IgA along the BMZ have been auto ma ti cally clas si fied as LAD. 3,4 In 1998, Honokik and cols demons tra ted the pre sen ce of IgG against cir cu la ting epi der mal anti gens in LAD patients. 5 Subsequent reports cor ro bo ra te the findings of this group. 3,[6][7][8] The pre sen ce of simul ta neous linear depo si tion of IgA and IgG was fun da men tal for the con cep tion of a new cli ni cal-patho lo gi cal entity, IgA and IgG linear der ma to sis (LAGD). 9,10 However, the mag ni tu de and cli ni cal rele van ce of this clas si fi ca tion remain con trover sial, as there seems to be no dif fe ren ces in the derma to lo gi cal pre sen ta tion and the ra peu tic spec trum of both disea ses. The rea son for such simi la rity has been attri bu ted to the fact that IgA is an anti body with very hete ro ge neous tar get anti gens, against seve ral BM com po nents. A pos si ble mecha nism of inter mo le cu lar expan sion of epi to pes could be res pon si ble for the autoim mu nity exten sion of BP180 anti gen to the

Management Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review

“…As lymphocytes utilize the de novo pathway for purine synthesis, blocking the T and B lymphocyte response leads to reduced antibody production and resolution of the autoimmune blistering disease. Other than for prevention of solid organ transplant rejection, MMF has been used to treat autoimmune diseases such as psoriasis, bullous pemphigoid, pemphigus vulgaris, and CBDC or LABD patients [42][43][44][45][46][47]. Reported dosages used in the treatment of LABD are 2 g/day for 5 to 14 weeks, while in CBDC, the dosage has been reported in the range of 155 to 310 mg/m 2 daily for eight months [42,43].…”