Lúpus eritematoso sistêmico bolhoso associado à doença de Kikuchi-Fujimoto : relato de caso como um desafio diagnóstico

Abstract

Bullous lupus is a rare systemic dermatosis, which is caused by antibodies against type VII collagen of the dermoepidermal junction. Although rarely, Kikuchi-Fujimoto disease can be associated with lupus and it is a histiocytic necrotizing lymphadenitis. Man, 22 years, presenting vesicolobullous lesions on the oral and genital mucosa, abdomen, buttocks, armpits and painful lymphadenomegaly, bicytopenia, proteinuria, ANF considerably positive and serositis. Skin histopathology was compatible with dermatitis herpetiformis, but direct immunofluorescence showed an IgG and IgA linear pattern on the basal membrane zone. Lymph node biopsy showed a CD8+ and CD68+ linfohistiocytic infiltrate surrounding a coagulative necrosis zone. Those clinical findings allowed the diagnosis of bullous lupus with Kikuchi-Fujimoto disease and the patient had a satisfactory response to the therapy.