LinearIgA Dermatosis With Systemic Lupus Erythematosus

B. Chelly, I. Chelly, A. Zehani, H. Azzouz, F. Abbes, I. Zaraa, M. Mokni, S. Haouet, N. Kchir

Abstract


Cutaneous involvement in systemic lupus erythematosus (SLE) is heterogeneous. In addition, the skin lesions in SLE patients are specific and nonspecific. In the nonspecific bullous lesion, linear IgA bullous dermatosis(LABD), the most common bullousdermatosis in children, is not considered as a possible association. We describe a patient with LABD, who developed characteristics of SLE. This association is very rare and might be considered in the spectrum of the nonspecific bullous lesions associated to SLE.
We describe a patient with LABD, who developed characteristics of SLE.
A18-year-old girl presented with fatigue and arthralgia. She was diagnosed as having SLE and was hospitalized. Laboratory studies revealed anemia, lymphopenia, and low complement levels. Antinuclear anti-bodies (ANA; 1/800, speckled pattern) and double strain antidesoxyribonucleic acid (DNA) test were positive. The direct immunofluorescent examination disclosed IgG, IgM, C3 and C1q deposit along the basement membrane. The patient was treated by Nivaquine with a good response. Six months later, the patient was seen with itching erythema and conglomerate vesicles arising on normal-appearing skin of lower abdomen, extremities and in the perioral region. The biopsy showed a subepidermal blister with infiltration of PNN and the immunofluorescence made in this tissue showed linear IgA deposit in dermoepidermic junction. Based on these results, the patient was diagnosed as having SLE complicated by linear IgA bullous dermatosis.
Linear IgA bullous dermatosis (LABD), also known as linear IgA disease, is a rare, idiopathic or drug-induced autoimmune blistering disease. The association with SLE is very rare and might be considered in the spectrum of the nonspecific bullous lesions associated to SLE.

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