Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child

Generalized Lupus Panniculitis and Antiphospholipid Syndrome in a Patient Without Complement Deficiency

“…Excluding a case of LEP associated with neonatal lupus (3) and a case of pancreatic panniculitis in a patient with lupus erythematosus (4), we have found four reported cases of generalized LEP (i.e., affecting more than one anatomic site) in children under age 13 in the English literature. In the only case with extensive complement studies, the patient was found to have hereditary deficiencies of C2 and C4 (5). In the remaining three cases, complement studies were either not reported (6,7) or the CH50 was normal (8).…”

Panniculitis in Childhood

“…Two children had lupus erythematosus (LE): one had discoid LE (DLE) (15) while the second had systemic LE (SLE) with pancreatitis that appeared to have precipitated the onset of panniculitis (8). A third child with genetic partial deficiency of C2 and C4 developed LE pannicuiitis after primary acquired toxoplasmosis (13). Our patient no.…”

“…The lesions occurred most frequently on the thighs, buttocks, arms, and legs. The neck and/or face was involved in eight patients (10,11,13,15,17; pts, no. 1, 2), the cheeks in three (13,16; pt, no.…”

Update on management of connective tissue panniculitides

“…The cause of lupus panniculitis is poorly understood. Some patients have reduced levels of C4 suggesting an underlying genetic component (20,22,23). Tuffanelli has described lupus profundus in two sisters and in a patient with four first degree family members with lupus erythematosus (12).…”

“…The use of chloroquine as monotherapy at doses of 250-500 mg a day has been reported (12,16,23,30). Chloroquine is also dosed by ideal body weight and dosages should not exceed 3.5 mg/kg/day.…”