Lupus erythematosus profundus of the scalp in five children

Abstract

Objective To investigate clinical and pathological features of lupus erythematosus profundus(LEP)of the scalp in children. Methods A retrospective study was carried out on 5 children with LEP. The clinical and histopathological features, treatment and prognosis of LEP were analyzed. Results The 5 children with LEP included 2 boys and 3 girls with a median age at onset of 5 months(range, 2-38 months)and a median clinical course of 15 months(range, 4-72 months). Clinically, the patients presented with arc-shaped or circular purple atrophic plaques on the scalp complicated by alopecia. The occipitalia and tempora were the most commonly involved sites. Antinuclear antibodies(ANA)and extractable nuclear antigens(ENAs)were negative in all the patients. Main histopathological changes were hyaline degeneration of the fat, mucin deposition and local aggregation of lymphocytes in fat lobules. Of the 5 patients, 2 were treated with oral prednisone(1.5-2 mg/kg/day), 1 with oral hydroxychloroquine(5 mg/kg/day), 1 with oral prednisone(1.5 mg/kg/day)combined with hydroxychloroquine(5 mg/kg/day), and another 1 with topical halometasone cream and 0.03% tacrolimus ointment. Lesions were remissed after 2-3 months of treatment, and subsided with growth of new hairs after 6 months. No recurrence was observed during a 1.5-year follow-up. Conclusion Prednisone and hydroxychloroquine are markedly effective for LEP, and pediatric patients with LEP may be treated by topical high-potency glucocorticoids and calcineurin inhibitors. Key words: Panniculitis, lupus erythematosus; Scalp dermatoses; Child; Skin manifestations; Pathologic processes; Treatment outcome