Clinical and prognostic analysis of 183 cases of pediatric cutaneous mastocytosis / 中华皮肤科杂志

ABSTRACT

Objective To characterize clinical features and prognosis of pediatric cutaneous mastocytosis. Methods Clinical data on 183 cases of pediatric cutaneous mastocytosis were collected and retrospectively analyzed. Some patients were followed up. Results Of the 183 patients, 136 (74.3%)had urticaria pigmentosa, 43 (23.5%) mastocytoma, 4 (2.2%)diffuse mastocytosis. The first attack of mastocytosis occurred at birth in 21 (48.8%)patients with mastocytoma and 35(25.7%)patients with urticaria pigmentosa, within 6 months after birth in 17(39.5%)patients with mastocytoma and 78(57.3%)patients with urticaria pigmentosa, and within 2 years after birth in 179(97.8%)out of the 183 patients. Of 33 patients with detailed description of symptoms, 10 had concomitant symptoms, which were flushing in 9 patients. Forty-five patients were followed up for 3 - 6 years (average, 4 years). The follow-up showed that skin lesions completely regressed in 1 patient with urticaria pigmentosa at 11 years of age, partially regressed in 18 patients. Lesions regressed completely at 8 years of age in 1 patient with mastocytoma, and subsided within 1 year after skin biopsy in 7 patients. Oral antihistamines could control the symptoms of mastocytosis, such as flushing, whealing and blistering, and oral glucocorticoids could effectively control the recurrence of generalized blisters and bullae in patients with diffuse mastocytosis. Conclusions Urticaria pigmentosa appears to be the most common type of cutaneous mastocytosis in children, followed by mastocytoma. Mastocytoma occurs most frequently at birth, while urticaria pigmentosa within 6 months after birth. Oral antihistamines may control inflammatory mediator-related symptoms. Serious diffuse mastocytosis may be controlled by systemic glucocorticoids.