继发于皮肤型肥大细胞增生症的皮肤松弛症

作     者：Mahajan V.K. Sharma N.L. Garg G. 朱国兴 

作者机构：Department of Dermatology Venereology and Leprosy Indira Gandhi Medical College Shimla 171001 IndiaDr. 

出 版 物：《世界核心医学期刊文摘（皮肤病学分册）》 (Digest of the World Core Medical JOurnals:Dermatology)

年 卷 期：2006年第2卷第11期

页      面：61-62页

学科分类：1002[医学-临床医学] 100206[医学-皮肤病与性病学] 10[医学] 

主　　题：皮肤松弛症 肥大细胞增生 皮肤型 色素性荨麻疹 融合性斑块 弹性纤维 活检结果 近亲婚配 异染颗粒 

摘      要：A female child, a product of a nonconsanguineous parentage and normal pregnancy, was seen first at the age of 4 years. She had suffered from recurrent episodes of multiple, erythematous, confluent plaques and a few bullous lesions all over the body since infancy. The child was irritable but had no history of flushing, dyspnea, wheezing, or frequent diarrhea. Darier’s sign was strongly positive and systemic examination did not reveal any abnormality. Routine laboratory investigations were normal and a skin biopsy specimen revealed an unremarkable epidermis and dense proliferation of round to spindle-shaped mast cells which stained positive for metachromatic granules (Fig. 1). With the diagnosis of urticaria pigmentosa, she was managed with antihistaminic drugs and the parents were advised to handle the child gently. She did not report for further follow-up. Recently, the child, now 8 years of age, was brought again with finely wrinkled, lax, and redundant skin, especially of the face, giving her a senile appearance (Fig. 2). Systemic examination did not show any abnormality. History revealed that the child had been asymptomatic during the last 4 years even without treatment, but developed slowly progressive lax and pendulous skin. A repeat biopsy from the lax facial skin revealed sparse and fragmented elastic fibres, normal collagen, and a perivascular and intradermal cellular infiltrate composed of metachromatic mast cells. Her serum α1-antitrypsin levels were 190.0 mg/dL (normal, 93-224 mg/dL) and a pulmonary function test showed no abnormality. Treatment with antihistaminic drugs was started again and she was advised to have a cosmetic facial uplift at a later stage.