婴儿恶性萎缩性丘疹病一例

Malignant atrophic papulosis in an infant

报道1例婴儿恶性萎缩性丘疹病.患儿女,8个月,出生后4天,躯干四肢反复发生红色丘疹,缓慢愈合后遗留瓷白色瘢痕,表面脱屑,周围绕以水肿性红晕.组织病理检查见表皮明显萎缩变薄,真皮中上部轻度水肿,少许黏蛋白沉积,局部附属器减少,未见明显血管病变及炎细胞浸润.因活动皮损少未予治疗,随访16个月仍有少量新发小皮损,无系统受累表现,是否属良性皮肤型仍需进一步随访.

A case of infant malignant atrophic papulosis（also known as Degos＇ disease）is reported. The 8-month-old girl presented with recurrent erythematous papules with central porcelain-white depressions on the trunk and extremities. An erythematous papule was noticed on the abdomen by her mother 4 days after birth, which then became necrotic and umbilicated, healed rather slowly to leave a small white scar, surrounded by pink edematous border and telangiectases. Additional lesions developed in sequential fashion. A skin biopsy revealed mild edema in the upper dermis, with little mucin deposition and decreased adnexal structures. The overlying epidermis was atrophic. There was no apparent vasculopathy or perivascular infiltration of inflammatory cells. Considering only few active lesions, the patient was followed up closely without any treatment in the next 16 months. During this period, only a few small lesions developed, and no systemic symptom was present. Further follow-up is required to ascertain whether the diagnosis of the case is a benign cutaneous form of malignant atrophic papulosis.