Dall'Oglio F, Nasca M R, Taparelli F, Bacchelli B, Micali G
Dermatology Clinic, University of Catania, Catania, Italy.
Pediatr Dermatol. 2001 May-Jun;18(3):230-3. doi: 10.1046/j.1525-1470.2001.018003230.x.
Atrophia maculosa varioliformis cutis is a rare and distinctive form of idiopathic facial macular noninflammatory atrophy that may rarely be observed in members of the same family. We describe two brothers, ages 14 and 16 years, with spontaneously appearing, asymptomatic, varioliform and linear atrophic lesions. Their past medical history was positive for varicella occurring in childhood without residual facial scarring. Routine laboratory investigations and screening for circulating autoantibodies were negative. Both patients were concordant for HLA A2 and DQ4.1. Routine and ultrastructural histologic examination of a punch biopsy specimen showed the presence of scarce, small, fragmented elastic fibers and compact collagen bundles associated with hypertrophic fibroblasts in the dermis. Our patients remained clinically stable, untreated, over a 2-year follow-up period. No long-term follow-up data have previously been reported.
痘疮样皮肤萎缩是一种罕见且独特的特发性面部黄斑非炎性萎缩形式，在同一家族成员中很少见。我们描述了两名分别为14岁和16岁的兄弟，他们有自发出现的、无症状的痘疮样和线性萎缩性病变。他们过去的病史显示童年时有水痘发作，但面部无残留疤痕。常规实验室检查和循环自身抗体筛查均为阴性。两名患者的HLA A2和DQ4.1均一致。对钻孔活检标本进行的常规和超微结构组织学检查显示，真皮中存在稀少、细小、断裂的弹性纤维以及与肥大的成纤维细胞相关的致密胶原束。在2年的随访期内，我们的患者未经治疗，临床情况保持稳定。此前尚无长期随访数据报道。
