家族性Dowling-Degos病合并化脓性汗腺炎1例并家系调查

Familial Dowling-Degos Disease Complicated with Hidradenitis Suppurativa:A Case Report and Family Survey

先证者女,50岁,皱褶部位反复脓肿、结节36年,黑褐色斑丘疹20年。皮肤科情况:面颈部、乳房、腋下、腹股沟区黑褐色斑丘疹,面颈部、躯干黑头粉刺、凹陷性瘢痕;后颈部、褶皱部位增生性瘢痕形成。皮损组织病理示:表皮突呈指状突起,伴色素增加,真皮浅层可见噬色素细胞。先证者家族中有9例有类似病史,其中男3例,女6例。诊断:家族性Dowling-Degos病合并化脓性汗腺炎。

The proband,a 50-year-old female,had recurrent abscesses and nodules in the flexures areas for 36 years,and black-brown maculopapules for 20 years.Physical examination showed there were black-brown maculopapules on the neck,breast,axilla and groins,blackheads and pitted scars on the face,neck and trunk.Hypertrophic scars were distributed over the hindneck and flexures areas.Histopathology showed elongated rete ridges in a finger-form with hyperpigmentation,and melanophage in the superficial dermis.Nine of the propositus′s family members had similar medical history,including three males and six females.Diagnosis of familial Dowling-Degos disease complicated with hidradenitis suppurativa was made.