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道林-迪戈斯病(屈侧网状色素异常症):6例临床及组织病理学研究

Dowling-Degos disease (reticulate pigmented anomaly of the flexures): a clinical and histopathologic study of 6 cases.

作者信息

Kim Y C, Davis M D, Schanbacher C F, Su W P

机构信息

Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.

出版信息

J Am Acad Dermatol. 1999 Mar;40(3):462-7. doi: 10.1016/s0190-9622(99)70498-6.

DOI:10.1016/s0190-9622(99)70498-6
PMID:10071319
Abstract

BACKGROUND

Few case series describing Dowling-Degos disease (DDD) have been reported.

OBJECTIVE

Our purpose was to review the clinical and histopathologic findings in DDD.

METHODS

We reviewed the clinical and histopathologic findings in 6 patients with DDD who were evaluated at the Mayo Clinic.

RESULTS

In addition to the typical flexural pigmented reticulate macules, comedo-like lesions on the back or neck or both were present in all 6 patients; 3 patients had pitted perioral scars, and 3 patients reported pruritus of affected flexural areas. Five patients were female, 5 patients had onset of pigmentation before age 24 years, and 3 patients had a family history of DDD. One patient had additional pigmentation involving the dorsum of the hands and proximal nailfolds and fingernail dystrophy. Histopathologically, pigmented rete ridge elongation with thinning of suprapapillary epithelium, dermal melanosis, and perivascular lymphohistiocytic infiltration were consistently observed.

CONCLUSION

Comedo-like lesions, pruritus, and pitted perioral scars are common features in association with the reticulate flexural pigmentation. Histopathologically, pigmented rete ridge elongation and dermal melanosis of biopsy specimens from flexural areas are seen.

摘要

背景

报道的描述Dowling-Degos病(DDD)的病例系列很少。

目的

我们的目的是回顾DDD的临床和组织病理学表现。

方法

我们回顾了在梅奥诊所接受评估的6例DDD患者的临床和组织病理学表现。

结果

除典型的屈侧色素沉着网状斑外,所有6例患者背部或颈部或两者均出现粉刺样损害;3例患者有口周凹陷性瘢痕,3例患者报告受累屈侧部位瘙痒。5例患者为女性,5例患者在24岁之前出现色素沉着,3例患者有DDD家族史。1例患者手部背部、近端甲襞和指甲营养不良处有额外色素沉着。组织病理学上,始终观察到色素沉着的 rete 嵴延长,乳头上方上皮变薄,真皮黑素沉着和血管周围淋巴细胞组织细胞浸润。

结论

粉刺样损害、瘙痒和口周凹陷性瘢痕是与网状屈侧色素沉着相关的常见特征。组织病理学上,可见屈侧部位活检标本的色素沉着 rete 嵴延长和真皮黑素沉着。

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