Dowling-Degos disease associated with squamous cell carcinomas on the dappled pigmentation

Abstract

We report the first case of Dowling-Degos disease associated with squamous cell carcinomas (SCCs) in the pigmented area of Dowling-Degos disease. A 64-year-old Japanese man manifested dappled pigmentation unusually localized to the buttocks, and two pigmented adenoid SCCs had developed on his left pigmented buttock. The other findings of Dowling-Degos disease were comedone-like lesions on the face and back, a finger-like fibroma in the right popliteal fossa, dystrophic fingernails, and a large number of seborrhoeic keratosis-like lesions predominantly on the flexural areas. Another unique clinical feature was the lack of vellus hair on the whole body surface. In addition to thin branching and elongation of rete ridges with basal hyperpigmentation, immature hair follicles surrounded by fibrosis and a lace-like pattern of the hair follicle epithelia were observed histologically. These epithelial hamartomatous features were consistent with Dowling-Degos disease. We speculate that the SCCs developed in relation to an underlying naevoid anomaly in pilosebaceous epithelia of Dowling-Degos disease.

A Study of Immunohistochemical and Electron Microscopic Changes in Dowling‐Degos Disease

“…The criteria for making the diagnosis of DDD include acquired pigmented reticulate maculae of flexures and, histopathologically, filiform or antler‐like epidermal downgrowths with basilar pigmentation. One patient with DDD was associated with squamous cell carcinomas on the dappled pigmentation (5). The clinical features and pathological changes in the case were consistent with the characteristic manifestations of DDD.…”

Caso para diagnóstico

“…3,4 Despite the lack of any consensus regarding the classification of hereditary pigmentary dermatoses, it was recently agreed that Dowling-Degos disease is a clinicopathological entity that has several variants including the acantholytic form referred to as GalliGalli disease and the acral form referred to as reticulate acropigmentation of Kitamura. 1,2,5 The present paper reports the case of a patient with clinical and histopathological characteristics of Dowling-Degos disease.…”

Dowling–Degos disease with hidradenitis suppurativa and inflammatory arthritis in two generations

“…DDD is often associated with other conditions such as hidradenitis suppurativa (HS). [ 1 ] Other associations include arthritis,[ 2 ] epidermoid cysts, keratoacanthomas,[ 3 ] squamous cell carcinoma,[ 4 ] seborrhoeic keratoses, and Haber's syndrome. [ 5 6 ]…”