Hidrotic ectodermal dysplasia with diffuse eccrine poromatosis

Abstract

Eccrine poromatosis (EP) of remarkable severity has been observed in a patient with hidrotic ectodermal dysplasia (HED), a member of the kindred originally described by Clouston. Neither the EP nor its association with HED has been previously reported.

A Case of Multiple Eccrine Porocarcinoma with Stepping‐Stone Distribution on the Foot

“…Another possibility is that the tumors developed in eccrine poromas of long standing, as has often been reported, although eccrine poroma is usually a solitary tumor (13), and this case was clearly different from the eccrine poromatosis reported as a rare variant (14,15). Ogino (16) reported that there was a histologic difference between solitary and multiple linear eccrine poroma, particularly in the manner of proliferation, based on histological findings in the case of a 44-year-old female with multiple papular lesions of20 years' duration distributed linearly on her right lower extremity.…”

Eccrine syringofibroadenomatosis associated with hidrotic ectodermal dysplasia

“…2,4,6,7,17,26,27,31 Ten of these patients had HED. 2,4,26,27,31 This disorder is an autosomal dominant syndrome whose genetic aberration has recently been localized to chromosome 13q. 32 In HED, ectodermal tissues such as hair, teeth and nails are poorly formed, sparse or absent.…”

Circumscribed acral hypokeratosis

“…Eccrine syringofibroadenoma has, historically, been described as a solitary lesion (putatively hamartomatous or, possibly, neoplastic), 13,14 as nevoid lesions sometimes presenting in linear patterns, 15 and as hyperplasias (eg, in scar sites, regressed keratoacanthoma fields, and some areas of vascular compromise, and rarely in patients with ectodermal dysplasias). 16,17 It is not fully clear why this association is present in case 2. We believe it probably is hyperplasia in the context presented here.…”