Keratoelastoidosis Marginalis

Abstract

Keratoelastoidosis marginalis is a chronic and progressive disease that is difficult to treat. The differential includes focal acral hyperkeratosis and acrokeratoelastoidosis.

Acquired disorders of elastic tissue: part I. increased elastic tissue and solar elastotic syndromes

“…Patients may present with asymptomatic or mildly tender, slowly progressive, bilateral, white, yellow, or erythematous, waxy or translucent, scaly or smooth telangiectatic linear papules that coalesce into plaques at the junction of the dorsal and palmar skin on the radial and, less often, ulnar sides of the hand, in the first web space extending along the ulnar surface of the thumb and radial surface of the index finger, and rarely on the palms and dorsa of the hands. 165,171 Supporting the trauma hypothesis, lesions occur more often on the dominant hand, which is more likely be used to perform manual labor. 166 Histopathology.…”

“…Several treatments for keratoelastoidosis marginalis have been reported anecdotally in 2 patients with little long-term success, including laser therapy, high-potency topical corticosteroids, topical tazarotene and tretinoin, oral isotretinoin, and cryotherapy. 171 …”

“…172 Focal acral hyperkeratosis typically occurs on the hands and feet of black children; histologic changes are confined to the epidermis and spare the dermis. 171 Elastotic nodules of the ear may show masses of amorphous elastotic material in the dermis that are larger and deeper than those seen in keratoelastoidosis marginalis and are surrounded by fibrosis. 168 PXE shows calcification of elastic fibers usually in the middle and lower third of the dermis, deeper that that seen in keratoelastoidosis marginalis.…”

A case of acrokeratoelastoidosis

“…Although AKE and FAH share similar clinical and histological manifestations, elastin staining reveals a decrease and/or fragmentation of elastic fibres in AKE, whereas there is no alteration in elastic fibres in FAH . The histological features of KM are prominent degeneration of collagen fibres, focal calcium deposition, ands fragmented and thickened elastic fibres …”

Collagenous and elastotic marginal plaques of the hand: A potential clue to the diagnosis of alkaptonuria

“…Synonyms for CEMPH include degenerative collagenous plaques of the hand, digital papular calcific elastosis, and marginal keratoderma of the palms. 5,6 Rarely, CEMPH has been reported in association with alkaptonuria (AKU). Herein, CEMPH is described as a manifestation of AKU in a 55-year-old woman, with histopathology permitting recognition of this previously undiagnosed genodermatosis.…”