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

Abstract

Collagenous and elastotic marginal plaques of the hand (CEMPH) is a rare, chronic keratoderma characterized by hyperkeratotic linear plaques located along the radial and ulnar aspects of the hands bilaterally. As an isolated finding, CEMPH occurs secondarily to chronic trauma and photodamage. Herein, CEMPH is described as a manifestation of alkaptonuria (AKU). In addition to keloidal collagen, ochronotic fibers and fragmented, thickened elastic fibers were observed. Additionally, mucin deposition—not previously described in this clinical context—was also identified. Given their overlapping clinicopathologic features, CEMPH due to AKU should be distinguished from the acquired variant as well as acrokeratoelastoidosis.

Two Case Reports of Elastolytic Giant Cell Granuloma on the Palms

“…With regard to diseases that occur on the lateral margins of the hands, elastorrhexis and degenerate collagen bundles are also found in collagenous and elastotic marginal plaques of the hands (CEMPH), a rare entity that often presents as a linear depressed band appearing symmetrically on the hands of the elderly. 17 , 18 However, CEMPH was reported to contain a relatively acellular dermis without giant cells or any other significant inflammation. Elastophagocytosis was also not a feature of CEMPH.…”

“…A comparison of the clinicopathological characteristics of GA, CEMPH, and EGCG is shown in Table 1 . 10 , 18 , 19 …”

“…A comparison of the clinicopathological characteristics of GA, CEMPH, and EGCG is shown in Table 1. 10,18,19 Treatment with EGCG is often not required because the lesions are benign and asymptomatic. Spontaneous remission over months to years has been documented.…”

A proposal for the term ochronotic keratoderma

“…I read the article by Good et al with interest. The authors are to be commended for their thoughtful analysis and conclusion.…”