Ann Dermatol.  2009 Nov;21(4):426-428. 10.5021/ad.2009.21.4.426.

A Case of Focal Acral Hyperkeratosis

• Lee EA
• Kim HS
• Kim HO
• Park YM

• KMID: 2156513
• DOI: http://doi.org/10.5021/ad.2009.21.4.426

Abstract

Focal acral hyperkeratosis (FAH) is a rare genodermatosis with an autosomal dominant pattern of inheritance; however, it may also be sporadic. FAH is characterized by late-onset crateriform keratotic papules, some coalescing into plaques, along the borders of the hands and feet. We herein report a case of FAH in a 47-year-old male with a family history of similar lesions in three generations. The histological findings revealed focal areas of orthohyperkeratosis over an area of depressed but otherwise normal epidermis. The dermis showed no specific changes, which distinguished this case from acrokeratoelastoidosis, which shows elastorrhexis of clinically similar lesions.

Keyword

Autosomal dominant inheritance; Focal acral hyperkeratosis

MeSH Terms

Dermis
Epidermis
Family Characteristics
Foot
Hand
Humans
Male
Middle Aged

Figure

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  Fig. 1 Asymptomatic, multiple, firm, yellowish to flesh colored, hyperkeratotic polygonal papules clustered on the dorsum and lateral aspects of the hands (A) and feet (B).

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  Fig. 2 The pedigree demonstrates seven individuals affected in three generations with the clinical features of FAH (black shading depicts affected individuals).

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  Fig. 3 (A) Focal orthohyperkeratosis overlying a crateriform depression in a slightly acanthotic epidermis with slight hypergranulosis (H&E, ×100). (B) No evidence of elastorrhexis was observed (Verhoeff-van Gieson, ×400).

Reference

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