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

A Case of Focal Acral Hyperkeratosis

Affiliations
  • 1Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. yymmpark6301@ hotmail.com

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

  • 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).

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

  • 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).


Cited by  1 articles

Focal Acral Hyperkeratosis
Ji Yeon Byun, Rosa Kim, Min Young Lee, You Won Choi, Hae Young Choi
Ewha Med J. 2021;44(4):144-145.    doi: 10.12771/emj.2021.44.4.144.


Reference

1. Dowd PM, Harman RR, Black MM. Focal acral hyperkeratosis. Br J Dermatol. 1983. 109:97–103.
Article
2. Rongioletti F, Betti R, Crosti C, Rebora A. Marginal papular acrokeratodermas: a unified nosography for focal acral hyperkeratosis, acrokeratoelastoidosis and related disorders. Dermatology. 1994. 188:28–31.
Article
3. Lee SE, Kim SC. Focal acral hyperkeratosis. Clin Exp Dermatol. 2007. 32:608–610.
Article
4. Kelsell DP, Leigh IM. Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Inherited keratodermas of palms and soles. Fitzpatrick's dermatology in general medicine. 2008. 7th ed. New York: McGraw-Hill;424–431.
5. Erkek E, Kocak M, Bozdogan O, Atasoy P, Birol A. Focal acral hyperkeratosis: a rare cutaneous disorder within the spectrum of Costa acrokeratoelastoidosis. Pediatr Dermatol. 2004. 21:128–130.
Article
6. Lambiris AG, Newman PL. Marginal papular acrokeratodermas: no racial limitations for a clinical spectrum that responds to acitretin. Dermatology. 2001. 203:63–65.
Article
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