Symmetrical acral keratoderma revisited: proposal for a new term, ‘pigmented carpotarsal hyperkeratosis’

W. Chen, Z. Song, Chao-Chun Yang, F. Hao

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

First reported from Taiwan mistakenly as acral acanthosis nigricans in 1991, pigmented carpotarsal hyperkeratosis or hyperkeratosis nigricans carpi et tarsi displays a peculiar distribution of velvety brown-grey hyperpigmented plaques symmetrically on the flexural side of the wrists and ankles and on the dorsal sides of the hands and feet. A marked epidermal hyperkeratosis with typically mild acanthosis and papillomatosis is observed in histology. Whitish maceration upon perspiration or water exposure, with exacerbation in summer but remission in winter, is common. The association with obesity, endocrine disorders, atopic dermatitis, ichthyosis or malignancy is unknown. Familial occurrence and hereditary patterns are ill-defined. There is preliminary evidence indicating a pathogenic role of missense mutation in the transcription factor 4 gene. Treatment is empirical, with good outcome with topical retinoids and keratolytic agents. Recurrence is common, and long-term prognosis is unclear. To be distinguished are acral acanthosis nigricans, palmoplantar keratoderma of the Nagashima type, palmoplantar keratoderma of the Bothnian type and aquagenic palmoplantar keratoderma. Most reported cases are from Southern China and are predominantly observed in men between the ages of 20 and 40 years. The currently used term ‘symmetrical acral keratoderma’ is non-specific and misleading and may lead to global unawareness, underreporting or misdiagnosis of this phenomenon. Further genetic and molecular studies are required to clarify its pathogenesis and relation to palmoplantar keratoderma.

Original languageEnglish
Pages (from-to)277-280
Number of pages4
JournalJournal of the European Academy of Dermatology and Venereology
Volume33
Issue number2
DOIs
Publication statusPublished - 2019 Feb 1

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Palmoplantar Keratoderma
Acanthosis Nigricans
Extremities
Ankle
Keratolytic Agents
Ichthyosis
Retinoids
Papilloma
Missense Mutation
Atopic Dermatitis
Diagnostic Errors
Wrist
Taiwan
Foot
Molecular Biology
China
Histology
Transcription Factors
Hand
Obesity

All Science Journal Classification (ASJC) codes

  • Dermatology
  • Infectious Diseases

Cite this

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title = "Symmetrical acral keratoderma revisited: proposal for a new term, ‘pigmented carpotarsal hyperkeratosis’",
abstract = "First reported from Taiwan mistakenly as acral acanthosis nigricans in 1991, pigmented carpotarsal hyperkeratosis or hyperkeratosis nigricans carpi et tarsi displays a peculiar distribution of velvety brown-grey hyperpigmented plaques symmetrically on the flexural side of the wrists and ankles and on the dorsal sides of the hands and feet. A marked epidermal hyperkeratosis with typically mild acanthosis and papillomatosis is observed in histology. Whitish maceration upon perspiration or water exposure, with exacerbation in summer but remission in winter, is common. The association with obesity, endocrine disorders, atopic dermatitis, ichthyosis or malignancy is unknown. Familial occurrence and hereditary patterns are ill-defined. There is preliminary evidence indicating a pathogenic role of missense mutation in the transcription factor 4 gene. Treatment is empirical, with good outcome with topical retinoids and keratolytic agents. Recurrence is common, and long-term prognosis is unclear. To be distinguished are acral acanthosis nigricans, palmoplantar keratoderma of the Nagashima type, palmoplantar keratoderma of the Bothnian type and aquagenic palmoplantar keratoderma. Most reported cases are from Southern China and are predominantly observed in men between the ages of 20 and 40 years. The currently used term ‘symmetrical acral keratoderma’ is non-specific and misleading and may lead to global unawareness, underreporting or misdiagnosis of this phenomenon. Further genetic and molecular studies are required to clarify its pathogenesis and relation to palmoplantar keratoderma.",
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Symmetrical acral keratoderma revisited : proposal for a new term, ‘pigmented carpotarsal hyperkeratosis’. / Chen, W.; Song, Z.; Yang, Chao-Chun; Hao, F.

In: Journal of the European Academy of Dermatology and Venereology, Vol. 33, No. 2, 01.02.2019, p. 277-280.

Research output: Contribution to journalReview article

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