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Classification of focal, striate, and punctate palmoplantar keratoderma

Classification of focal, striate, and punctate palmoplantar keratoderma
Type of PPK Mode of inheritance Responsible gene Characteristic clinical manifestation
Focal
Focal PPK AD KRT16, KRT6c, DSG1, TRPV3 Circumscribed calluses on the palms and soles with slight or no nail lesions. The lesion can be painful and induced by mechanical stress.
Striate
Striate PPK 1 AD DSG1 Skin thickening is prominent in a linear pattern along the flexor aspects of the fingers and over pressure points on the soles. Types I to III are classified by the responsible gene.
Striate PPK 2 AD DSP
Striate PPK 3 AD KRT1
Punctate
Punctate PPK type 1A
(Buschke-Fischer-Brauer type)
AD AAGAB Multiple tiny, punctate keratosis on the palms and soles appear during late childhood to adolescence and increase in number with age to form larger lesions. Type 1A and 1B are classified by the responsible genes.
Punctate PPK type 1B AD COL14A1
Punctate PPK type 2
(porokeratotic type)
AD Unknown Tiny, keratotic spines on the palms and soles begin during the early 20s. Histologic examination reveals columnar parakeratosis.
Punctate PPK type 3
(acrokeratoelastoidosis)
AD Unknown Small, keratotic papules that mainly involve the margins of the hands and feet appear in adolescence or adult life. Histologic examination reveals degeneration of elastic fibers.
PPK: palmoplantar keratoderma; AD: autosomal dominant.
From: Sakiyama T, Kubo A. Hereditary palmoplantar keratoderma "clinical and genetic differential diagnosis". J Dermatol 2016; 43(3):264-74. https://onlinelibrary.wiley.com/doi/full/10.1111/1346-8138.13219. Copyright © 2016 Japanese Dermatological Association. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
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