ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (50)
Punctate palmoplantar keratoderma in Adult
Other Resources UpToDate PubMed

Punctate palmoplantar keratoderma in Adult

Contributors: Vivian Wong MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Punctate palmoplantar keratoderma (PPK), or keratosis punctata, is a heterogeneous group of conditions characterized by small hyperkeratotic growths on the palms and soles. Many cases are inherited in an autosomal dominant fashion, although sporadic cases have also been reported. There is no sex predilection.

Numerous subtypes have been identified, and varied classification systems exist:

PPK type 1, also known as the diffuse type, Buschke-Fischer-Brauer disease, keratosis punctata palmaris et plantaris, and keratosis papulosa – Patients present with small hyperkeratotic papules on the palms or soles that appear in late childhood to adolescence. These lesions increase in size and number with time. Acquired forms have later onset and may be associated with malignancies (squamous cell carcinoma, Hodgkin disease, and renal, breast, lung, prostatic, pancreatic, and colonic adenocarcinomas), spastic paralysis, ankylosing spondylitis, anodontia, deuteranopia, deuteranomaly, freckle-like hyperpigmentation, or HIV disease.

PPK type 2, also known as spiny keratoderma and music box spine keratoderma – In the inherited form, patients present with tiny keratotic spines on the palms and soles that appear in childhood. Sporadic cases present later in life and may be associated with renal, pulmonary, and gastrointestinal carcinomas, as well as lymphoproliferative malignancies and melanoma. Other reported underlying disease associations have included polycystic kidney disease, chronic renal failure, type 4 hyperlipoproteinemia, and pulmonary tuberculosis.

PPK type 3, also known as acrokeratoelastoidosis (AKE), marginal keratoderma, acrokeratoelastoidosis lichenoides, focal acral hyperkeratosis (FAH), and acrokeratoelastoidosis of Costa – Patients present with flat or umbilicated keratotic shiny papules on the marginal border of the palms, soles, and digits that erupt during adolescence or young adulthood. Hyperhidrosis may occur in some patients.

FAH appears during young adulthood and is clinically identical to AKE. The major difference between these two disorders is the histopathologic finding of elastorrhexis (fragmentation and degeneration of elastic fibers) in the dermis, a feature that is present in AKE but absent in FAH. In addition, hyperhidrosis is rarely associated with AKE but is not associated with FAH. It is still controversial whether FAH is a distinct entity or a variant of AKE.

Punctate PPK of the palmar creases (keratosis punctata of the palmar creases) is a distinct variant that has been identified in patients of African descent.

In Cole disease, punctate PPK is seen in association with guttate hypopigmentation, with or without calcifications within tendons, skin, and breast tissue.

For more information on PPK type 1, see OMIM.

For more information on PPK type 2, see OMIM.

For more information on PPK type 3, see OMIM.

Related topic: Palmoplantar keratoderma

Codes

ICD10CM:
L85.2 – Keratosis punctata (palmaris et plantaris)

SNOMEDCT:
402773000 – Punctate palmoplantar keratoderma

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:12/06/2019
Last Updated:01/24/2020
Copyright © 2021 VisualDx®. All rights reserved.
Punctate palmoplantar keratoderma in Adult
Punctate palmoplantar keratoderma : Palms and soles, Pits, Thick scaly plaques
Copyright © 2021 VisualDx®. All rights reserved.