Punctate palmoplantar keratoderma in Adult
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Synopsis
Numerous subtypes have been identified, and varied classification systems exist:
Punctate 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. Mutations in the alpha and gamma adaptin binding protein (AAGAB) on chromosome 15 have been identified. Only a few reported kindreds have associated malignancy (squamous cell carcinoma, Hodgkin disease, and renal, breast, lung, prostatic, pancreatic, and colonic adenocarcinomas). Other rare associations include spastic paralysis, ankylosing spondylitis, anodontia, deuteranopia, deuteranomaly, freckle-like hyperpigmentation, or HIV disease.
Punctate 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.
Punctate 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 2 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.
Related topic: palmoplantar keratoderma
Codes
ICD10CM:L85.2 – Keratosis punctata (palmaris et plantaris)
SNOMEDCT:
402773000 – Punctate palmoplantar keratoderma
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Porokeratosis palmaris et plantaris
- Verrucae vulgaris
- Arsenical keratosis
- Callosities and corns
- Palmar pits – Darier disease, basal cell nevus syndrome, pitted keratolysis
- Secondary syphilis
- Keratoderma blennorhagica
- Aquagenic palmar keratoderma (hereditary translucent acrokeratoderma)
- Acrokeratosis paraneoplastica
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Management Pearls
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Therapy
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References
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Last Reviewed:05/05/2021
Last Updated:01/23/2022
Last Updated:01/23/2022
Patient Information for Punctate palmoplantar keratoderma in Adult
Overview
Punctate palmoplantar keratoderma (PPPK), also known as keratoderma punctata, is characterized by areas of tiny pits or bumps and thickened skin of the palms and soles.There are numerous subtypes of PPPK that are all usually inherited.
PPPK is a life-long condition in which skin lesions develop with age. The thickened skin lesions may interfere with engaging in certain activities. Although in most cases one's general health is not affected, cancers of the colon, lung, kidney, breast, and the lymphatic system can rarely occur.
Who’s At Risk
PPPK is rare. Because the disease can be inherited, family history is a strong indicator for diagnosis. For those with PPPK in the family, it is particularly important to know about any family history of internal cancers.PPPK of the palmar creases is a distinct subtype that has been identified in Black patients.
Signs & Symptoms
PPPK is characterized by areas of tiny pits or bumps and thickened skin of the palms and soles. Some present with pits and others with raised lesions or spines. Palms of the hands and soles of the feet are affected. In some subtypes, the instep of the sole and the side of the hand are also affected.Self-Care Guidelines
- Moisturizing creams
- Treatments for improving thickened skin (keratolytic agents) such as salicylic acid, propylene glycol, lactic acid, urea
When to Seek Medical Care
When the lesions on the palms and soles enlarge and come together to form calluses on pressure points, it may cause pain and discomfort, which would be a reason to see a doctor for treatment.The following red flag symptoms are an indication to seek medical treatment:
- Impaired hand or finger movements
- Difficulty walking
Treatments
Treatments your physician may prescribe:- Topical retinoids
- Topical corticosteroids
- Oral retinoids (eg, acitretin)
- Topical vitamin D ointment (calcipotriene)
- Topical 5-flurouracil cream
- Photochemotherapy (eg, psoralen plus UVA [PUVA])
- Pairing down of thickened areas of skin (eg, cryosurgery, mechanical debridement, excision)
Punctate palmoplantar keratoderma in Adult