Punctate palmoplantar keratoderma in Adult
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
L85.2 – Keratosis punctata (palmaris et plantaris)
402773000 – Punctate palmoplantar keratoderma
- 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