palmoplantar keratoderma

Etiology

• aquagenic: immersion of hands or feet in water

Epidemiology

• more common in women than men

Pathology

• thickening of horny layer of the volar epidermis (palms, soles)
• orthohyperkeratosis of the stratum corneum
• dilatation of intraepidermal eccrine ducts
• hyperplasia of the eccrine sweat glands

Genetics

• heterozygous mutation in the cystic fibrosis gene
• autosomal dominant nonsense point mutation c.370C->T in AAGAB gene (type 1)

Clinical manifestations

• burning pain
• pruritus variable
• edema
• hypopigmented, translucent papules & plaques
• slowly progressing, yellowish, hyperkeratotic papules & plaques on palms & soles of feet

* images

Laboratory

• skin biopsy
• sweat chloride test is normal

Complications

• palmoplantar keratoderma type 1 may be associated with certain types of cancer*

* regular screening for cancer may be indicated

• chest X-ray, upper endoscopy, colonoscopy, Papanicolaou testing, & mammography

Management

• emollients
• topical retinoids
• topical vitamin D ointment (calcipotriol)
• topical ointments with 40% urea & 20% salicylic acid
• systemic retinoids (acitretin)
• subcutaneous injections of botulinum toxin

More general terms

• keratoderma

More specific terms

• palmoplantar keratoderma epidermolytic (EPPK, EHPPK)
• palmoplantar keratoderma non-epidermolytic
• palmoplantar keratoderma striate
• palmoplantar keratoderma with deafness (PPKDFN)