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palmoplantar keratoderma
Etiology:
- aquagenic: immersion of hands or feet in water
Epidemiology:
- more common in women than men [2]
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 [2]
Genetics:
- heterozygous mutation in the cystic fibrosis gene [2]
- autosomal dominant nonsense point mutation c.370C->T in AAGAB gene (type 1) [4]
Clinical manifestations:
- burning pain
- pruritus variable [4]
- edema
- hypopigmented, translucent papules & plaques [2]
- slowly progressing, yellowish, hyperkeratotic papules & plaques on palms & soles of feet [4]
* images [3,4]
Laboratory:
- skin biopsy
- sweat chloride test is normal [2]
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 [4]
- systemic retinoids (acitretin) [3]
- subcutaneous injections of botulinum toxin [2]
Specific
palmoplantar keratoderma epidermolytic (EPPK, EHPPK)
palmoplantar keratoderma non-epidermolytic
palmoplantar keratoderma striate
palmoplantar keratoderma with deafness (PPKDFN)
General
keratoderma
References
- Stedmans Medical Dictionary,
Williams & Wilkins 1995, 26th ed
- Poletti ED, Munoz-Sandoval R
Aquagenic Keratoderma.
N Engl J Med 2014; 371:952. September 4, 2014
PMID: 25184867
http://www.nejm.org/doi/full/10.1056/NEJMicm1314806
- DermNet NZ. Acquired keratoderma (images)
http://www.dermnetnz.org/scaly/acquired-keratoderma.html
- Klein B, Treudler R
Images in Clinical Medicine: Palmoplantar Papules.
N Engl J Med 2021; 384:1447. April 15
PMID: 33471452
https://www.nejm.org/doi/full/10.1056/NEJMicm2032907