Palmoplantar Keratodermas

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Palmoplantar Keratodermas/Keratoses (PPK)/Keratosis palmaris et plantaris

Rare and clinically diverse group of genodermatoses characterised by diffuse thickening of plantar and/or palmer surfaces, usually the stratum corneum. Inheritance is autosomal dominant or recessive. Most probably due to mutation(s) of keratin gene or other structural epidermal proteins – there is evidence of abnormal maturation of fatty acids in the stratum corneum in some types. Many different clinical syndromes have been described.

May be:
• Diffuse – even thigh symmetrical hyperkeratosis; usually involves whole of palm or sole; usually appears at birth or shortly after
• Focal – large compact mass of hyperkeratosis; usually on feet
• Punctate – multiple tiny keratoses

Classification :
a) Diffuse hereditary PPK’s without associated features:
1) PPK diffuse circumscripta Unna-Thost
2) PPK transgrediens et progrediens Greither
3) PPK cum degeneratione granulosa Vorner
4) PPK of Sybert 1q
5) Mal de Meleda
6) PPK Gamborg Nielsen
7) Acral keratoderma
b) Diffuse hereditary PPK with associated features:
1) PPK mutilans Vohwinkel
2) Carcinoma of the oesophagus with keratosis palmaris et plantaris
3) PPK with sclerodactyly (Huriez syndrome)
4) Hidrotic ectodermal dysplasia (Clouston syndrome)
5) PPK and sensorineural deafness
6) Mutilating palmoplantar keratoderma with periorificial keratotic plaques (Olmsted syndrome)
7) Palmoplantar keratoderma with periodonitis (Papillon-Lefevre syndrome)
8) PPK with clubbing of the fingers and toes and skeletal deformity (Bureau, Barriere, Thomas)
c) Nummular hereditary PPK’s without associated features:
1) Keratosis palmoplantaris varians Wachters
2) Keratosis palmoplantaris nummularis (‘hereditary painful callosities’)
d) Nummular hereditary PPK’s with associated features:
1) Tyrosinaemia type II (Richner-Hanhart syndrome)
2) Pachyonychia congenita
3) Focal palmoplantar and oral mucosa hyperkeratosis syndrome
4) Keratosis palmoplantaris papillomatosa et verrucosa
e) Papular hereditary PPK’s without associated features:
1) Keratosis palmoplantaris punctata (Davie-Colley, Buschke, Fischer, Brauer)
2) Acrokeratoelastoidosis
3) Focal acral hyperkeratosis
f) Papular hereditary PPK’s with associated features:
1) Keratosis palmoplantaris with lipomata
2) Syndrome of cystic eyelids, palmoplantar keratosis, hypodontia and hypotrichosis (Schopf-Schulz-Passarge syndrome)

Can also be classified as:
1. Simple keratodermas (only have a palmoplantar hyperkeratosis)
2. Complex keratodermas (hyperkeratosis associated with other lesions)
3. Syndromic keratodermas (associated with involvement of other organs)

Most recent research is focused on using molecular biology to identify the genes responsible for the uncontrolled keratinocyte and keratin production.

Diffuse palmoplantar keratoderma:
Most common; autosomal dominant; presents in first few months of life
Well demarcated symmetric thickening of palms and soles

Mel De Meleda:
Autosomal recessive; presents at birth early infancy

Keratosis Punctata Palmaris et Plantaris:
Autosomal dominant; develops ages 12-30

Treatment:
Manual regular debridement.
Oral retinoid therapy
Keratolytics (eg 6-10% salicylic acid) with occlusion

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