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KERATODERMA OF PALMS AND SOLES (KERATOSIS PALMARIS ET PLANTARIS)

Excessive hyperkeratosis of the palms and soles may occur as a manifestation of a focal or generalized congenital hereditary skin disorder or may result from such chronic skin diseases as psoriasis, eczema, pityriasis rubra pilaris, lupus erythematosus, or Reiter disease. 

Diffuse Hyperkeratosis of Palms and Soles (Unna-Thost Syndrome, Tylosis).

This autosomal dominant disorder presents in the first few months of life with erythema that gradually progresses to sharply demarcated hyperkeratotic scaling plaques over the palms and soles. The margins of the plaques often remain red; plaques may extend along the lateral aspects of the hands and feet and onto the volar wrists and the heels.

Epidermolytic Hyperkeratosis.

This type of hyperkeratosis, which is localized to the palms and soles, is an autosomal dominant defect involving mutations in the gene for keratin 9 with clinical findings identical to those of the Unna-Thost type. There is no hyperhidrosis, however, and affected areas may blister.

Mal de Meleda (Keratoderma Palmoplantaris Transgrediens).

This rare, progressive autosomal recessive condition is characterized by erythema and thick scales on the palms, fingers, soles, and flexor aspects of the wrists, knees, and elbows. Hyperhidrosis, nail thickening or koilonychia, and eczema may also be present.

Mutilating Keratoderma (Vohwinkel Syndrome).

This is a progressive autosomal dominant disease with honeycombed hyperkeratosis of palms and soles, sparing the arches; starfish-like and linear keratoses on the dorsum of the hands, fingers, feet, and knees; and ainhum-like constriction of the digits that sometimes leads to autoamputation. This disorder may be associated with alopecia and hearing loss. Mutations of the gene for loricrin, a major protein of the cornified cell envelope.

Papillon-Lefevre Syndrome.

This autosomal recessive erythematous hyperkeratosis of the palms and soles sometimes extends to the dorsal hands and feet, elbows, and knees later in childhood. This syndrome is characterized by periodontal inflammation, leading to loss of teeth by age 4-5 yr if untreated.

Keratoderma of palms and soles also occurs as a feature of some forms of ichthyosis and ectodermal dysplasia. Richner-Hanhart syndrome is an autosomal recessive palmoplantar keratoderma with corneal ulcers, progressive mental impairment, and a deficiency of tyrosine aminotransferase, which leads to tyrosinemia. Pachyonychia congenita is transmitted as an autosomal dominant trait with variable expressivity. The classic type I form (Jadassohn-Lewandowski syndrome) is due to mutations in the gene for keratin 16. Major features of the syndrome are onychogryphosis; palmoplantar keratoderma.

The nail dystrophy is the most striking feature and may be present at birth or develop early in life. The nails are thickened and tubular, projecting upward at the free edge to form a conical roof over a mass of subungual keratotic debris. Repeated paronychial inflammation may result in shedding of the nails. The feature seen most consistently among patients with this condition is keratoderma of the palms and soles. Additional associated features include hyperhidrosis of the palms and soles, and bullae and erosions on the palms and soles. Some patients have shown a selective cell-mediated defect in recognition and processing of Candida. Surgical removal of the nails and excision of the nail matrix.

Patients with palmoplantar hyperhidrosis may have macerated plaques that become secondarily infected and malodorous. Morbidity is lessened if the hyperkeratosis can be controlled by treatment; however, only mild palliation is achieved with applications of lubricants, keratolytic agents (urea, salicylic acid, lactic acid), and oral retinoids including etretinate, isotretinoin, and acitretin.