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Keratosis Palmaris Plantaris Syndrome: Overview

Introduction

Palmoplantar Keratoderma (PPK) constitutes a heterogeneous group of disorders characterized by severe skin scalding as a result of thickening of the palms and the soles of affected individuals. They are divided based on whether they are inherited or acquired. The inherited forms are classified into three distinct clinical patterns of epidermal involvement.

  • Diffuse PPK: Uniform involvement of the palmoplantar surface. The age of onset of the disease is within the first few months of life.

  • Focal and Striate PPK: Consisting of localized areas of hyperkeratosis located mainly on pressure points and sites of recurrent friction.

  • Punctate Keratoderma: Characterized by multiple small, hyperkeratotic papules, spicules, or nodules on the palms and soles. These tiny keratoses may involve the entire palmoplantar surface or may be restricted to certain locations (eg, palmar creases).

It is also divided into a subclassification of which variants are based on the presence of simple keratoderma or isolated PPK. Others involve lesions of nonvolar skin, hair, teeth, nails, or sweat glands and/or with abnormalities of other organs.

Finally, the acquired forms of PPK are divided into keratoderma climactericum, keratoderma associated with internal malignancy, PPK due to inflammatory and reactive dermatoses, PPK caused by infections, drug-related PPK, and systemic disease-associated PPK.

Diffuse hereditary palmoplantar keratoderma types without associated features are summarized in Table K-1.

TABLE K-1Diffuse Hereditary Palmoplantar Keratoderma without Associated Features
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TABLE K-1 Diffuse Hereditary Palmoplantar Keratoderma without Associated Features
Usual Name Synonyms Typical Features Genetics
Diffuse non-epidermolytic palmoplantar keratoderma (NEPPK) Diffuse NEPPK; Thost-Unna disease; PPK diffusa circumscripta

Onset: <2 years

Palmoplantar hyperkeratosis, hyperhidrosis, thick nails

Autosomal dominant

Mutation in type II keratin locus (12q11-13)
Diffuse epidermolytic palmoplantar keratoderma (EPPK) Diffuse EPPK; Vorner disease; PPK cum degeneration granulosa

Onset: <1 year

Same phenotype as NEPPK but with epidermolytic hyperkeratosis

Autosomal dominant

Mutation in 1A rod domain segment of the keratin 9 gene (chromosome 17)
Progressive palmoplantar keratoderma (PPK) Greither disease, PPK transgrediens et progrediens

Onset: 8-10 years

Extension onto dorsum of hands and feet, hyperhidrosis; improves after 50 years of age

 Autosomal dominant
Palmoplantar keratosis Gamborg-Nielsen Gamborg-Nielsen keratoderma Mutilating constriction bands surrounding fingers Autosomal recessive

Diffuse hereditary palmoplantar keratoderma types with associated features are summarized in Table K-2.

TABLE K-2Diffuse Hereditary Palmoplantar Keratoderma with Associated Features
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TABLE K-2 Diffuse Hereditary Palmoplantar Keratoderma with Associated Features
Usual Name Synonyms Typical Features Genetics
Meleda disease Keratosis extremitatum hereditaria transgrediens et progrediens; Mal de Meleda; Mljet disease

Onset: early infancy

Keratoderma with erythematous border, hyperhidrosis, periorbital erythema, lingua plicata, syndactyly, hairy palms and soles

Autosomal recessive

Gene map locus: 8qter Greither variant: autosomal dominant
Palmoplantar keratoderma with periodontitis and onychogryposis ☞Haim-Munk Syndrome; prepubertal periodontitis Syndrome

Onset: 1-5 years

Periodontosis, disorder of leukocyte function (increased infections), hyperhidrosis, malodor, ...

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