Punctured palmoplantar keratosis type 3 L85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acrokeratoelastoidosis Costa; acrokeratoelastoidosis marginalis; Acrokeratoelastoidosis marginalis of the hands; Acrokeratoelastosis; Acrokeratoelastosis of the hands; acrokeratoelastosis verruciformis; Focal acral hyperkeratosis; Keratoelastoidosis; Keratoelastoidosis marginalis manuum; Keratoelastoidosis marginalis of the hands; Palmoplantar keratoderm

History
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Costa, 1956

Definition
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Autosomal dominant inherited or sporadically occurring (Hussain A et al. 2017), small papular disease (palmoplantar keratosis, classification see below keratosis palmoplantaris) of the hands and feet with signs of a cornification anomaly and elastosis.

The genetic cause of the hereditary form is still unknown.

The "focal acral hyperkeratosis" (see below hyperkeratosis, focal acral) is considered to be a variant. Keratoelastoidosis marginalis (Kocsard 1965) is also a special form of punctate palmoplantar keratosis type 3.

Occurrence/Epidemiology
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More abundant in South America.

Manifestation
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Especially for people with chronic exposure to sunlight and mechanical stress (people who work outdoors) Usually creeping on in adolescence or in young adulthood.

Localization
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Transition zone from back of the hand to palm, also on the back of the foot and transition zone from back of the foot to soles.

Clinical features
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Often linearly arranged or grouped, skin-coloured, translucent or ivory-coloured, hard horn papules with a shiny smooth surface, possibly lichenoid aspect.

Histology
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Orthohyperkeratosis with acanthosis and hypergranulosis. Dermis with signs of actinic damage, evidence of cloddy elastic material in the upper dermis and rarefaction of the elastic fibre network.

Therapy
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No causal therapy known.

Literature
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  1. Bogle MA et al (2002) Acrokeratoelastoidosis. J Am Acad Dermatol 47: 448-451
  2. Costa OG (1956) Acrokerato-elastoidosis. Dermatologica 107: 164-168
  3. Drobnitsch I et al (1988) Acrokeratoelastoidosis. Dermatologist 39: 311-313
  4. Erkek E et al (2004) Focal acral hyperkeratosis: a rare cutaneous disorder within the spectrum of Costa acrokeratoelastoidosis. Pediatric dermatol 21: 128-130
  5. Highet AS et al (1982) Acrokeratoelastoidosis. Br J Dermatol 106: 337-344
  6. Hu W et al (2002) Acrokeratoelastoidosis. Pediatric Dermatol 19: 320-322
  7. Hussain A et al (2017) New-onset acrokeratoelastoidosis in an immunosuppressed patient.
    JAAD Case Rep 4(1):75-76.
  8. Lewis KG et al (2004) Acquired disorders of elastic tissue: Part II. decreased elastic tissue. J Am Acad Dermatol 51: 165-185
  9. Tajima S et al (2002) A variant of acrokeratoelastoidosis in systemic scleroderma: report of 7 cases. J Am Acad Dermatol 46: 767-770
  10. Yoshinaga E et al (2003) Acrokeratoelastoidosis associated with nodular scleroderma. Eur J Dermatol 13: 490-492

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Last updated on: 29.10.2020