Hereditary palmoplantar keratosis

Classification according to ICD-10
Q82.8	Hereditary palmoplantar keratosis
ICD-10 online (WHO version 2019)

A hereditary palmoplantar keratosis , English hereditary palmoplantar keratoderma , describes a group of congenital diseases with a cornification disorder affecting the skin of the hands and feet ( hyperkeratosis ).

Classification

This includes a variety of diseases that can be classified as follows:

• Palmoplantar hyperkeratosis
  • Diffuse palmoplantar hyperkeratosis
    • Epidermolytic palmoplantar keratosis (synonyms: EPPK; keratosis palmoplantaris diffusa Vörner; palmoplantar hyperkeratosis type Vörner; keratosis palmoplantaris diffusa Vörner-Unna-Thost; epidermolytic palmoplantar hyperkeratosis, EPPK, Unna-Thost's disease))
    • Keratoderma palmoplantaris transgrediens et progrediens ( Greither syndrome ) (synonyms: Keratosis palmoplantaris diffusa transgrediens et progrediens Greither Palmoplantar keratoderma of Sybert; Greither's keratosis; Greither's palmoplantar progressive keratoderma; Meleda syndrome)
    • Mal de Meleda (synonyms: Keratosis palmoplantaris transgrediens et progrediens Mljet; Meleda disease; Keratosis palmoplantaris transgrediens Siemens; Keratodermia palmo-plantaris transgrediens et progrediens type Mljet or Kogoj; Akrokeratodermia type Meleda)
    • Keratoderma hereditarium mutilans (synonyms: Keratosis palmoplantaris mutilans Vohwinkel; Vohwinkel syndrome; palmoplantar hyperkeratosis type mutilans Vohwinkel; Nockemann syndrome)
    • KID Syndrome Acronym for Keratitis-Ichthyosis-Deafness-Syndrome, (synonyms: Keratitis-Ichthyosis-Deafness Syndrome; Hystrix-like Ichthyosis-Deafness; Senter's Syndrome)
    • Keratoderma hereditarium mutilans with ichthyosis (synonyms: Loricrin keratoderma; Camisa disease; keratodermatosis - ichthyosiform dermatosis - increased beta-glucuronidase activity; Vohwinkel syndrome - ichthyosis; Camisa variant of the mutilating palmoplantar hyperkeratosis with mutilating palmoplantar hyperkeratosis; mutilating palmoplantar hyperkeratosis)
    • Olmsted syndrome (synonyms: Mutilating palmoplantar keratosis with periorifical keratotic plaques)
    • Click Syndrome , acronym for Keratosis Linearis - Ichthyosis Congenita - Sclerosing Keratoderm
    • Huriez syndrome (synonyms: palmoplantar keratosis-sclerodactyly syndrome; scleroatrophic syndrome; sclerotylosis; palmoplantar keratoderm with sclerodactyly and increased risk of skin cancer)
  • Focal palmoplantar hyperkeratosis
    • Keratosis palmoplantaris striata (synonyms: Keratosis palmoplantaris striata et areata; Keratosis palmoplantaris varians Wachters; Brünauer-Fuhs-Siemens type, Wachters type, Keratosis palmoplantaris areate Siemens)
    • Focal palmoplantar and gingival keratosis (synonyms: palmoplantar keratosis, focal gingival; focal palmoplantar and oral mucosal hyperkeratosis syndrome)
    • Keratoderm with esophageal carcinoma ( Howel-Evans syndrome ) (synonyms: Clark-Howel-Evans-McConnell syndrome; tylosis; palmoplantar keratosis - esophageal carcinoma; Bennion-Patterson syndrome; Howell-Evans syndrome)
    • Tyrosinemia type II (synonyms: oculocutaneous tyrosinemia; Richner-Hanhart syndrome)
    • Acrokeratoelastoidosis Costa (synonyms: AKE; hypokeratosis, palmoplantar dotted, type 3; PPKP3; palmoplantar keratosis, dotted, type 3; acrokeratoelastoidosis focal acral hyperkeratosis).

Syndromal forms

In the context of syndromes , palmoplantar keratosis can be an essential feature. This is the case with palmoplantar keratosis - congenital alopecia, autosomal recessive .

Individual evidence

1. ↑ Hereditary palmoplantar keratosis. In: Orphanet (Rare Disease Database).
2. ↑ ^{a b} Derma-net-online ( memento of the original from November 20, 2015 in the Internet Archive ) Info: The archive link was inserted automatically and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. 
3. ↑ Epidermolytic palmoplantar keratosis. In: Orphanet (Rare Disease Database).
4. ↑ Who named it
5. ↑ Keratoderma palmoplantaris transgrediens et progrediens. In: Orphanet (Rare Disease Database).
6. ↑ ^{a b c} Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
7. ↑ Mal de Meleda. In: Orphanet (Rare Disease Database).
8. ↑ Keratoderma hereditarium mutilans. In: Orphanet (Rare Disease Database).
9. ↑ KID syndrome. In: Orphanet (Rare Disease Database).
10. ↑ Keratoderma hereditarium mutilans with ichthyosis. In: Orphanet (Rare Disease Database).
11. ↑ Mutilating palmoplantar keratosis with periorified keratotic plaques. In: Orphanet (Rare Disease Database).
12. ↑ Keratosis linearis - Ichthyosis congenita - sclerosing keratoderm. In: Orphanet (Rare Disease Database).
13. ↑ Palmoplantar Keratosis-Sclerodactyly Syndrome. In: Orphanet (Rare Disease Database).
14. ↑ Keratosis palmoplantaris striata. In: Orphanet (Rare Disease Database).
15. ↑ Focal palmoplantar and gingival keratosis. In: Orphanet (Rare Disease Database).
16. ↑ Tyrosinemia type 2. In: Orphanet (database for rare diseases).
17. ↑ PPKP3.  In: Online Mendelian Inheritance in Man . (English)
18. ↑ Costa acrokeratoelastoidosis. In: Orphanet (Rare Disease Database).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !