Poikiloderma, congenital with warty hyperkeratosisICD-10: Q82.8; ICD-11: LD2B

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.11.2023

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Synonym(s)

congenital development malformation; Dowling Syndrome; Thomson syndrome type verrucosus; Type verrucosus of Thomson syndrome

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HistoryThis section has been translated automatically.

Dowling, 1936; Greither, 1958

DefinitionThis section has been translated automatically.

Congenital poikiloderma with hyperkeratosis, considered by some authors to be a special form of Thomson's syndrome. There is overlap with Kindler syndrome.

EtiopathogenesisThis section has been translated automatically.

Probably autosomal recessive inheritance.

ManifestationThis section has been translated automatically.

Poikiloderma: Between 6 and 12 months of age. Hyperkeratoses: Between 7 and 10 years of age.

Clinical featuresThis section has been translated automatically.

Poikiloderma, especially in the face, warty hyperkeratosis over bony prominences. Proportionate dwarfism. Associated symptoms: palmoplantar keratoses.

General therapyThis section has been translated automatically.

Regular skin check-ups to rule out malignant neoplasia.

External therapyThis section has been translated automatically.

Vitamin A acid low concentration R256, or 3-10% salicylic acid (e.g. salicylic vaseline Lichtenstein, R228, R227 ), 5-10% urea or lactic acid, if necessary in combination R108/ R105 for keratolysis, if necessary under occlusion.

Internal therapyThis section has been translated automatically.

Trial with acitretin (neotigason) dosage 0.5-1.0 mg/kg bw/day over a longer period of time as tumor prophylaxis.

Progression/forecastThis section has been translated automatically.

Occurrence of spinocellular carcinomas.

Note(s)This section has been translated automatically.

There have been no further messages since 1981!

LiteratureThis section has been translated automatically.

  1. Dowling GB (1936) Congenital developmental malformation. Proc R Soc Med 29: 1633-1634
  2. Greither A (1958) On hereditary dysplasia of the skin associated with keratoses and pigment disorders. Dermatologist 9: 364-369
  3. Pickenacker A et al (1998) Congenital poikiloderma with verruciform hyperkeratoses (Dowling type). dermatologist 49: 586-590
  4. Schrallhammer K et al (1988) Congenital poikiloderma with warty hyperkeratoses. Dermatologist 39: 143-148

Authors

Last updated on: 07.11.2023

Author: Prof. Dr. med. Peter Altmeyer