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

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 07.11.2023

Dieser Artikel auf Deutsch

Synonym(s)

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

History
This section has been translated automatically.

Dowling, 1936; Greither, 1958

Definition
This 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.

Etiopathogenesis
This section has been translated automatically.

Probably autosomal recessive inheritance.

Manifestation
This section has been translated automatically.

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

Clinical features
This section has been translated automatically.

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

General therapy
This section has been translated automatically.

Regular skin check-ups to rule out malignant neoplasia.

External therapy
This 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 therapy
This 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/forecast
This section has been translated automatically.

Note(s)
This section has been translated automatically.

There have been no further messages since 1981!

Literature
This 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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 07.11.2023