Leschke syndrome
| Classification according to ICD-10 | |
|---|---|
| Q82.8 | Other specified congenital skin malformations |
| ICD-10 online (WHO version 2019) | |
The Leschke syndrome is a congenital neurocutaneous disorder with the main features cafe-au-lait spots and malformations . The disease is viewed as a special form of M Recklinghausen without the occurrence of skin tumors.
Synonyms are: congenital pigment dystrophy; Latin dystrophia pigmentosa ; English Naegeli’s type of incontinentia pigmenti
The name, which is no longer in use today, refers to the author of the first description from 1922, the German internist Erich Leschke .
Clinical manifestations
Clinical criteria are:
- numerous pigment spots (café au lait)
- somatic and psychological infantilism , dystrophy
- Metabolic disorders with genital malformations , obesity , impaired function of the adrenal glands
Skin tumors do not occur.
Differential diagnosis
The main symptom of café-au-lait spots occurs in addition to neurofibromatosis type I, also in fibrous dysplasia and Noonan syndrome .
therapy
Treatment is usually not necessary.
Individual evidence
- ↑ a b 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 .
- ↑ a b Entry on Leschke syndrome in Flexikon , a wiki from DocCheck
- ↑ a b c Encyclopedia Dermatology
- ↑ Who named it
- ↑ EW Leschke: About pigmentation in functional disorders of the adrenal gland and the sympathetic nervous system in Recklinghausen's disease. In: Klinische Wochenschrift , Berlin, 1922, vol. 28, p. 1433.
