Pityriasis rubra pilaris
| Classification according to ICD-10 | |
|---|---|
| L44.0 | Pityriasis rubra pilaris |
| ICD-10 online (WHO version 2019) | |
The Pityriasis rubra pilaris (also Stachelflechte or Devergie's disease ) is a rare chronic skin disease that of the hair follicles emanates. The cause is unknown. The appearance of this largely painless disease is similar to that of psoriasis (psoriasis).
The name refers to the author of the first description from 1856 by the Parisian dermatologist Marie Guillaume Devergie (1789–1879).
distribution
The frequency is given as less than 1 in 1,000,000, most cases occur sporadically, but there are also familial forms (Devergie disease) with an autosomal dominant inheritance.
root cause
The disease is based on mutations in the CARD14 gene at gene location 17q25.3.
clinic
After the relapsing appearance of small orange-red follicular papules (small nodules) at the beginning (on the scalp, arms and legs), yellow-red, extensive and sometimes flaky foci form. These can lead to reddening of the entire skin ( erythroderma ). There is also a palmoplantar hyperkeratosis . A typical sign of Pityriasis rubra pilaris are islands of unaffected skin between the foci.
therapy
Without therapy, the disease lasts for years, but eventually heals. Treatment with etretin fades and flattens the foci within a few weeks. However, complete healing takes months.
Individual evidence
- ↑ a b Pityriasis rubra pilaris. In: Orphanet (Rare Disease Database).
- ↑ 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 .
- ^ MG Devergie: Pityriasis pilaris, maladie de peau non décrite par les dermatologistes. In: Gazette Hebdomedaire de Médecine (Paris) Vol. 3, 1856, pp. 197-201.
- ^ Pityriasis rubra pilaris. In: Online Mendelian Inheritance in Man . (English)
