Scleromyxedema
| Classification according to ICD-10 | |
|---|---|
| L98.5 | Mucinosis of the skin |
| ICD-10 online (WHO version 2019) | |
The Scleromyxoedema is a very rare skin disease , a severe form of Lichen myxoedematosus with lichen-like papules and scale thickening and hardening of the skin ( pachydermia ).
Synonyms are: Arndt-Gottron syndrome; Generalized papular and sclerodermoid lichen myxedematosus; Mucinosis, lichenoid papular, generalized variant; Scleromyxedema; Scleromyxedema
The name refers to Georg Arndt and Heinrich Gottron , from whom the first description comes from 1954.
distribution
The frequency is not known; the disease usually begins between the ages of 40 and 60.
root cause
The cause is not known; monoclonal paraproteinemia is present in over 90% .
Clinical manifestations
Clinical criteria are:
- scleroderma-like picture
- hyperpigmented pachydermia
- Lichen-like papules in the skin lines on the face, glabella , neck, arms, hands and trunk
- Atherosclerosis in the kidneys and coronary vessels
- Pulmonary fibrosis
- Peripheral neuropathy
- Esophageal motility disorders
Differential diagnosis
The following are to be distinguished:
- Scleroderma
- Nephrogenic systemic fibrosis
- Scleroedema adultorum
- Eosinophilic fasciitis
- pretibial myxedema
- other mucinosis
literature
- M. Neufeld, C. Sunderkötter, RK Moritz: [Scleromyxedema]. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Volume 69, number 11, November 2018, pp. 916-921, doi: 10.1007 / s00105-018-4257-8 , PMID 30135969 (review).
- AC Sala, PR Cunha, CA Pinto, CA Alves, IB Paiva, AP Araujo: Scleromyxedema: clinical diagnosis and autopsy findings. In: Anais brasileiros de dermatologia. Volume 91, number 5 suppl 1, 2016 Sep-Oct, pp. 48-50, doi: 10.1590 / abd1806-4841.20164527 , PMID 28300892 , PMC 5324991 (free full text).
- V. Mehta, C. Balachandran, R. Rao: Arndt Gottron scleromyxedema: successful response to treatment with steroid minipulse and methotrexate. In: Indian journal of dermatology. Volume 54, number 2, 2009, pp. 193-195, doi: 10.4103 / 0019-5154.53183 , PMID 20101325 , PMC 2807169 (free full text).
Individual evidence
- ↑ a b c d e Encyclopedia Dermatology
- ↑ Scleromyxedema. In: Orphanet (Rare Disease Database).
- ↑ Entry on scleromyxedema in Flexikon , a wiki of the DocCheck company
- ↑ Lichen Myxedematosus emedicine
- ^ HA Gottron: Scleromyxedema (a peculiar form of myxothesauroderma). In: Archive for Dermatology and Syphilis , Berlin, 1954, 199: 71–91.
- ↑ Who named it
