Peeling skin syndrome
Synonyms: Deciduous skin · Familial continuous skin peeling syndrome · Idiopathic deciduous skin · Keratosis exfoliativa congenita · PSS · Peeling skin disease
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Autosomal recessive
- Age of onset
- Infancy, Neonatal
- Status
- active
A group of rare autosomal recessive forms of ichthyosis clinically characterized by superficial, asymptomatic, spontaneous peeling of the skin and histologically by a shedding of the outer layers of the epidermis. PSS presents with either an acral (acral PSS) or a generalized distribution for generalized PSS type A (noninflammatory) or B (inflammatory).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Peeling skin syndrome | Deciduous skin · Familial continuous skin peeling syndrome · Idiopathic deciduous skin · Keratosis exfoliativa congenita · PSS · Peeling skin disease |
| fr | Peeling skin syndrome | Keratosis exfoliativa congenita · PSS · Peau déciduale |
| de | Peeling Skin-Syndrom | Abschälende Haut · Familiäres fortdauerndes Peeling Skin-Syndrom · Hautablösung, idiopathische · Keratosis exfoliativa congenita · PSS · Peeling Skin-Krankheit · Schalenhaut-Syndrom |
| es | Síndrome de descamación cutánea | Enfermedad de descamación de la piel · PSS · Piel decidual · Piel decidual idiopática · Queratosis exfoliativa congénita · Síndrome de descamación continua de la piel familiar |
| it | Sindrome da desquamazione cutanea | Cheratolisi esfoliativa congenita · Malattia da desquamazione cutanea · PSS · Pelle deciduale · Pelle deciduale idiopatica · Sindrome familiare da desquamazione cutanea continua |
| nl | 'Peeling skin'-syndroom | 'Peeling skin'-ziekte · Afschilferende huid · Familiaal continu afschilferende huid-syndroom · Idiopathische afschilferende huid · Keratosis exfoliativa congenita · PSS |
| pt | Síndrome pele descascada | Pele decícua |
| pl | Zespół złuszczającej się skóry | Skóra liściasta |
| cs | Syndrom olupování kůže | Keratolysis exfoliativa · Keratosis exfoliativa congenita · PSS · Familiární trvalý syndrom olupování kůže · Idiopatická opadavá kůže · Nemoc olupování kůže |
| uk | Синдром лущення шкіри | Листяна шкіра · Сімейний синдром безперервного лущення шкіри · Ідіопатичне висихання шкіри · Вроджений ексфоліативний кератоз · Лущення шкіри · PSS |
| zh | 皮肤剥离综合征 | 家族性持续皮肤剥离综合征 |
| tr | Peeling cilt sendromu | PSS · Belirli mevsimlerde dökülen cilt · Cilt soyulması hastalığı · İdiyopatik belirli mevsimlerde dökülen cilt · Keratoz eksfoliativa konjenita · Ailesel sürekli cilt soyulması sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | EC20.1 | E | specific | validated |
| MONDO | 0019347 | E | validated | |
| MONDO | 19347 | E | validated | |
| MeSH | C564818 | E | validated | |
| UMLS | C1849193 | E | validated | |
| GARD | 7347 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:281082Inherited non-syndromic ichthyosis
rare genetic diseases; rare skin diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/817
- This record as JSON: /data/d/817.json