Pemphigus

This article discusses pemphigus as a skin disease, see Pemphigus (disambiguation) for more meanings .

Classification according to ICD-10
L10	Pemphigus
ICD-10 online (WHO version 2019)

Pemphigus

Pemphigus (from Greek πέμφιξ pemphix bladder, edema on the skin) is a generic term for a group of skin diseases that belong to the autoimmune blistering dermatoses and, as pemphigus diseases, are counted among the bullous dermatoses .

Incidence

The incidence of pemphigus in Central Europe is one to two new cases per 1 million population per year. 80% of people with pemphigus suffer from pemphigus vulgaris .

Etiopathogenesis

In pemphigus diseases, the autoantibodies formed are directed against desmosomal adhesion molecules ( desmogleine ) of the cadherin group , which leads to intraepidermal loss of cell contact.

classification

According to ICD-10, the following subtypes are distinguished in group 'L10':

• L10.0 Pemphigus vulgaris
• L10.1 Pemphigus vegetans
• L10.2 Pemphigus foliaceus
• L10.3 Brazilian pemphigus (fogo selvagem)
• L10.4 Pemphigus seborrhoicus ( syn.pemphigus erythematosus or Senear-Usher syndrome )
• L10.5 drug-induced pemphigus
• L10.8 Other pemphigus diseases
• L10.9 Pemphigus disease, unspecified

Outside of ICD-10 group 'L10', the following pemphigus subtypes are listed:

• Q82.2 Pemphigus chronicus benignus familiaris ( syn.Hailey -Hailey's disease )
• L00 Pemphigus (acutus) neonatorum - through the transfer of maternal IgG autoantibodies to the unborn fetus (the disease is also sometimes referred to as staphylococcal scalded skin syndrome (SSS syndrome), which is triggered by staphylococci in newborns.)

Other forms of pemphigus are:

• IgA pemphigus
• Paraneoplastic pemphigus
• Dermatitis herpetiformis Duhring (Syn .: Duhring's disease, Duhring-Brocq disease )
• Bullous pemphigoid ( syn.parapemphigus )

Guidelines

The guidelines of the Working Group of the Scientific Medical Societies in Germany (AWMF) for the treatment of pemphigus vulgaris and bullous pemphigoid can be found under the registration number 013-071.

literature

• Enno Schmidt; Detlef Zillikens : Diagnosis and therapy of bullous autoimmune dermatoses (medical review) . In: Dtsch Arztebl Int . tape 108 (23) , June 11, 2011, pp. 399–405 , doi : 10.3238 / arztebl.2011.0399 ( aerzteblatt.de ).

Individual evidence

1. ↑ http://www.enzyklopaedie-dermatologie.de/artikel?id=2959 Etiology of pemphigus
2. ↑ Archived copy ( Memento of the original dated August 2, 2016 in the Internet Archive ) Info: The archive link has been inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. ICD-10 classification (L10) 
3. ↑ http://www.enzyklopaedie-dermatologie.de/artikel?id=2962 Senear Usher Syndrome
4. ↑ http://flexikon.doccheck.com/de/Pemphigus Other forms of pemphigus
5. ↑ http://www.enzyklopaedie-dermatologie.de/artikel?id=14884 IgA-Pemphigus
6. ↑ AWMF registration number 013-071 (as of December 18, 2014); valid until December 31, 2018; Classification: S2k guideline pemphigus vulgaris and bullous pemphigoid, diagnosis and therapy . In: AWMF online

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !