Dermatology Book

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Pemphigus

Aka: Pemphigus, Intraepidermal Blister
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  1. See Also
    1. Bullous Disease
    2. Pemphigoid
  2. Definition
    1. IntraepidermalAcantholyticBlister
    2. Mnemonic: Pemphigus ends in S for Superficial
      1. Contrast with the deeper Pemphigoid
  3. Types: Primary forms of Pemphigus
    1. Deep Pemphigus (above Basal Cell Layer of Epidermis)
      1. Pemphigus Vulgaris (most common and most severe)
      2. Pemphigus Vegetans (rare Pemphigus Vulgaris variant)
      3. Drug-Triggered Pemphigus (umasks Pemphigus Vulgaris)
    2. Superficial Pemphigus (involves Stratum Granulosum)
      1. Pemphigus Foliaceus
      2. Pemphigus erythematosus (Senear-Usher Syndrome)
      3. Pemphigus herpetiformis
      4. Fogo selvagem
      5. Pemphigus
  4. Types: Secondary forms of Pemphigus
    1. Paraneoplastic Pemphigus
    2. Drug-Induced Pemphigus (similar to Pemphigus Foliaceus)
  5. Signs
    1. Flaccid Blister (superficial)
    2. Nikolsky's Sign
      1. Positive in Pemphigus Vulgaris
      2. Negative in Pemphigus Foliaceus (most superficial)
  6. Histology
    1. All Pemphigus conditions show epidermal Acantholysis

Pemphigus (C0030807)

Definition (NCI) An autoimmune blistering skin disorder. Morphologically it is characterized by acantholysis and intraepidermal blister formation.
Definition (MEDLINEPLUS)

Pemphigus is an autoimmune disorder in which your antibodies attack healthy cells in your skin and mouth, causing blisters and sores. No one knows what causes this attack. Pemphigus does not spread from person to person. It does not appear to be inherited. But some people’s genes put them more at risk for pemphigus.

Pemphigoid is also an autoimmune skin disease. It leads to deep blisters that do not break easily. Pemphigoid is most common in older adults and may be fatal.

The treatment of pemphigus and pemphigoid is the same: one or more medicines. These may include

  • Steroids, which reduce inflammation
  • Drugs that suppress the immune system response
  • Antibiotics to treat associated infections

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases
Definition (CSP) group of chronic blistering diseases characterized histologically by acantholysis and blister formation within the epidermis.
Definition (MSH) Group of chronic blistering diseases characterized histologically by ACANTHOLYSIS and blister formation within the EPIDERMIS.
Concepts Disease or Syndrome (T047)
MSH D010392
ICD9 694.4
ICD10 L10, L10.9
SnomedCT 156355008, 200908008, 65172003
English Pemphigus, PEMPHIGUS, Pemphigus, NOS, Pemphigus NOS, Pemphigus, unspecified, pemphigus, pemphigus (diagnosis), Pemphigus NOS (disorder), Pemphigus [Disease/Finding], Pemphigus (disorder)
French PEMPHIGUS, Pemphigus
Portuguese PENFIGO, Penfigo Vulgar, Penfigo, Pênfigo
Japanese 天疱瘡, ﾃﾝﾎﾟｳｿｳ
Swedish Pemfigus
Czech pemfigus, Pemfigus
Spanish Pemphigus, pénfigo (trastorno), pénfigo, SAI (trastorno), pénfigo, SAI, pénfigo, Penfigo, Pénfigo
Finnish Pemfigus
Russian PEMFIGUS, PUZYRCHATKA, ПЕМФИГУС, ПУЗЫРЧАТКА
German Pemphiguskrankheit, nicht naeher bezeichnet, Pemphiguskrankheiten, Blasensucht, Pemphigus
Korean 상세불명의 천포창, 천포창
Polish Pęcherzyca
Hungarian Pemphigus
Dutch Pemphigus, niet gespecificeerd, pemphigus, Pemphigus
Italian Pemfigo
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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