II. Epidemiology

  1. Mean age of onset: 50 years

III. Pathophysiology

  1. Autoimmune Subepidermal Blistering condition

IV. Signs

  1. Trauma prone areas more commonly affected
  2. Tense Blisters and erosions over extensor surfaces
    1. Knuckles
    2. Dorsal hands
    3. Elbows
    4. Knees
    5. Ankles
  3. Mucosal involvement common
    1. Oral, nasal, and esophageal mucosa
    2. Conjunctival mucosa

V. Labs

  1. Histology
    1. Subepidermal Blister with inflammatory infiltrate
  2. Immunofluorescence
    1. IgG deposits at dermal-epidermal junction

VI. Complications

  1. Scarring skin lesions (with associated milia)

VII. Differential Diagnosis

  1. Porphyria cutanea tarda
  2. Hereditary Epidermolysis Bullosa (non-immune mediated)
  3. Bullous Pemphigoid (non-scarring)

VIII. Course

  1. Chronic waxing and waning course

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Related Studies

Ontology: Epidermolysis Bullosa (C0014527)

Definition (NCI_NCI-GLOSS) A group of chronic skin disorders in which fluid-filled blisters form on the skin and mucosa (the moist, inner lining of some organs and body cavities). Epidermolysis bullosa is inherited and usually starts at birth. Patients with epidermolysis bullosa may be at increased risk of squamous cell cancer of the skin.
Definition (NCI) An autosomal recessive inherited skin disorder caused by mutations in the genes encoding keratins 5 and 14, collagen VII or laminin 5. It is characterized by skin fragility and the formation of blisters. The blisters may become large and ulcerated, resulting in skin infections and loss of body fluids.
Definition (MSH) Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties.
Definition (CSP) inherited chronic noninflammatory skin disease manifested by vesicles, large bullae (blisters), and skin erosions which often result from trauma.
Concepts Congenital Abnormality (T019) , Disease or Syndrome (T047)
MSH D004820
ICD10 Q81 , Q81.9
SnomedCT 205580008, 61003004
English Acantholysis Bullosa, Epidermolysis bullosa, unspecified, acantholysis bullosa, Epidermolysis Bullosa [Disease/Finding], Epidermolysis Bullosa, bullosa epidermolysis, epidermolysis bullosa, epidermolysis bullosa (diagnosis), Epidermolysis bullosa, Acantholysis bullosa, Acanthosis bullosa, Bullous recurrent eruption, Dermatitis bullosa hereditaria, Fox disease, Keratolysis bullosa hereditaria, EB - Epidermolysis bullosa, Epidermolysis bullosa (disorder), bullosa; epidermolysis, epidermolysis; bullosa, Epidermolysis bullosa, NOS
Swedish Epidermolysis bullosa
Japanese ヒョウヒスイホウショウ, 機械刺激性水疱症, 表皮水疱症, 先天性表皮水疱症, 水疱性棘融解
Czech epidermolysis bullosa, Epidermolysis bullosa
Finnish Epidermolysis bullosa
Italian Acantolisi bollosa, Epidermolisi bollosa
German Epidermolysis bullosa, nicht naeher bezeichnet, Acantholysis bullosa, Epidermolysis bullosa hereditaria, Epidermolysis bullosa
Korean 표피 수포증, 상세불명의 표피 수포증
Polish Oddzielanie się naskórka pęcherzowe
Hungarian Epidermolysis bullosa
Norwegian Epidermolysis bullosa
Croatian Epidermolysis bullosa, Bulozna epidermoliza
Spanish Acantólisis Bulosa, Epidermólisis Bullosa, acantólisis bullosa, dermatitis bullosa hereditaria, enfermedad de Fox, epidermólisis bullosa (trastorno), epidermólisis bullosa, erupción recurrente bullosa, queratólisis bullosa hereditaria, Epidermolisis bullosa, Acantólisis Ampollosa, Epidermólisis Ampollosa
Dutch bullosa; epidermolyse, epidermolyse; bullosa, Epidermolysis bullosa, niet gespecificeerd, epidermolysis bullosa, Acantholysis bullosa, Epidermolysis bullosa
Portuguese Epidermólise bolhosa, Acantólise Bolhosa, Epidermólise Bolhosa
French Epidermolyse bulleuse, Épidermolyse bulleuse, Acantholyse bulleuse

Ontology: Epidermolysis Bullosa Acquisita (C0079293)

Definition (NCI) A chronic autoimmune inflammatory disorder characterized by the formation of subepidermal blisters in the skin and the mucous membranes.(NICHD)
Definition (MSH) Form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. Direct immunofluorescence shows IMMUNOGLOBULIN G deposited at the dermo-epidermal junction.
Concepts Disease or Syndrome (T047)
MSH D016107
ICD10 L12.3 , L12.30
SnomedCT 200911009, 2772003
English Acquired Epidermolysis Bullosa, Epidermolysis Bullosa Acquisita, Epidermolysis Bullosa, Acquired, EBA - Epidermoly bull acquisit, Epidermolysis bull acquisita, Acquired epidermolysis bullosa, unspecified, Epidermolysis Bullosa Acquisita [Disease/Finding], Acquired Form of Epidermolysis Bullosa, acquisita bullosa epidermolysis, epidermolysis bullosa acquisita, acquired epidermolysis bullosa (diagnosis), EBA, Acquired epidermolysis bullosa, EBA - Epidermolysis bullosa acquisita, Epidermolysis bullosa acquisita, Acquired epidermolysis bullosa (disorder), epidermolysis; bullosa, acquired
Swedish Epidermolysis bullosa acquisita
Japanese コウテンセイヒョウヒスイホウショウ, 後天性表皮水疱症, 表皮水疱症-後天性
Czech epidermolysis bullosa acquisita, Získaná bulózní epidermolýza
Finnish Hankinnainen rakkulainen epidermolyysi
German Erworbene Epidermolysis bullosa, Epidermolysis bullosa acquisita, Epidermolysis bullosa, erworbene
Korean 후천성 수포성 표피박리증
Polish Oddzielanie się naskórka pęcherzowe nabyte
Hungarian Szerzett epidermolysis bullosa
Norwegian Epidermolysis bullosa acquisita, Ervervet epidermolysis bullosa
Spanish Epidermólisis Bullosa Acquisita, epidermólisis bullosa adquirida (trastorno), epidermólisis bullosa adquirida, Epidermolisis bullosa adquirida, Epidermólisis Ampollosa Adquirida, Epidermólisis Bullosa Adquirida
Dutch epidermolyse; bullosa, verworven, verworven epidermolysis bullosa, Epidermolysis bullosa acquisita, Verworven epidermolysis bullosa
Portuguese Epidermolise bulhosa adquirida, Epidermólise Bolhosa Adquirida
French Epidermolyse bulleuse héréditaire, Épidermolyse bulleuse acquise
Italian Epidermolisi bollosa acquisita