Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita
Other names: Acquired epidermolysis bullosa[1]
Epidermolysis bullosa acquisita
SymptomsBlisters, fragile skin, scarring, hair loss[2]
Usual onsetAround age 50-years[1]
DurationLongterm
TypesMechanobullous and non-mecahnobullous[2]
CausesAntibodies to type VII collagen[3]
Diagnostic methodBehaviour of symptoms, DIF, autoantibodies against collagen VII[2]
Differential diagnosisPorphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, drug eruption[3]
MedicationCorticosteroids, azathioprine, dapsone[3]
PrognosisLongterm, disabling[1]
FrequencyRare, males=females[2]
DeathsNot fatal[1]

Epidermolysis bullosa acquisita, also known as acquired epidermolysis bullosa, is a longterm autoimmune blistering skin disease.[1] It generally presents with fragile skin that blisters and becomes red with or without trauma.[2] Marked scarring is left with thin skin, milia and nail changes.[3] It typically begins around age 50-years.[2]

It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin.[3] Damaged skin may become infected.[3]

Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen.[2] It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption.[3] The condition is longterm and has no cure.[1] A good response may be seen with corticosteroids, either alone or combined with azathioprine or dapsone.[3]

It is rare, with around 0.08 to 0.5 new cases per million people per year, and it affects males and females equally.[2]

Signs and symptoms

It generally presents with fragile skin that blisters and becomes red with or without trauma.[2] Marked scarring is left with thin skin, milia and nail changes.[3] It typically begins around age 50-years.[2]

  • Epidermolysis bullosa acquisita
    Epidermolysis bullosa acquisita
  • Epidermolysis bullosa acquisita
    Epidermolysis bullosa acquisita

Cause

It is caused by antibodies to type VII collagen within anchoring fibril structures located at the dermoepidermal junction in skin.[3]

Diagnosis

Diagnosis is by observing the persistence of the condition, direct immunofluorescence, and detecting autoantibodies against type VII collagen.[2] It can appear similar to porphyria cutanea tarda, pemphigoid, pemphigus, dermatitis herpetiformis, or blistering drug eruption.[3]

Treatment

The condition is longterm and has no cure.[1] A good response may be seen with corticosteroids, either alone or combined with azathioprine or dapsone.[3]

Epidemiology

It is rare, with around 0.08 to 0.5 new cases per million people per year, and it affects males and females equally.[2]

See also

References

  1. 1 2 3 4 5 6 7 "Orphanet: Acquired epidermolysis bullosa". www.orpha.net. Archived from the original on 30 July 2017. Retrieved 19 April 2019.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 Kridin, Khalaf; Kneiber, Diana; Kowalski, Eric H.; Valdebran, Manuel; Amber, Kyle T. (August 2019). "Epidermolysis bullosa acquisita: A comprehensive review". Autoimmunity Reviews. 18 (8): 786–795. doi:10.1016/j.autrev.2019.06.007. ISSN 1873-0183. PMID 31181325. Archived from the original on 2022-03-13. Retrieved 2022-03-25.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "21. Chronic blistering dermatoses". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. p. 468-469. ISBN 978-0-323-54753-6. Archived from the original on 2022-03-25. Retrieved 2022-03-24.
Classification
External resources
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