Medicine:Epidermolysis bullosa acquisita
| Epidermolysis bullosa acquisita | |
|---|---|
| Other names | Acquired epidermolysis bullosa[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.[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.[2]
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
- List of cutaneous conditions
- List of target antigens in pemphigoid
- List of immunofluorescence findings for autoimmune bullous conditions
- List of human leukocyte antigen alleles associated with cutaneous conditions
References
- ↑ 1.0 1.1 1.2 1.3 "Orphanet: Acquired epidermolysis bullosa" (in en). https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=46487.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 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. https://pubmed.ncbi.nlm.nih.gov/31181325/.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "21. Chronic blistering dermatoses" (in en). Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. p. 468-469. ISBN 978-0-323-54753-6. https://books.google.com/books?id=UEaEDwAAQBAJ&dq=Chronic+blistering&pg=PA468.
External links
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