Bullous pemphigoid
Bullous pemphigoid | |
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A patient present with legs covered in popped blisters caused by bullous pemphigoid. The blisters cover his entire body. | |
Specialty | Dermatology |
Bullous pemphigoid (a type of
Signs and symptoms
Clinically, the earliest lesions may appear as a hives-like red raised rash, but could also appear dermatitic, targetoid, lichenoid, nodular, or even without a rash (essential pruritus).[1] Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Oral lesions are present in a minority of cases.[2] The disease may be acute, but can last from months to years with periods of exacerbation and remission.[3]
Several other skin diseases may have similar symptoms. However,
Causes
In most cases of bullous pemphigoid, no clear precipitating factors are identified.
Pathophysiology
The bullae are formed by an immune reaction, initiated by the formation of
Diagnosis
Diagnosis consist of at least 2 positive results out of 3 criteria (2-out-of-3 rule): (1) pruritus and/or predominant cutaneous blisters, (2) linear IgG and/or C3c deposits (in an n- serrated pattern) by direct
Treatment
Treatments include
The anti-CD20 monoclonal antibody rituximab has been found to be effective in treating some otherwise refractory cases of pemphigoid.[13] A 2010 (updated in 2023) meta-analysis of 14 randomized controlled trials showed that oral steroids and potent topical steroids are effective treatments, although their use may be limited by side-effects, while lower doses of topical steroids are safe and effective for treatment of moderate bullous pemphigoid.[12]
IgA-mediated pemphigoid can often be difficult to treat even with usually effective medications such as rituximab.[14]
Prognosis
Bulbous pemphigoid may be self-resolving in a period ranging from several months to many years even without treatment.[2] Poor general health related to old age is associated with a poorer prognosis.[2]
Epidemiology
Very rarely seen in children, bullous and non-bullous pemphigoid most commonly occurs in people 70 years of age and older.[2] Its estimated frequency is seven to 14 cases per million per year, but has been reported to be as high as 472 cases per million per year in Scottish men older than 85.[2] At least one study indicates the incidence might be increasing in the United Kingdom.[15] Some sources report it affects men twice as frequently as women,[11] while others report no difference between the sexes.[2]
Many mammals can be affected, including dogs, cats, pigs, and horses, as well as humans. It is very rare in dogs; on average, three cases are diagnosed around the world each year.[citation needed]
Research
Animal models of bullous pemphigoid have been developed using transgenic techniques to produce mice lacking the genes for the two known autoantigens, dystonin and collagen XVII.[7][8]
See also
- Cicatricial pemphigoid
- Dystonin
- Gestational pemphigoid
- List of target antigens in pemphigoid
- List of immunofluorescence findings for autoimmune bullous conditions
References
- PMID 25617817.
- ^ ISBN 978-0-07-146690-5. Retrieved July 21, 2012.
- ISBN 9780071632447.
- PMID 23254748.
- ^ a b Chan LS (2011). "Bullous Pemphigoid". EMedicine Reference.
- ^ "Dorlands Medical Dictionary:bullous pemphigoid". Retrieved 2010-06-24.[permanent dead link]
- ^ a b c Online Mendelian Inheritance in Man (OMIM): DYSTONIN; DST - 113810
- ^ a b Online Mendelian Inheritance in Man (OMIM): COLLAGEN, TYPE XVII, ALPHA-1; COL17A1 - 113811
- PMID 29713318.
- PMID 30624575.
- ^ a b c ""Bullous Pemphigoid." Quick Answers to Medical Diagnosis and Therapy". Retrieved 2012-07-21.
- ^ PMID 37572360.
- PMID 29520266.
- PMID 25901938.
- PMID 18614511.
Further reading
- Wojnarowska F, Kirtschig G, Highet AS, Venning VA, Khumalo NP (August 2002). "Guidelines for the management of bullous pemphigoid". The British Journal of Dermatology. 147 (2): 214–21. S2CID 762307.