IgA pemphigus
| IgA pemphigus | |
|---|---|
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| Subcorneal pustular dermatosis | |
 | |
| Specialty | Dermatology |
| Symptoms | Painful itchy blisters[1] |
| Diagnostic method | Skin biopsy[1] |
| Treatment | Corticosteroids[1] |
| Deaths | Rare[1] |
IgA pemphigus is a skin disease typically presenting with itchy painful blisters and sores.[1] It begins with a few small fragile bumps on a reddish base, which soon burst to form roundish areas of crust. Although the whole body can be affected, it generally affects the underarms and groin, but it does not affect the mouth.[1]
It is due to IgA antibodies acting against the components on skin cell surfaces responsible for keeping the skin intact.[1] Why this occurs is not clear, but the condition is associated with several other conditions, including monoclonal IgA gammopathy, multiple myeloma, (HIV) infection, Sjogren disease, rheumatoid arthritis, and Crohn's disease.[1] There are two types; subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic dermatosis (IND).[1]
Diagnosis is confirmed by skin biopsy and the results of direct and indirect immunofluorescence.[2] Treatment is with corticosteroids, both by applying to skin and by mouth.[1]
It is rare.[1] Early descriptions were made by Darrell Wilkinson.[3]
Definition and types
There are two distinct forms:
- Subcorneal pustular dermatosis (also known as Sneddon–Wilkinson disease and pustulosis subcornealis)[4] is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.[5][6]: 203 This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddon's syndrome, also known as Ehrmann-Sneddon syndrome, is also a different syndrome.[7]
- Intraepidermal neutrophilic IgA dermatosis is characterized histologically by intraepidermal bullae with neutrophils, some eosinophils, and acantholysis.[6]: 465
Signs and symtoms
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Subcorneal pustular dermatosis
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Subcorneal pustular dermatosis
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Subcorneal pustular dermatosis
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Subcorneal pustular dermatosis
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History
Early descriptions were made by Darrell Wilkinson, a British dermatologist.[3]
See also
- List of cutaneous conditions
- List of conditions caused by problems with junctional proteins
- List of immunofluorescence findings for autoimmune bullous conditions
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Aslanova, Minira; Yarrarapu, Siva Naga S.; Zito, Patrick M. (2021). "IgA Pemphigus". StatPearls. StatPearls Publishing. PMID 30085605. Archived from the original on 2022-04-05. Retrieved 2022-04-04.
- ↑ Fullen, Douglas; Chan, May P.; Andea, Aleodor A.; Arps, David P. (2018). "6. IgA pemphigus". Handbook of Direct Immunofluorescence: A Pattern-Based Approach to Skin and Mucosal Biopsies. JP Medical Ltd. pp. 31–35. ISBN 978-1-907816-90-1. Archived from the original on 2022-04-05. Retrieved 2022-04-04.
- ↑ 3.0 3.1 "Munks Roll Details for Peter Edward Darrell Sheldon Wilkinson". munksroll.rcplondon.ac.uk. Archived from the original on 2017-11-10. Retrieved 2017-11-10.
- ↑ "Orphanet: Subcorneal pustular dermatosis". www.orpha.net. Archived from the original on 12 March 2017. Retrieved 8 March 2017.
- ↑ Irwin M. Freedberg; et al., eds. (2003). Fitzpatrick's dermatology in general medicine (6th ed.). New York, NY [u.a.]: McGraw-Hill. p. 625. ISBN 978-0-07-138076-8.
- ↑ 6.0 6.1 James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
- ↑ Berlit, Peter. "Sneddon's Syndrome". Orphanet. Archived from the original on 2020-11-12. Retrieved 2017-03-08.
External links
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