IgA pemphigus: Difference between revisions
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{{Infobox medical condition | |||
| name = IgA pemphigus | |||
| image = [[File:Dimeric_IgA_schematic_01.svg|250px]] | |||
| caption = Schematic representation of dimeric [[Immunoglobulin A|IgA]] | |||
| field = [[Dermatology]] | |||
| synonyms = | |||
| symptoms = [[Blister]]s, [[skin]] [[erosion]]s, [[pruritus]] | |||
| complications = [[Secondary infection]] | |||
| onset = | |||
| duration = | |||
| types = Subcorneal pustular dermatosis type, Intraepidermal neutrophilic type | |||
| causes = Autoimmune reaction involving [[IgA]] antibodies | |||
| risks = | |||
| diagnosis = [[Skin biopsy]], [[direct immunofluorescence]] | |||
| differential = [[Pemphigus vulgaris]], [[bullous pemphigoid]], [[dermatitis herpetiformis]] | |||
| prevention = | |||
| treatment = [[Dapsone]], [[corticosteroids]], [[immunosuppressive drug]]s | |||
| medication = | |||
| prognosis = Variable, often chronic | |||
| frequency = Rare | |||
}} | |||
{{Short description|An autoimmune blistering skin disease}} | {{Short description|An autoimmune blistering skin disease}} | ||
'''IgA pemphigus''' is a rare [[autoimmune disease]] characterized by the presence of [[blister]]s on the skin and mucous membranes. It is a subtype of [[pemphigus]], which is a group of autoimmune blistering disorders. Unlike other forms of pemphigus, IgA pemphigus is mediated by [[immunoglobulin A]] (IgA) antibodies rather than [[immunoglobulin G]] (IgG). | '''IgA pemphigus''' is a rare [[autoimmune disease]] characterized by the presence of [[blister]]s on the skin and mucous membranes. It is a subtype of [[pemphigus]], which is a group of autoimmune blistering disorders. Unlike other forms of pemphigus, IgA pemphigus is mediated by [[immunoglobulin A]] (IgA) antibodies rather than [[immunoglobulin G]] (IgG). | ||
== Pathophysiology == | == Pathophysiology == | ||
IgA pemphigus is caused by the production of IgA antibodies against components of the [[epidermis]]. These antibodies target proteins in the [[desmosome]]s, which are structures that help maintain the integrity of the skin by holding [[keratinocyte]]s together. The binding of IgA antibodies to these proteins leads to the disruption of cell adhesion, resulting in the formation of blisters. | IgA pemphigus is caused by the production of IgA antibodies against components of the [[epidermis]]. These antibodies target proteins in the [[desmosome]]s, which are structures that help maintain the integrity of the skin by holding [[keratinocyte]]s together. The binding of IgA antibodies to these proteins leads to the disruption of cell adhesion, resulting in the formation of blisters. | ||
== Clinical Presentation == | == Clinical Presentation == | ||
Patients with IgA pemphigus typically present with [[erythematous]] plaques and [[vesicle|vesicles]] that may coalesce into larger blisters. These lesions are often pruritic and can appear on any part of the body, but are commonly found on the [[trunk]] and [[extremities]]. Unlike other forms of pemphigus, mucosal involvement is less common in IgA pemphigus. | Patients with IgA pemphigus typically present with [[erythematous]] plaques and [[vesicle|vesicles]] that may coalesce into larger blisters. These lesions are often pruritic and can appear on any part of the body, but are commonly found on the [[trunk]] and [[extremities]]. Unlike other forms of pemphigus, mucosal involvement is less common in IgA pemphigus. | ||
== Diagnosis == | == Diagnosis == | ||
The diagnosis of IgA pemphigus is confirmed through a combination of clinical examination, [[histopathology]], and [[direct immunofluorescence]]. A skin biopsy will show subcorneal pustules with neutrophilic infiltration. Direct immunofluorescence of perilesional skin will reveal IgA deposition in the epidermis. | The diagnosis of IgA pemphigus is confirmed through a combination of clinical examination, [[histopathology]], and [[direct immunofluorescence]]. A skin biopsy will show subcorneal pustules with neutrophilic infiltration. Direct immunofluorescence of perilesional skin will reveal IgA deposition in the epidermis. | ||
== Treatment == | == Treatment == | ||
The management of IgA pemphigus involves the use of medications to reduce inflammation and suppress the immune response. [[Dapsone]] is often the first-line treatment due to its anti-inflammatory and immunomodulatory effects. In cases where dapsone is ineffective or not tolerated, systemic [[corticosteroid]]s or other immunosuppressive agents may be used. | The management of IgA pemphigus involves the use of medications to reduce inflammation and suppress the immune response. [[Dapsone]] is often the first-line treatment due to its anti-inflammatory and immunomodulatory effects. In cases where dapsone is ineffective or not tolerated, systemic [[corticosteroid]]s or other immunosuppressive agents may be used. | ||
== Prognosis == | == Prognosis == | ||
The prognosis for patients with IgA pemphigus is generally favorable, especially with appropriate treatment. The disease tends to have a chronic course with periods of remission and exacerbation. Long-term management may be necessary to control symptoms and prevent relapses. | The prognosis for patients with IgA pemphigus is generally favorable, especially with appropriate treatment. The disease tends to have a chronic course with periods of remission and exacerbation. Long-term management may be necessary to control symptoms and prevent relapses. | ||
== See also == | |||
== | |||
* [[Pemphigus vulgaris]] | * [[Pemphigus vulgaris]] | ||
* [[Bullous pemphigoid]] | * [[Bullous pemphigoid]] | ||
* [[Autoimmune blistering disease]] | * [[Autoimmune blistering disease]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
Latest revision as of 00:32, 8 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| IgA pemphigus | |
|---|---|
| |
| Synonyms | |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Blisters, skin erosions, pruritus |
| Complications | Secondary infection |
| Onset | |
| Duration | |
| Types | Subcorneal pustular dermatosis type, Intraepidermal neutrophilic type |
| Causes | Autoimmune reaction involving IgA antibodies |
| Risks | |
| Diagnosis | Skin biopsy, direct immunofluorescence |
| Differential diagnosis | Pemphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis |
| Prevention | |
| Treatment | Dapsone, corticosteroids, immunosuppressive drugs |
| Medication | |
| Prognosis | Variable, often chronic |
| Frequency | Rare |
| Deaths | N/A |
An autoimmune blistering skin disease
IgA pemphigus is a rare autoimmune disease characterized by the presence of blisters on the skin and mucous membranes. It is a subtype of pemphigus, which is a group of autoimmune blistering disorders. Unlike other forms of pemphigus, IgA pemphigus is mediated by immunoglobulin A (IgA) antibodies rather than immunoglobulin G (IgG).
Pathophysiology[edit]
IgA pemphigus is caused by the production of IgA antibodies against components of the epidermis. These antibodies target proteins in the desmosomes, which are structures that help maintain the integrity of the skin by holding keratinocytes together. The binding of IgA antibodies to these proteins leads to the disruption of cell adhesion, resulting in the formation of blisters.
Clinical Presentation[edit]
Patients with IgA pemphigus typically present with erythematous plaques and vesicles that may coalesce into larger blisters. These lesions are often pruritic and can appear on any part of the body, but are commonly found on the trunk and extremities. Unlike other forms of pemphigus, mucosal involvement is less common in IgA pemphigus.
Diagnosis[edit]
The diagnosis of IgA pemphigus is confirmed through a combination of clinical examination, histopathology, and direct immunofluorescence. A skin biopsy will show subcorneal pustules with neutrophilic infiltration. Direct immunofluorescence of perilesional skin will reveal IgA deposition in the epidermis.
Treatment[edit]
The management of IgA pemphigus involves the use of medications to reduce inflammation and suppress the immune response. Dapsone is often the first-line treatment due to its anti-inflammatory and immunomodulatory effects. In cases where dapsone is ineffective or not tolerated, systemic corticosteroids or other immunosuppressive agents may be used.
Prognosis[edit]
The prognosis for patients with IgA pemphigus is generally favorable, especially with appropriate treatment. The disease tends to have a chronic course with periods of remission and exacerbation. Long-term management may be necessary to control symptoms and prevent relapses.
