Paraneoplastic pemphigus: Difference between revisions
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{{Infobox medical condition | |||
| name = Paraneoplastic pemphigus | |||
| synonyms = [[Paraneoplastic autoimmune multiorgan syndrome]] | |||
| field = [[Dermatology]], [[Oncology]] | |||
| symptoms = Painful [[mucosal]] erosions, [[skin]] blisters, [[oral]] lesions | |||
| complications = [[Bronchiolitis obliterans]], [[infection]], [[sepsis]] | |||
| onset = Typically in [[adulthood]] | |||
| duration = Chronic | |||
| causes = Associated with [[neoplasms]] such as [[non-Hodgkin lymphoma]], [[chronic lymphocytic leukemia]], [[Castleman disease]], [[thymoma]], [[sarcoma]] | |||
| risks = Presence of [[malignancy]] | |||
| diagnosis = [[Clinical examination]], [[biopsy]], [[immunofluorescence]] | |||
| differential = [[Pemphigus vulgaris]], [[Stevens-Johnson syndrome]], [[toxic epidermal necrolysis]] | |||
| treatment = [[Immunosuppressive therapy]], [[corticosteroids]], [[rituximab]], [[plasmapheresis]] | |||
| prognosis = Variable, often poor due to underlying malignancy | |||
| frequency = Rare | |||
}} | |||
'''Paraneoplastic Pemphigus''' is a rare, multiorgan autoimmune disease that is associated with malignancies. It is characterized by painful mucosal erosions and polymorphous skin lesions. The disease is often associated with lymphoproliferative disorders such as non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman's disease. | '''Paraneoplastic Pemphigus''' is a rare, multiorgan autoimmune disease that is associated with malignancies. It is characterized by painful mucosal erosions and polymorphous skin lesions. The disease is often associated with lymphoproliferative disorders such as non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman's disease. | ||
==Etiology== | ==Etiology== | ||
The exact cause of Paraneoplastic Pemphigus is unknown. However, it is believed to be an autoimmune disorder, which means the body's immune system mistakenly attacks healthy cells. In the case of Paraneoplastic Pemphigus, the immune system attacks the skin and mucous membranes. | The exact cause of Paraneoplastic Pemphigus is unknown. However, it is believed to be an autoimmune disorder, which means the body's immune system mistakenly attacks healthy cells. In the case of Paraneoplastic Pemphigus, the immune system attacks the skin and mucous membranes. | ||
==Symptoms== | ==Symptoms== | ||
The symptoms of Paraneoplastic Pemphigus can vary greatly from person to person. However, common symptoms include: | The symptoms of Paraneoplastic Pemphigus can vary greatly from person to person. However, common symptoms include: | ||
* Painful sores in the mouth and on the lips | * Painful sores in the mouth and on the lips | ||
* Skin rashes, often on the chest, back, and abdomen | * Skin rashes, often on the chest, back, and abdomen | ||
| Line 14: | Line 26: | ||
* Severe conjunctivitis | * Severe conjunctivitis | ||
* Difficulty swallowing due to sores in the esophagus | * Difficulty swallowing due to sores in the esophagus | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of Paraneoplastic Pemphigus often involves a combination of clinical findings, histopathology, immunofluorescence studies, and serology. A skin biopsy is often performed to confirm the diagnosis. | Diagnosis of Paraneoplastic Pemphigus often involves a combination of clinical findings, histopathology, immunofluorescence studies, and serology. A skin biopsy is often performed to confirm the diagnosis. | ||
==Treatment== | ==Treatment== | ||
Treatment of Paraneoplastic Pemphigus is primarily aimed at managing the symptoms and treating the underlying malignancy. This may involve the use of corticosteroids, immunosuppressive drugs, and other therapies. | Treatment of Paraneoplastic Pemphigus is primarily aimed at managing the symptoms and treating the underlying malignancy. This may involve the use of corticosteroids, immunosuppressive drugs, and other therapies. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for individuals with Paraneoplastic Pemphigus is generally poor, with a high mortality rate. The prognosis is often dependent on the underlying malignancy and the individual's response to treatment. | The prognosis for individuals with Paraneoplastic Pemphigus is generally poor, with a high mortality rate. The prognosis is often dependent on the underlying malignancy and the individual's response to treatment. | ||
==See Also== | ==See Also== | ||
* [[Autoimmune diseases]] | * [[Autoimmune diseases]] | ||
* [[Non-Hodgkin's lymphoma]] | * [[Non-Hodgkin's lymphoma]] | ||
* [[Chronic lymphocytic leukemia]] | * [[Chronic lymphocytic leukemia]] | ||
* [[Castleman's disease]] | * [[Castleman's disease]] | ||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
[[Category:Skin diseases]] | [[Category:Skin diseases]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
{{stub}} | {{stub}} | ||
{{No image}} | {{No image}} | ||
Latest revision as of 01:32, 4 April 2025
| Paraneoplastic pemphigus | |
|---|---|
| Synonyms | Paraneoplastic autoimmune multiorgan syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Painful mucosal erosions, skin blisters, oral lesions |
| Complications | Bronchiolitis obliterans, infection, sepsis |
| Onset | Typically in adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Associated with neoplasms such as non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman disease, thymoma, sarcoma |
| Risks | Presence of malignancy |
| Diagnosis | Clinical examination, biopsy, immunofluorescence |
| Differential diagnosis | Pemphigus vulgaris, Stevens-Johnson syndrome, toxic epidermal necrolysis |
| Prevention | N/A |
| Treatment | Immunosuppressive therapy, corticosteroids, rituximab, plasmapheresis |
| Medication | N/A |
| Prognosis | Variable, often poor due to underlying malignancy |
| Frequency | Rare |
| Deaths | N/A |
Paraneoplastic Pemphigus is a rare, multiorgan autoimmune disease that is associated with malignancies. It is characterized by painful mucosal erosions and polymorphous skin lesions. The disease is often associated with lymphoproliferative disorders such as non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman's disease.
Etiology[edit]
The exact cause of Paraneoplastic Pemphigus is unknown. However, it is believed to be an autoimmune disorder, which means the body's immune system mistakenly attacks healthy cells. In the case of Paraneoplastic Pemphigus, the immune system attacks the skin and mucous membranes.
Symptoms[edit]
The symptoms of Paraneoplastic Pemphigus can vary greatly from person to person. However, common symptoms include:
- Painful sores in the mouth and on the lips
- Skin rashes, often on the chest, back, and abdomen
- Blisters on the skin and mucous membranes
- Severe conjunctivitis
- Difficulty swallowing due to sores in the esophagus
Diagnosis[edit]
Diagnosis of Paraneoplastic Pemphigus often involves a combination of clinical findings, histopathology, immunofluorescence studies, and serology. A skin biopsy is often performed to confirm the diagnosis.
Treatment[edit]
Treatment of Paraneoplastic Pemphigus is primarily aimed at managing the symptoms and treating the underlying malignancy. This may involve the use of corticosteroids, immunosuppressive drugs, and other therapies.
Prognosis[edit]
The prognosis for individuals with Paraneoplastic Pemphigus is generally poor, with a high mortality rate. The prognosis is often dependent on the underlying malignancy and the individual's response to treatment.
