Paraneoplastic Pemphigus : All you should know about Paraneoplastic Pemphigus (PNP)

Sponsored Links

Paraneoplastic pemphigus (PNP ) disorder is characterized with the uniform distribution of painful sores on the lips, mouth, and the esophagus. Unlike other forms of pemphigus, this severe tumor form basically invades all over the lungs leading to constrictive bronchiolitis (bronchiolitis obliterans). Although, the skin disease can be completely cured by the complete surgical removal of the tumor, however PNP disorder generally causes an irreparable damage to the lungs.

Read About

What is paraneoplastic pemphigus?

Paraneoplastic pemphigus (PNP) is an auto-immune, mucocutaneous condition that is indirectly associated with a pre-existing lympho-proliferative disorder. Paraneoplastic pemphigus usually develops rarely and is the most severe form of pemphigus. In general, paraneoplastic conditions are health complications associated with a cancer disease. Hence, PNP disorder is one of the cancer complications, especially Castleman's disease (CD) and lymphoma. It usually manifests before diagnosing the associated tumors.

Paraneoplastic pemphigus (PNP ) disorder is characterized with the uniform distribution of painful sores on the lips, mouth, and the esophagus. Unlike other forms of pemphigus, this severe tumor form basically invades all over the lungs leading to constrictive bronchiolitis (bronchiolitis obliterans). Although, the skin disease can be completely cured by the complete surgical removal of the tumor, however PNP disorder generally causes an irreparable damage to the lungs.

Paraneoplastic pemphigus (PNP) is a mucocutaneous disorder characterized with the formation of tumors. Apart from lymphoproliferative neoplasms, PNP condition can also develop in patients bearing various other tumors such as sarcoma, thymoma, and lung carcinoma.

Who is affected by paraneoplastic pemphigus?

Race
Paraneoplastic pemphigus has no apparent racial predilection.
Sex
paraneoplastic pemphigus equally affects both men and women.
Age
An average onset of age for paraneoplastic pemphigus is around 60 years. Although, the PNP disorder may develop in children, however it also affects older age group patients ranging from 7 to 76 years. The PNP condition basically indicates Castleman disease in children and thus increases the risk of developing bronchiolitis obliterans.

What are the signs and symptoms ?

Signs and symptoms of paraneoplastic pemphigus

http://www.youtube.com/watch?v=z3PaMeUksMA

Paraneoplastic pemphigus disorder commonly presents with the following signs and symptoms:
Mucosal erosions that usually develop on the lips, esophagus and oral cavity which could be often painful leading to progressive stomatitis.

Sometimes the condition is accompanied by skin eruptions which eventually progresses to form erosions and blisters in association with an occult malignancy

Causes of paraneoplastic pemphigus

It is a disorder of autoimmune origin that is basically triggered by an underlying lymphoproliferative malignancy. It has been reported that tumor antigens can stimulate both cellular as well as humoral immune response thus evoking the blister formation in the mucosal and other epithelial linings. Commonly affected systems include the gastrointestinal tract, respiratory tract, and the integument. It has also been reported that the benign tumors are often accompanied by a paraneoplastic pemphigus disorder.

Complications

Paraneoplastic pemphigus is a serious life threatening disorder and can prove fatal, particularly when accompanied by a malignant neoplastic disease. It causes serious complications that can eventually lead to death. Some of the serious PNP associated complications that can cause death include respiratory failure as the tumor involves the epithelium of the respiratory tract, sepsis, with an eventual multi-organ failure. The lung associated tumors with significant pulmonary involvement have been increasingly found in nearly 30-40 percent of cases. The patients become susceptible to infections due to lack of skin integrity which is usually predisposed by the potent immunosuppressive agents that are administered to treat the PNP disorder.

Investigations

The paraneoplastic pemphigus disorder can be diagnosed to detect the following abnormalities:
Histopathology examinations to determine an interface or lichenoid dermatitis and demonstrate altered cell acantholysis.

Immunoprecipitation test :

DIF observation to demonstrate immunoreactants
IDIF observation to demonstrate circulating antibodies

What are the treatments of paraneoplastic pemphigus

The treatment for pulmonary disease associated paraneoplastic pemphigus includes administration of the most potent immune suppressant drugs such as azathioprine, methylprednisolone, cyclosporin, and thalidomide as it inhibits the rapid development of bronchiolitis obliterans. Plasmapheresis is also helpful in certain cases.

SUGGESTED READING :

About the Author

Dr Chaitra S's picture
Author: Dr Chaitra S

You May Also Like..