PARANEOPLASTIC PEMPHIGUS

ALTERNATE NAMES

PNP; Paraneoplastic Autoimmune Multi-organ Syndrome

DESCRIPTION

Paraneoplastic Pemphigus (PNP) is a rare disease that demonstrates many of the clinical and laboratory findings of pemphigus vulgaris. It occurs in individuals who have concurrent cancers such as non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, and Castleman disease. The binding of antibodies to the surface of the cells of the outer layer of skin (epidermis) causes pemphigus. When the disease involves the airways, it can cause fatal respiratory disease. PNP is characterized by severe ulceration (blistering) of the mouth, lips, skin, and may involve the esophagus, conjunctiva and other mucous membranes.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

There is no single test to confirm a diagnosis of PNP. Because PNP is a rare disease of the skin, a dermatologist is consulted to diagnose and treat this disease. The dermatologist will document the appearance and location of the blisters. Direct immunofluorescence (on skin or mucosal biopsies) and indirect immunofluorescence (on blood) are done to identify the antibodies and the type of pemphigus causing the skin blisters. While a tumor is essential in the diagnosis, people with tumors other than lymphoproliferative neoplasm can develop paraneoplastic pemphigus. These include thymoma, sarcoma, and lung carcinoma.

ICD 9: 694.4

ONSET AND PROGRESSION

Men and women are equally affected by PNP. Research suggests a genetic predisposition for the disease. PNP can occur in adults and children ranging in age from 7 to 76 years of age. The average age of onset in adults is between 50-60 years of age. In children, paraneoplastic pemphigus is often the presenting sign of Castleman disease, and an increased incidence of bronchiolitis obliterans is evident. PNP is often fatal.

TREATMENT

The primary goals of treatment in PNP are to treat malignancy, to decrease blister formation and to promote healing of lesions. Immunosuppressive therapies control the disease. Prescribed corticosteroid medication like prednisone and immunosuppressive drugs like azathioprine treat the blistering. Complete removal of the tumor may improve the skin disease, but damage to the lungs may be irreversible. The major causes of death for people with PNP is respiratory failure and infections.

Suggested MER for Evaluation:

• Clinical examination describing physical findings, dermatology and oncology consultation reports documenting disease progression and response to treatment, and

• laboratory testing reports documenting paraneoplastic pemphigus serum antibody screen, and results of biopsies are needed to confirm the diagnosis.

Suggested Listings for Evaluation:

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