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Paraneoplastic Pemphigus

Autoimmune bullous disease associated with underlying malignancy with severe mucosal involvement

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Paraneoplastic Pemphigus?

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease with high mortality occurring in the presence of an underlying lymphoproliferative or solid tumor. Castleman disease, non-Hodgkin lymphoma, and chronic lymphocytic leukemia are the most commonly associated malignancies.

Severe, refractory oral mucositis dominates the clinical picture; bloody crusting on the lips, conjunctival involvement, and polymorphic skin lesions resembling pemphigus, pemphigoid, erythema multiforme, and lichen planus are intertwined.

Diagnosis requires clinical findings, plakoglobin, desmoplakin, and desmoglein autoantibodies, and systemic investigation for malignancy. Treatment is challenging and control of the underlying tumor is essential.

Symptoms

Severe and refractory stomatitis, lips with bloody crusts
Conjunctivitis and pseudomembranous eye involvement
Polymorphic skin eruption (pemphigus-pemphigoid-erythema multiforme mixture)
Lichen planus-like plaques on palms and soles
Shortness of breath due to bronchiolitis obliterans
Bullous erosions and painful ulcers
Weight loss and weakness

Risk Factors

Castleman disease
Non-Hodgkin lymphoma
Chronic lymphocytic leukemia
Thymoma
Waldenström macroglobulinemia
Sarcomas and solid tumors

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • If you have refractory, widespread, and painful oral lesions
  • If multiple types of lesions appear together on the skin (bullae + plaques + target lesions)
  • If bloody crusting develops on the eyelids and conjunctiva
  • If shortness of breath or respiratory distress is added
  • If skin eruption begins in known lymphoma history

Treatment Methods

01
Resection or chemotherapy of the underlying tumor
02
High-dose systemic corticosteroids
03
Rituximab and intravenous immunoglobulin (IVIG)
04
Cyclosporine or mycophenolate mofetil
05
Intensive oral care and pain management
06
Pulmonary follow-up for bronchiolitis obliterans
07
Infection prophylaxis and supportive care

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dermatoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.