eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus, Paraneoplastic: Follow-up

Author: Lynne J Goldberg, MD, Associate Professor, Departments of Dermatology and Pathology, Boston University School of Medicine
Coauthor(s): Nauman Nisar, MD, Fellow, Department of Dermatology, Section of Dermatopathology, Boston University Medical Center
Contributor Information and Disclosures

Updated: Nov 6, 2009

Follow-up

Complications

The main complication of paraneoplastic pemphigus is impaired skin barrier function, which can lead to localized infection, sepsis, and death. Additionally, painful oral and pharyngeal ulceration can interfere with eating, which can compromise nutritional status. Finally, involvement of respiratory tract epithelium can lead to respiratory insufficiency due to bronchiolitis obliterans and can result in death.

Prognosis

In general, the prognosis of paraneoplastic pemphigus is poor; however, the prognosis is somewhat better when the disease is associated with benign tumors.  

The mortality rate when associated with malignancy is estimated at 90%. Nearly all patients with the 2 most common associated tumors, non-Hodgkin lymphoma and chronic lymphocytic lymphoma, are dead of disease within 2 years of diagnosis. Note, however, that outcome does not parallel the course of the underlying malignancy. Both the presence of an underlying neoplasm and the adverse effects of the potent medications required to treat the disease add to both the morbidity and the mortality.

Paraneoplastic pemphigus is the only form of pemphigus that affects epithelia other than squamous. Involvement of respiratory mucosa, which manifests clinically as dyspnea with normal chest radiograph findings, is an ominous finding that progresses via an unknown mechanism to bronchiolitis obliterans. The most recent estimates are that approximately one third of the deaths from paraneoplastic pemphigus are due to pulmonary insufficiency.

Patient Education

As with any serious illness, patients should be made aware of the poor prognosis. For excellent patient education resources, visit eMedicine's Procedures center. In addition, see eMedicine's patient education article Skin Biopsy.

Miscellaneous

Medicolegal Pitfalls

The biggest legal pitfall in paraneoplastic pemphigus is making the correct diagnosis. The eruption is polymorphous. Light microscopy findings can be easily misinterpreted as lichen planus, erythema multiforme, or pemphigus vulgaris; immunofluorescent findings of intercellular deposition of complement exclude the first 2 diagnoses. Immunoprecipitation findings confirm the diagnosis, aided by a high degree of suspicion and careful clinicopathologic correlation. In one study, an initial diagnosis of pemphigus vulgaris was made in 27% of cases.

 


More on Pemphigus, Paraneoplastic

Overview: Pemphigus, Paraneoplastic
Differential Diagnoses & Workup: Pemphigus, Paraneoplastic
Treatment & Medication: Pemphigus, Paraneoplastic
Follow-up: Pemphigus, Paraneoplastic
References
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References

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Further Reading

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Keywords

paraneoplastic pemphigus, PNP, pemphigus

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Contributor Information and Disclosures

Author

Lynne J Goldberg, MD, Associate Professor, Departments of Dermatology and Pathology, Boston University School of Medicine
Lynne J Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, Massachusetts Academy of Dermatology, New England Dermatological Society, North American Hair Research Society, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Coauthor(s)

Nauman Nisar, MD, Fellow, Department of Dermatology, Section of Dermatopathology, Boston University Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Ponciano D Cruz Jr, MD, Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center
Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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