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Paraneoplastic cicatricial pemphigoid.

J Setterfield¹, P J Shirlaw, Z Lazarova

¹St John's Institute of Dermatology, St Thomas' Hospital (GKT), London SE1 7EH, UK. j.setterfield2umds.ac.uk

The British Journal of Dermatology

|July 27, 1999

Summary

This study details the first reported case of paraneoplastic cicatricial pemphigoid (CP) in a lung cancer patient. Treatment of the non-small cell lung carcinoma with gemcitabine improved both cancer and CP symptoms.

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Area of Science:

Oncology
Dermatology
Immunology

Background:

Cicatricial pemphigoid (CP) is a rare autoimmune blistering disease.
Association between CP and malignancy is infrequently reported.
Antiepiligrin antibodies define a subset of CP.

Observation:

A 39-year-old woman presented with antiepiligrin CP.
Mucosal lesions showed poor response to immunosuppressants.
Non-small cell lung carcinoma was diagnosed.

Findings:

Chemotherapy with gemcitabine led to significant reduction in tumor burden and blistering.
Recurrent oral erosions correlated with metastatic disease progression.
This represents the first reported case of paraneoplastic CP.

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Implications:

Suggests a paraneoplastic etiology for CP in select cases.
Highlights the potential benefit of treating the underlying malignancy for CP.
Warrants further investigation into the link between CP and non-small cell lung carcinoma.