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Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption
S R Stevens1, C E Griffiths, G J Anhalt
1Department of Dermatology, University of Michigan, Ann Arbor.
Archives of Dermatology
|July 1, 1993
Summary
Paraneoplastic pemphigus, a rare autoimmune disorder, can present with lichen planus pemphigoides-like eruptions. This finding expands the known clinical spectrum of this serious neoplasia-associated condition.
Area of Science:
- Dermatology
- Autoimmunology
- Oncology
Background:
- Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease.
- It is characterized by severe mucosal ulceration and polymorphous skin eruptions.
- PNP shares immunofluorescence similarities with pemphigus vulgaris but has unique serum binding and immunoprecipitation patterns.
Observation:
- A 63-year-old woman with chronic lymphocytic leukemia developed a severe mucocutaneous eruption.
- The eruption exhibited features of lichen planus pemphigoides, progressing to a Stevens-Johnson-like presentation.
- Immunofluorescence studies revealed findings consistent with pemphigus vulgaris.
Findings:
- Serum evaluation confirmed autoantibodies specific for paraneoplastic pemphigus.
- Indirect immunofluorescence and immunoprecipitation criteria were met.
- The patient's presentation expanded the known clinical and histopathologic spectrum of PNP.
Implications:
- The clinical spectrum of paraneoplastic pemphigus should be broadened.
- Cases presenting as lichen planus pemphigoides-like eruptions should be considered within PNP.
- This expands diagnostic considerations for patients with neoplasia and severe mucocutaneous disease.