This 60+ year-old gentleman presents after being seen in another dermatology office for ulcerated plaques, some with excessive eschar, on the face, trunk and extremities. He reports a 3 month history of skin lesions, treated with short bursts of prednisone and topical steroids only, resulting in no improvement and instead with disease progression. He has a history of laryngeal carcinoma, is post-chemotherapy, radiation, and surgery, and now has a permanent tracheostomy.
Patient was evaluated and a biopsy was performed.
What is the likely correct diagnosis and other differential diagnosis you should consider?
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This is pemphigus vulgaris. Rule out paraneoplastic pemphigus in a patient with a history of past malignancy.
Two (2) biopsies for standard H&E and direct immunofluorescence (DIF) studies were obtained. H&E stained sections show intraepidermal acantholysis of keratinocytes with a prominent suprabasalar split. In the superficial dermis there is an infiltrate of lymphocytes mixed with occasional scattered eosinophils. The DIF studies show intracellular and cell surface deposition of IgG and C3 in the epidermis, consistent with the clinical impression of pemphigus vulgaris.
DIAGNOSIS & CASE SUMMARY:
In addition to biopsy, a PET CT was ordered in consultation with the patient’s prior oncologist. As comfort and pain control were extremely important as well as wound care and treatment, high dose systemic corticosteroids were initiated with a topical regimen while metastatic workup was pursued. Biopsy confirmed pemphigus vulgaris and metastatic disease workup was negative. Mycophenolate mofetil was initiated along with steroid course, as the patient refused inpatient care or infusions.
After 2 weeks on high dose steroids with mycophenolate mofetil with minimal improvement and trouble with wound care and pain management, the patient was eventually convinced to initiate inpatient care as well as Rituximab infusions. The patient is improving on Rituximab.
By: Dr. Betsy Wernli, MD and Dr. Ling Xia, MD