Abstract

A 56-year-old female presented with a three-month history of nasal redness and swelling recalcitrant to topical antibiotic and antifungal treatments. Examination revealed erythema, prominent edema, serum-crusting, and pustules involving the nasal tip and alae. Punch biopsy demonstrated pseudoepitheliomatous hyperplasia, intraepidermal eosinophilic/neutrophilic microabscesses, focal acantholysis, and a mixed infiltrate with abundant eosinophils. No vasculitis was identified. Ancillary studies evaluating for infection, or a lymphoproliferative disorder were non-revealing. An immunobullous disorder like pemphigus vegetans (PV) was raised and the possibility of recreational drug use was suggested. Subsequent laboratory studies showed peripheral eosinophilia, positive IgA, IgE, ANA and c-ANCA (PR3) antibodies, and negative tissue cultures. A urine drug screen was positive for cocaine. The patient was lost to follow-up and a biopsy for direct immunofluorescence studies (DIF) could not be obtained.

Cocaine-associated midline lesions can be under-recognized as patients may deny drug use. The association of PV with intranasal recreational drug use is rarely reported in the literature and the etiology remains unclear. Clinically, patients have vegetative plaques, erosions, pustules, and swelling of the nares, lips, and oral mucosa. Histologically, there is suprabasilar acantholysis, epidermal hyperplasia, intraepidermal eosinophilic and neutrophilic pustules, and an eosinophil-rich infiltrate. DIF studies are less consistent than idiopathic disease, showing intercellular deposition of C3 with or without faint IgG. Treatment includes cessation of the drug and systemic steroids.

As clinical histories may be unreliable, dermatopathologists and dermatologists should be aware of the association of cocaine with midline vegetative nasal lesions including PV to prompt confirmatory testing and avoid misdiagnosis.