Pyoblepharitis Vegetans in Association With Pyodermatitis-Pyostomatitis Vegetans: Expanding the Spectrum of a Rare, Multisystem Disorder.

BACKGROUND: Pyodermatitis-pyostomatitis vegetans (PDPSV) is a rare inflammatory disorder of the skin and oral mucosa that is commonly associated with inflammatory bowel disease (IBD). Ocular involvement is uncommon. OBJECTIVE: Given the association with IBD, it is important for dermatologists to be aware of the varying manifestations of PDPSV. METHODS: A biopsy was performed for diagnosis confirmation, and the literature was reviewed for similar cases. OBSERVATIONS: Our 48-year-old PDPSV patient presented with rare ocular findings. There have been 8 PDPSV reports of ocular involvement. Only 3 of those patients developed blepharitis and/or conjunctival disease. None showed simultaneous involvement of sites at disease onset like our patient. RESULTS: Our patient's concomitant presentation at onset of multiple mucocutaneous sites with blepharitis and conjunctivitis is unique. CONCLUSIONS: We propose the term pyoblepharitis vegetans (PBV) to describe vegetating lesions of the eyelids. The diagnosis of PDPSPBV should be considered with presentation of these signs.

Pemphigus vegetans associated with intranasal cocaine abuse.

BACKGROUND: Pemphigus vegetans is a rare variant of pemphigus vulgaris, comprising 1 to 2% of all pemphigus cases. Exposures to oral agents such as captopril and penicillamine and, less commonly, physical or chemical factors have been implicated in the development of pemphigus. METHODS: We report a 42-year-old white male with a 12-month history of hypertrophic, vegetative plaques affecting primarily his external nares and upper lips. The patient had a history of alcoholism and intermittent drug abuse, primarily intranasal cocaine, since his youth. He had been using cocaine heavily three to four times/week for 1 month prior to and 1 month following the onset of the eruption but has since ceased use. His clinical features and histopathologic findings were consistent with a diagnosis of pemphigus vegetans. Treatment with high-dose prednisone (80 mg/d) and mycophenolate mofetil (1.5 g/d) resulted in resolution of the lesions after 18 months. RESULTS AND CONCLUSIONS: To our knowledge, this is the second report proposing an association between intranasal cocaine use and the pemphigus family of disorders. Although the relationship between illicit drug use and the development of pemphigus is unclear, we postulate that intranasal cocaine abuse is operative in our patient's disease. Herein we discuss drug and other external precipitants of pemphigus and review previous case reports of pemphigus associated with illicit drugs.