Introduction: We report a case of a 61-year-old woman with locally destructive vulvar pyoderma gangrenosum (PG) with pulmonary involvement who was refractory to numerous systemic therapies and developed complications resulting in her demise.
Objective: To report a rare case of treatment-resistant vulvar PG with pulmonary involvement that proved to be fatal.
Methods: PubMed was used to search for other reports that discuss PG, or more specifically perigenital PG, with pulmonary involvement.
Background: Pityriasis rubra pilaris (PRP) is a rare dermatosis of unknown etiology. Most cases of PRP are sporadic; however, rare cases of familial PRP have been reported.
Objectives: To present a case of PRP inherited in an autosomal dominant (AD) fashion and to evaluate the current literature on familial PRP and formulate a comprehensive, up-to-date summary of this rare condition.