Fatal case of vulvar pyoderma gangrenosum with pulmonary involvement: case presentation and literature review.

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. RESULTS AND CONCLUSION: A thorough review of the literature revealed 33 cases of PG with pulmonary involvement, with only 4 involving the perigenital region. We report the second case of a female with vulvar PG and pulmonary involvement. In contrast to the first case described, our patient did not respond to systemic therapy, and, ultimately, her disease was fatal. It is hoped that with continued documentation of this rare and potentially lethal presentation of PG, physicians will determine more effective treatments.

Familial pityriasis rubra pilaris: case report and review.

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. METHODS: PubMed was used to conduct a search for articles pertaining to familial PRP published through May 2011. RESULTS: The first documented case was published in 1910, and 36 subsequent familial cases of PRP have been reported. Familial PRP typically presents very early in childhood, has a gradual onset, and persists throughout life. Given the rarity of this subtype, determining the best therapy has been a challenge. In the pediatric population, a conservative treatment approach, including topical therapy, is frequently used, whereas systemic treatments are reserved for patients with a severe disease that is refractory to therapy. CONCLUSION: Rare cases of PRP inherited in an AD fashion have been described and tend to have a chronic clinical course and are treatment refractory. Therefore, the awareness of familial PRP is important for early and accurate diagnosis and administration of appropriate therapy.