title: Severe, Disfiguring, Pityriasis Rubra Pilaris in a Woman in the Dominican Republic: Histopathologic Diagnosis and Response to Antiretroviral Therapy.
abstract: Pityriasis rubra pilaris (PRP) is a poorly understood dermatologic condition usually accompanied by keratoderma and intense erythroderma with islands of unaffected skin. The PRP categories include HIV-associated PRP VI. A 23-year-old HIV-infected, dark-skinned woman in the Dominican Republic developed an extremely severe, disfiguring process characterized first by a dry scaly rash involving her face, trunk, and extremities with hyperpigmentation and islands of spared skin and minimal erythroderma, followed by alopecia and development of a thick horny layer on the scalp and face. The condition, histologically proven to be PRP, was accompanied by fever, wasting, and decline in CD4 count. Initiation of combination antiretroviral therapy (cART) was followed by rapid and sustained resolution of PRP. Nine years after ART initiation, she remains well, with viral suppression and immune recovery, without PRP recurrence but with sparse hair regrowth and facial scarring. In some dark-skinned patients, severe PRP may not feature characteristic erythroderma but will respond to combination ART.
authors.affiliation: Clínica de Familia La Romana, La Romana, Dominican Republic.
authors.forename: Leonel
authors.initials: L
authors.lastname: Lerebours-Nadal
authors.affiliation: Clínica de Familia La Romana, La Romana, Dominican Republic Robert Stempel College of Public Health and Social Work, Florida International University, Miami, FL, USA cbecksag@fiu.edu.
authors.forename: Consuelo M
authors.initials: CM
authors.lastname: Beck-Sague
authors.affiliation: Department.of Pathology, Emory University School of Medicine, Atlanta, GA, USA.
authors.forename: Douglas
authors.initials: D
authors.lastname: Parker
authors.affiliation: Department.of Pediatric Plastic Surgery, University of California, San Diego Health System, San Diego, CA, USA.
authors.forename: Amanda
authors.initials: A
authors.lastname: Gosman
authors.affiliation: Massachusetts General Hospital, Harvard University, Boston, MA, USA.
authors.forename: Arturo
authors.initials: A
authors.lastname: Saavedra
authors.affiliation: Hospital Buen Samaritano, La Romana, Dominican Republic.
authors.forename: Kristy
authors.initials: K
authors.lastname: Engel
authors.affiliation: Clínica de Familia La Romana, La Romana, Dominican Republic Department of Epidemiology and Public Health, Miller School of Medicine, University of Miami, Miami, FL, USA.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Dean
mesh_terms: D019380:Anti-HIV Agents / Q000009:adverse effects* / Q000627:therapeutic use; D018791:CD4 Lymphocyte Count; D004293:Dominican Republic; D005260:Female; D015658:HIV Infections / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D010916:Pityriasis Rubra Pilaris / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D055815:Young Adult
chemical_list: D019380:Anti-HIV Agents
keywords: AIDS; Dominican Republic; HIV; antiretroviral therapy; pityriasis rubra pilaris