title: Treatment of refractory adult-onset pityriasis rubra pilaris with TNF-alpha antagonists: a case series.
abstract: BACKGROUND
  Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis with frequent clinical presentation as erythroderma. Conventional systemic treatment is often unsatisfactory and limited by long-term toxicity. The use of tumour necrosis factor (TNF) antagonists has been reported previously in single cases, but lacking long-term follow-up or comparison between different biological agents.
  
  
  OBJECTIVE
  To assess the long-term efficacy and safety of TNF-alpha antagonist, infliximab and etanercept, either in monotherapy or in combination therapy of severe, refractory adult-onset PRP.
  
  
  METHODS
  Seven patients of adult-onset PRP, six newly diagnosed type-I and 1 type-II, which were resistant or ineligible to conventional systemic treatment, received a single course of infliximab or etanercept therapy, alone or in combination with low-dose acitretin (>0.25 mg/kg/daily). After complete remission and treatment discontinuation, a follow-up period of 12 months was evaluated for relapses.
  
  
  RESULTS
  Six patients obtained complete remission after a single course of anti-TNF-alpha therapy: mean therapy duration was 19.3 weeks (range 6-48 weeks). All patients obtained significant clearing (>75% of body surface area) of skin lesions at week 12. Two patients with marked keratoderma developed localized disease recurrence during treatment. During follow-up, only a single patient, affected by type II PRP, had disease relapse.
  
  
  CONCLUSIONS
  Both TNF-alpha antagonists proved successful for the treatment of refractory, adult-onset PRP, yielding complete and persistent clinical responses in type-I PRP. Infliximab was associated with a more rapid onset of action, while treatment duration was comparable with etanercept. PRP type II warranted long-term therapy and showed relapse after drug discontinuation.
authors.affiliation: Department of Dermatology, Policlinico Agostino Gemelli, Catholic University Sacred Heart, Rome, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Garcovich
authors.forename: A R
authors.initials: AR
authors.lastname: Di Giampetruzzi
authors.forename: G
authors.initials: G
authors.lastname: Antonelli
authors.forename: A
authors.initials: A
authors.lastname: Garcovich
authors.forename: B
authors.initials: B
authors.lastname: Didona
mesh_terms: D017255:Acitretin / Q000009:adverse effects / Q000627:therapeutic use; D000328:Adult; D000911:Antibodies, Monoclonal / Q000009:adverse effects / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000009:adverse effects / Q000627:therapeutic use*; D000069285:Infliximab; D007641:Keratolytic Agents / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010916:Pityriasis Rubra Pilaris / Q000188:drug therapy* / Q000473:pathology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D000911:Antibodies, Monoclonal; D007074:Immunoglobulin G; D007641:Keratolytic Agents; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab; D017255:Acitretin; D000068800:Etanercept