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.

OBJECTIVES:

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: S Garcovich, A R Di Giampetruzzi, G Antonelli, A Garcovich, B Didona
• Journal: Journal of the European Academy of Dermatology and Venereology : JEADV (J Eur Acad Dermatol Venereol) Vol. 24 Issue 8 Pg. 881-4 (Aug 2010) ISSN: 1468-3083 [Electronic] England
• PMID: 20002243 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal
• Immunoglobulin G
• Keratolytic Agents
• Receptors, Tumor Necrosis Factor
• Tumor Necrosis Factor-alpha
• Infliximab
• Acitretin
• Etanercept

Topics

• Acitretin (adverse effects, therapeutic use)
• Adult
• Antibodies, Monoclonal (adverse effects, therapeutic use)
• Dose-Response Relationship, Drug
• Drug Therapy, Combination
• Etanercept
• Female
• Humans
• Immunoglobulin G (adverse effects, therapeutic use)
• Infliximab
• Keratolytic Agents (adverse effects, therapeutic use)
• Male
• Middle Aged
• Pityriasis Rubra Pilaris (drug therapy, pathology)
• Receptors, Tumor Necrosis Factor (therapeutic use)
• Retrospective Studies
• Severity of Illness Index
• Treatment Outcome
• Tumor Necrosis Factor-alpha (antagonists & inhibitors)