Treatment of refractory adult-onset pityriasis rubra pilaris with TNF-alpha antagonists: a case series.
J Eur Acad Dermatol Venereol
; 24(8): 881-4, 2010 Aug.
Article
in En
| MEDLINE
| ID: mdl-20002243
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.
Full text:
1
Database:
MEDLINE
Main subject:
Pityriasis Rubra Pilaris
/
Immunoglobulin G
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Tumor Necrosis Factor-alpha
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Receptors, Tumor Necrosis Factor
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Antibodies, Monoclonal
Type of study:
Observational_studies
/
Risk_factors_studies
Limits:
Adult
/
Female
/
Humans
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Male
/
Middle aged
Language:
En
Year:
2010
Type:
Article