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.
Subject(s)
Antibodies, Monoclonal/therapeutic use , Immunoglobulin G/therapeutic use , Pityriasis Rubra Pilaris/drug therapy , Receptors, Tumor Necrosis Factor/therapeutic use , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Acitretin/adverse effects , Acitretin/therapeutic use , Adult , Antibodies, Monoclonal/adverse effects , Dose-Response Relationship, Drug , Drug Therapy, Combination , Etanercept , Female , Humans , Immunoglobulin G/adverse effects , Infliximab , Keratolytic Agents/adverse effects , Keratolytic Agents/therapeutic use , Male , Middle Aged , Pityriasis Rubra Pilaris/pathology , Retrospective Studies , Severity of Illness Index , Treatment OutcomeABSTRACT
Klippel-Trenaunay (KT) syndrome is a vascular malformation characterized by a port-wine stain, varicose veins and hypertrophy of the affected limb. Ulceration is considered an uncommon complication of KT syndrome and occurrence of skin cancer has been previously reported only in one case. We observed a case of KT syndrome in a 48-year-old woman who developed a large ulcer and a squamous cell carcinoma on the affected leg.
