Treatment of schamberg's disease with pentoxifylline - therapeutic trial

Introduction

Schamberg′s disease (Progressive pigmented purpuric dermatoses) is a capillarits of unknown etiology characterized by orange to fawn-colored macules and plaques usually localized to the lower limbs.[1] Characteristic ′cayenne pepper′ spots due to hemosiderin deposition in the skin are seen at the periphery of the lesions.[2] Histopathology consists of a superficial lymphocytic perivascular inflammation with increased capillaries and siderophages in the upper dermis.[3] The disease follows a chronic course with spontaneous clearance in a few cases. Treatment modalities which have been used include topical and systemic corticosteroids, vitamin C and topical and systemic anti inflammatory agents. Pentoxifylline, a methylxanthine derivatice, has been used successfully in treatment of various types of vasculitides, specially leucocytoclastic vasculitis. A report of its successful use in Schamberg′s disease′ prompted us to conduct a larger trial.

Materials and Methods

Twenty patients presenting with characteristic lesions of Schamberg′s disease confirmed on histopathology were included in the trial. Pentoxifylline was started in a dose of 400 mg once daily. No other topical or systemic treatment was given during the study period. Response to treatment was assessed independently by two observers at 2 week intervals and graded as percentage of clearance of lesions. Improvement was graded as mild (< 25%), moderate (25-50%) and marked (>50%).

Results

The improvement as assessed by two different observers at 2 weekly intervals is as shown in [Table - 1].

Discussion

Successful treatment in Schamberg′s disease presents a veritable challenge to the treating physician. Spontaneous clearance, though well documented, occurs uncommonly. Commonly recommended treatment modalities include physical measures aided at reducing venous pressure (graduated compression stocking), topical and systemic steroids, ascorbic acid, topical and systemic anti -1nflamatory agents and griseofulvin. Pentoxifylline, a methylxanthine derivative, has multiple effects at cellular level including potent rheological properties. This results in increased erythrocyte deformability, immunomodulator action by modifying cytokine profile and antifiroblastic action.[4] Specially interesting is its role.

The improvement as assessed by two different observers at 2 weekly intervals is as shown in [Table - 1] in cutaneous vasculitides such as rheumatoid, urticarial, leucocytoclastic and livedoid vasculitis. Recently, Kano et al have demonstrated a decrease in expression of ICAM-I on endothelial cells of blood vessels of lesional skin following successful treatment with pentoxifylline.[3] This results in decreased exudation of inflammatory cells from capillaries into the sorrounding perivascular tissue. Moreover, pentoxifylline inhibits synthesis and release of TNF-a.[5],[6] This results in reduced TNFa mediated extravasation from the capillaries. Our preliminary study shows marked improvement in 10/20 (50%) patients after 8 weeks of treatment. Pentoxifylline is an economical and easily available drug which is devoid of serious side effects. So we conclude that pentoxifylline should be considered as first line therapy in Schamberg′s disease.