Purpura annularis telangiectodes of Majocchi: case report and review of the literature

Abstract

Background: Purpura annularis telangiectodes of Majocchi is an uncommon form of pigmented purpuric dermatosis which may present a therapeutic challenge. Given the rare nature of this condition, there is limited anecdotal information available regarding optimal therapy. Although pigmented purpuric dermatoses are generally innocuous, in some cases they may cause patients significant distress, and there is a need to exclude cutaneous T-cell lymphoma.

Methods: We reviewed the literature on the treatment of pigmented purpuric dermatoses and managed a 69-year-old woman who presented with purpuric annular patches on the legs.

Results: Three separate biopsies demonstrated an interstitial to perivascular lymphocytic infiltrate with erythrocyte extravasation, consistent with pigmented purpuric dermatosis. The patient's condition proved refractory to many of the previously reported modes of management, but markedly improved with methotrexate. Treatment alternatives for pigmented purpuric dermatosis are reviewed, and a treatment algorithm is proposed.

Conclusion: This is the first reported case regarding the successful use of methotrexate for pigmented purpuric dermatosis. Methotrexate may offer a therapeutic alternative to patients with highly symptomatic pigmented purpuric dermatosis refractory to other, more conservative, treatment modalities.