A case of Schamberg's disease responding dramatically to PUVA treatment

Abstract

Pigmented purpuric dermatoses are a group of chronic, recurrent disorders characterized by purpuric lesions mainly involving the lower extremities. Their etiology is unknown. Treatment options are limited and none of them have proven benefit. Phototherapy has been reported to be effective in a small number of patients in the literature. We present a case of Schamberg's disease showing a dramatic response to psoralen plus ultraviolet A therapy and discuss the current therapeutic options focusing mainly on phototherapy. We believe that phototherapy is a valuable alternative, especially for patients with long-standing and widespread pigmented purpuric dermatitis.

Treatment of pigmented purpuric dermatoses with Narrow‐band UVB: a report of six cases

“…Schamberg’s disease is the most common variant and is characterized by aggregation of pinhead‐sized petechiae resembling grains of cayenne pepper together with golden‐brown pigmentation. The lesions favour the extremities and only rarely the trunk is involved 3 . Majocchi’s disease – an uncommon variant of PPD – is characterized by symmetrical, annular patches, including punctuate red‐brown macules, with a predilection for the lower extremities 4 .…”

“…A cohort of 11 patients with PPD were successfully treated with PUVA, five of whom had Schamberg’s disease, five had pigmented purpuric lichenoid dermatosis (Gougerot‐Blum) and one had eczematoid‐like purpura of Doucas and Kapetanakis 20 . More recently, lichen aureus 5 and Schamberg’s disease 3 were effectively treated with PUVA in two case reports. The immune modulatory effect of phototherapy in PPD is further supported by the finding of Ghersetich et al.…”

“…The lesions favour the extremities and only rarely the trunk is involved. 3 Majocchi's disease -an uncommon variant of PPD -is characterized by symmetrical, annular patches, including punctuate red-brown macules, with a predilection for the lower extremities. 4 The disease affects predominantly children and young adult.…”

“…The disease entity remains an enigma and a therapeutic challenge 1 . The options of treatments are limited and none of them has proven benefit 3 . In this study, we used narrow‐band ultraviolet B (NB UVB) to treat patients with PPD.…”

A case of pigmented purpuric dermatosis similar to mycosis fungoides

“…The patient was recovered by these treatments, his itching complaints and PPD lesions were completely resolved at the end of first month of the treatment. [10][11][12][13][14]. This study shows that purpuric pigmented dermatitis can be associated to or evolve into lymphoproliferative disease and mycosis fungoides.…”