Atypical presentation of Schamberg’s disease – diagnostic challenges

Abstract

Introduction. Pigmented purpuric dermatoses (purpura simplex; inflammatory purpura without vasculitis) constitute a heterogenic group of inflammatory skin diseases. Objective. To present a patient with extensive skin lesions secondary to pigmented purpuric dermatosis, Schamberg's disease type. Case report. A 34-year-old man patient was admitted to the Dermatology Clinic in Krakow, Poland, due to disseminated pigmented purpuric lesions. Schamberg's disease was diagnosed on the basis of clinical, dermoscopic and histopathological examinations. The treatment involved preparations improving venous microcirculation, antihistamine drugs, and a series of UVB 311 treatment sessions-partial improvement was achieved. Conclusions. An overview of literature and the presented case show that diagnosing pigmented purpuric dermatoses constitutes a challenge in everyday dermatological practice. The patients require a deepend diagnostic analysis and follow-up also because of possible coexistence or risk of future development of mycosis fungoides. Achieving a therapeutic effects is difficult. Treatment decisions are adjusted individually. streszczenie Wprowadzenie. Postępujące plamice barwnikowe (pigmented purpuric dermatoses, purpura simplex; inflammatory purpura without vasculitis) to heterogenna grupa chorób zapalnych skóry. Cel pracy. Opis przypadku pacjenta z rozległymi zmianami skórnymi w przebiegu postępującej plamicy barwnikowej typu choroby Schamberga. Opis przypadku. Przedstawiono 34-letniego pacjenta, który zgłosił się do krakowskiej Kliniki Dermatologii z powodu rozsianych plamiczych Case report/Opis przypadku Atypical presentation of schamberg's disease-diagnostic challenges nietypowy obraz choroby schamberga-trudności diagnostyczne

Lichen sclerosus mimicking mycosis fungoides preceding B-cell lymphoma – diagnostic challenges

“…Diversity of the presentation of the disease in the poikylodermic variant necessitates the diagnosis of MF also in atypical cases of progressive pigmented purpuric dermatoses (PPD). Clinical factors that lead to the diagnosis of MF in patients with PPD symptoms include: progressive nature of lesions, a large percentage of the skin area covered by purpura, and duration of the disease exceeding 1 year [12]. The relationship between MF and PPD is a rare but recognized phenomenon.…”

“…Qualitative expression of CD markers is expressed by the CD4 : CD8 ratio, which is 2 : 1. It is typically increased czynników klinicznych, które naprowadzają na rozpoznanie MF u chorych z objawami PPD, należą: postępujący charakter zmian, duży procent powierzchni skóry zajętej przez zmiany plamicze oraz czas trwania choroby przekraczający rok [12]. Związek między MF a PPD jest rzadkim, jednak uznanym zjawiskiem.…”

Therapeutic Approach in Pigmented Purpuric Dermatoses—A Scoping Review

“…Combinations of topical steroids with other agents, such as oral vitamin C [ 47 ] or vitamin C and rutoside [ 48 ] have been proven ineffective. Partial temporary responses were reported in patients undergoing a combined treatment of topical steroids with oral antihistaminic drugs [ 49 ] and antihistaminic drugs, vitamin C, rutoside, diosmin, and hesperidin combination [ 49 ].…”

“…Narrowband UVB therapy (UVB-NB) has proven successful in the past for treating Schamberg disease [ 2 , 37 , 46 , 49 , 76 , 77 , 78 , 79 ]. The majority of cases demonstrated a positive response, with only a few instances of recurrence that were effectively addressed through retreatment [ 78 ].…”

“…Another successful combination involved vitamin C and calcium pantothenate [ 85 ]. More intricate drug combinations, such as diosmin/hesperidin/Euphorbia prostata extract/calcium dobesilate [ 86 ] or antihistaminic drug/topical steroid/ascorbic acid/rutoside/hesperidin/diosmin [ 49 ], also resulted in remission. Regarding other forms of PPD, a male patient suffering from eczematoid purpura of Doucas and Kapetanakis [ 87 ] benefited from the standard treatment combination of 500 mg of ascorbic acid twice a day and 50 mg of the bioflavonoid rutoside twice a day.…”