165 Pigmented Purpura

Fig. 165.2 Close-up of the torso of the child in Fig. 165.1. Petechiae (‘cayenne pepper’ spots) are present at the periphery and within the lesions in Schamberg disease.


Fig. 165.3 Segmental distribution of Schamberg disease in a 3-year-old Asian girl (molluscum inferior to the eruption are an incidental finding).



Although this disorder is not associated with systemic disease, it is a distressing condition because of the cosmetically disfiguring nature of the eruption. Schamberg disease has a chronic course marked by phases of resolution and recurrence. It is notoriously resistant to treatment. Lesions may clear within a few months or may persist for as long as 40 years.

Differential Diagnosis.

Progressive pigmented purpura may be thought to be purpura or ecchymoses. Platelet disorders (decrease in number or functions), coagulopathies, vasculitis, Henoch–Schönlein purpura, cryoglobulinaemia and drug eruptions are included in the differential diagnosis. The lesions in Schamberg disease are macular and never palpable, in contrast to those seen in leucocytoclastic vasculitis. Chronic hyperpigmentation is not a typical feature of Henoch–Schönlein purpura. Referrals to haematologists or rheumatologists are not necessary.


Unfortunately, there is no highly effective treatment for this condition. Time, patience, education and reassurance are the keys to management. PUVA and NB-UVB have been shown to be effective in some cases [14,15] but PUVA therapy should be restricted to (older) adults only. The effects of phototherapy may be related to the involvement of Langerhans cells. Therapies that have been recommended but with variable success include ascorbic acid, antihistamines, topical and systemic corticosteroids and support stockings. Although systemic corticosteroids may be the most effective of all therapies, the disease tends to recur when the medication is discontinued. Additionally, it is questionable whether systemic corticosteroids should be prescribed for a chronic benign condition [16].


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Lichen aureus


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Apr 26, 2016 | Posted by in Dermatology | Comments Off on 165 Pigmented Purpura
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