progressive pigmentary dermatosis; Schamberg's purpura; pigmented purpuric dermatosis; purpura annularis telangiectodes of Majocchi; cutaneous T-cell lymphoid dyscrasia; capillaritis (CTLD)

Etiology

• unknown
• venous hypertension, lack of exercise, & gravitational dependency are cofactors

Epidemiology

• uncommon in children

Pathology

• benign disorder
• leaky capillaries
• perivascular T-cell lymphocytic infiltrate centered on superficial small blood vessels of the skin
• signs of endothelial cell swelling & narrowing of the lumen
• extravasation of red blood cells with marked hemosiderin deposition in macrophages

* histopathology images

Clinical manifestations

• progressive pigmented purpura
• cutaneous petechiae & bronze discolorations located predominantly on the leg, with many clinical variations
• lower extremities affected in all cases
• lesions may improve in 1/4-1/3 of patients with 1-6 years

* images

Laboratory

normal

Complications

• development of cutaneous T-cell lymphoma

Management

• no treatment indicated

More general terms

• purpura; retiform purpura
• pigmentation disorder