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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 [3] Clinical manifestations: 1) progressive pigmented purpura 2) cutaneous petechiae & bronze discolorations located predominantly on the leg, with many clinical variations 3) lower extremities affected in all cases 4) lesions may improve in 1/4-1/3 of patients with 1-6 years * images [3,5] Laboratory: normal Complications: - development of cutaneous T-cell lymphoma Management: - no treatment indicated

General

purpura; retiform purpura pigmentation disorder

References

  1. Draelos ZK, Hansen RC. Schamberg's purpura in children: case study and literature review. Clin Pediatr (Phila). 1987 Dec;26(12):659-61. Review. PMID: 3315387
  2. Torrelo A, Requena C, Mediero IG, Zambrano A. Schamberg's purpura in children: a review of 13 cases. J Am Acad Dermatol. 2003 Jan;48(1):31-3. PMID: 12522367
  3. Mehregan D, Elston DM (images) eMedicine: Pigmented Purpuric Dermatitis http://emedicine.medscape.com/article/1084594-overview
  4. Magro CM et al. Pigmented purpuric dermatosis: Classification by phenotypic and molecular profiles. Am J Clin Pathol 2007 Aug; 128:218 PMID: 17638655
  5. DermNet NZ. Capillaritis (images) http://www.dermnetnz.org/vascular/capillaritis.html