Approach to the patient with retiform (angulated) purpura
- Robert Kelly, MD
Robert Kelly, MD
- Head of Clinical Dermatology, Department of Dermatology
- St Vincent’s Hospital / Melbourne University
Purpura are nonblanchable, hemorrhagic skin lesions that result from the leakage of red blood cells into the skin. The term "retiform purpura" describes lesions that demonstrate an angulated or branched configuration (picture 1A-C). Retiform purpura can occur in a variety of disorders; thus, identifying the underlying cause is an important component of patient management.
The potential causes of retiform purpura and the approach to the assessment of patients with these lesions will be reviewed here. Additional details on the pathogenesis, clinical manifestations, and treatment of disorders that present with retiform purpura are discussed elsewhere in UpToDate. (See 'Associated disorders' below.)
Retiform purpura develop as a consequence of complete vascular occlusion and vascular damage involving blood vessels in the skin. As in livedo reticularis (picture 2A-B) and livedo racemosa (picture 3) (additional disorders associated with vasculopathy or vasculitis), the angulated or branched shape of retiform purpura reflects the vascular architecture in the skin (see 'Differential diagnosis' below). The hemorrhagic appearance of purpura results from the local extravasation of red blood cells. Often, skin necrosis due to infarction is present.
Associated disorders — Multiple noninflammatory and inflammatory vasculopathic disorders may present with retiform purpura. Lesions may occur in the setting of intravascular abnormalities in which thrombi, proteins, or emboli obstruct cutaneous vessels, or may involve direct damage to vessel walls, as occurs in vasculitis, calciphylaxis, and some severe opportunistic infections.
Intravascular abnormalities — As noted above, thrombosis, intravascular deposition of abnormal proteins, and embolic phenomena may result in the development of retiform purpura.
- Levi M, Ten Cate H. Disseminated intravascular coagulation. N Engl J Med 1999; 341:586.
- Levi M. Current understanding of disseminated intravascular coagulation. Br J Haematol 2004; 124:567.
- Harris, EN. Antiphospholipid syndrome. In: Rheumatology, 2nd ed, Klippel, JH, Dieppe, PA (Eds), Mosby, London, UK 1998. p.7.35.1-7.35.6.
- Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: a systematic review. JAMA 2006; 295:1050.
- Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46:1019.
- Erkan D, Lockshin MD. New treatments for antiphospholipid syndrome. Rheum Dis Clin North Am 2006; 32:129.
- Lau DH, Wun T. Early manifestation of thrombotic thrombocytopenic purpura. Am J Med 1993; 95:544.
- Bouw MC, Dors N, van Ommen H, Ramakers-van Woerden NL. Thrombotic thrombocytopenic purpura in childhood. Pediatr Blood Cancer 2009; 53:537.
- Nazarian RM, Van Cott EM, Zembowicz A, Duncan LM. Warfarin-induced skin necrosis. J Am Acad Dermatol 2009; 61:325.
- Bauer KA. Coumarin-induced skin necrosis. Arch Dermatol 1993; 129:766.
- Bovill EG, Bauer KA, Dickerman JD, et al. The clinical spectrum of heterozygous protein C deficiency in a large New England kindred. Blood 1989; 73:712.
- Arepally G, Cines DB. Heparin-induced thrombocytopenia and thrombosis. Clin Rev Allergy Immunol 1998; 16:237.
- Warkentin TE. Heparin-induced thrombocytopenia: a ten-year retrospective. Annu Rev Med 1999; 50:129.
- Gorevic PD, Kassab HJ, Levo Y, et al. Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am J Med 1980; 69:287.
- Della Rossa, A, Tavoni, et al. Cryoglobulinemia. In: Rheumatology, 3rd ed, Hochberg, M (Eds), Mosby, Philidelphia, PA 2003. p.1697-1703.
- Saadoun D, Elalamy I, Ghillani-Dalbin P, et al. Cryofibrinogenemia: new insights into clinical and pathogenic features. Am J Med 2009; 122:1128.
- Bloch KJ, Maki DG. Hyperviscosity syndromes associated with immunoglobulin abnormalities. Semin Hematol 1973; 10:113.
- Lane JE, Lane TN, Shams M, Lane CE. Cutaneous cholesterol embolization. J Am Acad Dermatol 2009; 60:711.
- Kusaba A, Imayama S, Furue M. Delayed appearance of livedo reticularis in 3 cases with a cholesterol embolism. Arch Dermatol 1999; 135:725.
- Aggarwal SK, Barik R, Sarma TC, et al. Clinical presentation and investigation findings in cardiac myxomas: new insights from the developing world. Am Heart J 2007; 154:1102.
- Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001; 80:159.
- Callen, JP. Cutaneous vasculitis: relationship to systemic disease and therapy. Curr Probl Dermatol 1993; :45.
- Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol 2006; 24:414.
- Carlson JA, Ng BT, Chen KR. Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis. Am J Dermatopathol 2005; 27:504.
- Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial 2002; 15:172.
- Kalajian AH, Malhotra PS, Callen JP, Parker LP. Calciphylaxis with normal renal and parathyroid function: not as rare as previously believed. Arch Dermatol 2009; 145:451.
- Bobrowski AE, Langman CB. Hyperoxaluria and systemic oxalosis: current therapy and future directions. Expert Opin Pharmacother 2006; 7:1887.
- Cochat P. Primary hyperoxaluria type 1. Kidney Int 1999; 55:2533.
- Jorizzo JL. Livedoid vasculopathy: what is it? Arch Dermatol 1998; 134:491.
- Bard JW, Winkelmann RK. Livedo vasculitis. Segmental hyalinizing vasculitis of the dermis. Arch Dermatol 1967; 96:489.
- Anavekar NS, Kelly R. Heterozygous prothrombin gene mutation associated with livedoid vasculopathy. Australas J Dermatol 2007; 48:120.
- Hurwitz RM, Haseman JH. The evolution of pyoderma gangrenosum. A clinicopathologic correlation. Am J Dermatopathol 1993; 15:28.
- Jorizzo JL, Solomon AR, Zanolli MD, Leshin B. Neutrophilic vascular reactions. J Am Acad Dermatol 1988; 19:983.
- Su WP, Davis MD, Weenig RH, et al. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol 2004; 43:790.
- Piette W. Purpura: mechanisms and differential diagnosis. In: Dermatology, 2nd ed, Bolognia JL, Jorizzo JL, Rapini RP. (Eds), Elsevier Limited, Spain 2008. Vol 1, p.321.
- Wysong A, Venkatesan P. An approach to the patient with retiform purpura. Dermatol Ther 2011; 24:151.
- Associated disorders
- - Intravascular abnormalities
- Thrombotic and coagulopathic disorders
- Intravascular protein deposition
- Embolic disorders
- - Vessel wall pathology
- - Other
- PATIENT ASSESSMENT
- Life-threatening emergency (purpura fulminans)
- Patient history
- Physical examination
- - Lesion color and palpability
- - Lesion distribution
- - Differential diagnosis
- - Associated physical findings
- Skin biopsy
- - Type and location of biopsy
- - Assessment of biopsy results
- Laboratory tests
- SUMMARY AND RECOMMENDATIONS