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.
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- 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