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Postvaricella purpura fulminans

Oregon Health & Science University

View larger version (90K): [in this window] [in a new window]   Figure 1. Patient with Postvaricella purpura fulminans showing extent of necrotic lesions

View larger version (118K): [in this window] [in a new window]   Figure 2. Patient's leg after skin grafting

View larger version (87K): [in this window] [in a new window]   Figure 3. Close-up of skin graft site demonstrating size of necrotic area

	Clinical Summary

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

Patient was treated with heparin and plasma infusion which resulted in stabilization of his lesions. For his presumed autoimmune protein S deficiency he received immunoglobulin. Over the course of the next several months his protein S levels increased back into the normal range but his skin lesions required extensive grafting (Figures 2 and 3).

Sex
Male

Age
14

Ethnicity
Native American

	Diagnosis

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
Postvaricella purpura fulminans

	Discussion

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
Purpura fulminans which is DIC in association with symmetrical limb ecchymosis and necrosis of the skin, is seen in two situations. One, primary purpura fulminans, is most often seen after a viral infection. In these patients the purpura fulminans starts with a painful red area on an extremity that rapidly progresses to a black ischemia area. In many patients acquired deficiency of protein S is found. These patients will be found to have DIC upon laboratory evaluation.

Secondary purpura fulminans is most often associated with meningealococcemia infections but can be seen in any patient with overwhelming infections. Post-splenectomy sepsis syndrome patients are also at risk. Patients present with signs of sepsis, and the skin lesions often involve the digits leading to amputations.

The mechanism of post-varicella purpura fulminans is thought to be due to development on anti-protein S antibodies. These antibodies lead to an acquired severe protein S deficiency, which results in the DIC and microthrombosis.

Therapy for purpura fulminans is controversial. Primary purpura fulminans, especially those with post-varicella autoimmune protein S deficiency, have responded to plasma infusion titrated to keep the protein S level higher than 25%. Anecdotes exist about response to immunoglobulin (1mg/kg x 2 days) or steroids in these patients. Heparin has been reported to control the DIC and extent of necrosis A reasonable starting dose in these patients is 5-8 units/kg/h.

	Differential Diagnosis

TOP Clinical Summary Diagnosis Discussion Differential Diagnosis

 
Hemophilia

DIC

Amyloid

Post-infection purpura fulminans

Acquired factor inhibitors

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Related Image Bank Image Sets

Idiopathic Thrombocytopenic Purpura

John Lazarchick
ASH Image Bank 2001 2001: 100177. [Full Text]

Related ASH-SAP Chapter:

This Article Figures Only Services Similar articles in this journal Alert me to new content in the Image Bank Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by DeLoughery, T. Search for Related Content PubMed Articles by DeLoughery, T. Related Collections Hypercoagulable States - arterial and venous thrombosis, hereditary thrombophilias Related ASH-SAP Chapter Related Image Bank Image Sets Social Bookmarking                   What's this?