In:
Lupus, SAGE Publications, Vol. 27, No. 13 ( 2018-11), p. 2170-2173
Abstract:
We report the case of a 25-year-old female patient who presented with purpura fulminans as a manifestation of primary antiphospholipid syndrome. Purpura fulminans is considered a rare cutaneous manifestation of antiphospholipid syndrome. Most frequently, it occurs in the context of catastrophic antiphospholipid syndrome and is associated with significant morbidity and mortality, either due to loss of affected extremities or thromboembolic damage to internal organs. After insufficient efficacy of parenteral anticoagulation and oral glucocorticosteroid treatment, we escalated treatment to high-dose intravenous glucocorticosteroid and five consecutive sessions of plasma exchange with good and sustained clinical response. At follow-up six months after admission, skin manifestations had healed with scarring, and no additional thrombotic events had occurred. Plasma exchange may hold promise as a therapeutic option in refractory or severe cases of antiphospholipid syndrome-related purpura fulminans with extensive cutaneous necrosis, although evidence is limited.
Type of Medium:
Online Resource
ISSN:
0961-2033
,
1477-0962
DOI:
10.1177/0961203318804884
Language:
English
Publisher:
SAGE Publications
Publication Date:
2018
detail.hit.zdb_id:
2008035-9
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