Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vitamin K deficiency bleeding (VKDB) disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages.([1]) Though skin and mucosal bleeding may occur in 1/3(rd) of infants with VKDB, 'nodular purpura' is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.
Indian J Dermatol 2013 Sep
PMID:Vitamin k deficiency bleeding presenting as nodular purpura in infancy: a rare and life-threatening entity. 2408 4

Enoxaparin sodium is a heparin of low molecular weight with antithrombotic properties that acts by inhibiting factor Xa. We present a 59-year-old woman who developed heparin-related bullous hemorrhagic dermatosis, at sites distant from the injections, one month after starting treatment with enoxaparin.
Dermatol Online J 2014 Oct 15
PMID:Bullous hemorrhagic dermatosis distant from the site of heparin injection. 2552 9

Anticoagulants are the cornerstone of treatment of venous thromboembolism associated with various medical conditions and surgical procedures. They act on different steps of the coagulation pathway and are broadly categorized into heparins, vitamin K antagonists, and inhibitors of thrombin and factor Xa. The classification is evolving as newer and better oral and parenteral anticoagulants are being added. Anticoagulants in dermatology are important not only for their therapeutic application in cutaneous thrombotic dermatoses such as livedoid vasculitis, purpura fulminans, superficial and deep venous thrombosis and others but also for their use in non-thrombotic dermatoses such as lichen planus, recurrent oral aphthosis, chronic urticaria and several others. Further, the use of anticoagulants for any indication is associated with various adverse effects with dermatologic manifestations including specific reactions such as warfarin-induced skin necrosis, heparin-induced thrombocytopenia and anticoagulant-associated cholesterol embolization syndrome.
Indian J Dermatol Venereol Leprol
PMID:Anticoagulants in dermatology. 2732 Jul 65

Acquired hemophilia A (AHA) is a rare autoimmune disorder with high morbidity and mortality. It results from the development of circulating autoantibodies against factor VIII. AHA can be seen in association with autoimmune vesiculobullous diseases, autoimmune diseases, malignancy, pregnancy, and medications. We report a 68-year-old Thai woman diagnosed and treated for bullous pemphigoid (BP) for 11 months who recently presented with a 3-day history of extensive hemorrhagic bullae and large intra-oral buccal hematoma. Laboratory investigations confirmed a prolonged activated partial thromboplastin time, a low factor VIII level, a high factor VIII inhibitor level, and elevated anti-BPAG180 and anti-BPAG230 titers, confirming the diagnosis of BP associated with AHA. Immunosuppressive therapy with systemic corticosteroids and cyclophosphamide combined with bypassing agents for bleeding control resulted in significant clinical improvement and subsequent negative antibody levels. There was no recurrence after a 7-month follow-up period. Due to life-threatening bleeding in severe AHA cases, early diagnosis and effective treatment in this condition are essential.
Case Rep Dermatol
PMID:Bullous Pemphigoid Associated with Acquired Hemophilia A: A Case Report and Review of the Literature. 3118 47

Tufted angioma is a rare, benign vascular tumor of uncertain pathogenesis, characterized histopathologically by "tufts" of capillaries within the dermis. A life-threatening coagulopathy, Kasabach-Merritt phenomenon, occurs in approximately 10% of cases of tufted angioma and is characterized by profound thrombocytopenia and fibrinogen consumption. We present an asymptomatic 10-month-old boy who presented with an erythematous patch of the right upper extremity and subsequently was diagnosed by biopsy with tufted angioma. Baseline laboratory workup of our patient revealed mildly decreased fibrinogen and elevated D-dimer levels without thrombocytopenia or elevated partial thromboplastin time. This suggests that asymptomatic patients with tufted angioma may present with coagulopathy in the absence of Kasabach-Merritt phenomenon. This also highlights the importance of obtaining baseline laboratory workup in patients presenting with tufted angioma.
Dermatol Online J 2019 Sep 15
PMID:Tufted angioma presenting with subclinical coagulopathy. 3173 44

Novel oral anticoagulant (NOAC) medications have revolutionized hematology and cardiology. Recently, NOACs have demonstrated additional promise in dermatology. Specifically, rivaroxaban, a direct factor Xa inhibitor NOAC, has been shown to be successful in the treatment of livedoid vasculopathy. Herein, we describe a patient with systemic lupus erythematosus who presented with painful cutaneous vasculopathy, demonstrated on biopsy with occlusive microvascular fibrin thrombi without evidence of concurrent vasculitis. Interestingly, imaging and laboratory studies did not show evidence of hypercoagulability, arterial disease, or embolic disease. The patient’s vasculopathy and pain progressed despite antiplatelet therapy, often considered first-line in cases of microvascular occlusive disease. However, with rivaroxaban therapy, the patient experienced complete regression of her painful lesions, thereby supporting a further role for NOACs in cutaneous vasculopathy treatment. J Drugs Dermatol. 2020;19(5) doi:10.36849/JDD.2020.4684.
J Drugs Dermatol 2020 May 01
PMID:Successful Treatment of Painful Cutaneous Vasculopathy With Rivaroxaban in a Patient With Systemic Lupus Erythematosus. 3248 18


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