Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Kasabach-Merritt syndrome includes the triad of vascular tumors, thrombocytopenia, and a hemorrhagic diathesis. The vascular tumors are usually benign but the associated coagulopathy may be life threatening. We describe a patient whose clinical course illustrates the potential difficulties in management.
Pediatr Dermatol 1991 Jun
PMID:Kasabach-Merritt syndrome. 192 82

Ischemic venous thrombosis that led to necrosis of four toes developed in an 81-year-old man. Despite the extensive thrombosis, results of blood clotting tests showed an extremely low prothrombin time (20%). Plasma mixing studies demonstrated an inhibitor that may have features in common with the lupus anticoagulant. Lupus anticoagulant also simulates hemorrhagic diathesis in vitro, whereas in vivo it is associated with thrombosis and thromboembolism. In contrast to the lupus anticoagulant, the inhibitor found in this patient was active within the extrinsic clotting system. Systemic steroids led to rapid clinical resolution paralleled by normalization of the prothrombin time and disappearance of the inhibitor.
J Am Acad Dermatol 1993 May
PMID:Ischemic venous thrombosis caused by a distinct disturbance of the extrinsic clotting system. 849 74

Cytophagic histiocytic panniculitis (CHP) is a disorder characterized by a histiocytic infiltrate in the subcutaneous adipose tissue which often extends to involve systemic organs. Fever, pancytopenia, hepatosplenomegaly, mucosal ulcers and serositis are common systemic symptoms. Although remissions have been reported, the disease tends to follow a chronic course often complicated by terminal hemorrhagic diathesis and death. Several reports in the recent literature have suggested that cyclosporine is the treatment of choice in CHP. We report the first case of cyclosporine treatment failure in CPH. Despite an initial response to treatment and persistent resolution of cutaneous lesions, the internal disease progressed resulting in the patient's death. Therefore the evolution of skin lesions and signs of systemic involvement may not be an accurate assessment of disease activity in patients with CHP being treated with cyclosporine.
J Eur Acad Dermatol Venereol 1998 May
PMID:Fatal cytophagic histiocytic panniculitis after a short response to cyclosporine. 964 34

We report the result of a randomized, controlled, open trial of anti-thrombin therapy for herpes zoster-associated pain. Fifty-five herpes zoster patients within 8 days after the onset of skin lesion were enrolled in the trial. Patients were treated with an optimal dose of oral acyclovir (4000 mg/day for 7 days) with or without intravenous administration of a specific anti-thrombin agent, argatroban (10 mg/day, three times a week). Administration of argatroban reduced pain intensity at the 4th through 21st day after the initiation of treatment as determined by visual analogue scale (Mann-Whitney U test, p < 0.05). It also shortened the median time to cessation of analgesic use (14 days vs. 24 days, p = 0.02, logrank test), although it did not significantly reduce the median time to cessation of pain (21 days vs. 43 days, p = 0.07, logrank test). None of the enrolled patients showed evidence of adverse effects including hemorrhagic diathesis. The results suggested that relatively low doses of argatroban are effective in reducing herpes zoster-associated pain. Up-regulation of prothrombin expression by the vascular endothelial and sweat gland epithelial cells in the active skin lesion and transient elevation of plasma thrombin-antithrombin III complex levels in a proportion of patients suggest a lesional generation of thrombin in herpes zoster. This may be relevant to the beneficial effects of the anti-thrombin treatment on the resolution of herpes zoster-associated pain.
J Dermatol 2001 Apr
PMID:A specific thrombin inhibitor, argatroban, alleviates herpes zoster-associated pain. 1144 71

Congenital afibrinogenaemia is a rare hemorrhagic disorder characterized by the absence of fibrinogen. We report a case of congenital afibrinogenaemia presented with leg ulcer. A 30-year-old man presented with a history of prolonged bleeding from birth. His parents are cousins. He repeatedly showed haematoma after traumas on his leg. He was diagnosed as having congenital afibrinogenaemia because of plasma fibrinogen deficiency. Because his leg ulcer gradually increased in size, he was admitted to our department for treatment. Laboratory examinations revealed prolonged bleeding time, prolonged coagulation time, prolonged prothrombin time, prolonged activated partial thromboplastin time and plasma fibrinogen was not measurable. Histological examination revealed hyperkeratosis, acanthosis and severe fibrotic change in the whole dermis. Severe hemosiderin deposit was found in the middle dermis. His leg ulcer cured 2 months after the beginning of fresh frozen plasma administration (FFP), but recurrence of the leg ulcer after FFP treatment was found. This is the second reported case of congenital afibrinogenaemia presented with leg ulcer.
Eur J Dermatol
PMID:Leg ulcer presenting in a patient with congenital afibrinogenaemia. 1180

Individual cases of so-called Weber-Christian disease with a bleeding diathesis have been reported for several years. These were originally diagnosed as Weber-Christian disease, but have been recategorized on review as a chronic, visceral, and cutaneous histiocytic (cytophagic) panniculitis, progressing to liver dysfunction and jaundice and a terminal hemorrhagic diathesis. We report here a rare catastrophic form of systemic panniculitis in an adolescent girl. Despite compelling clinical evidence, the diagnosis was made only on postmortem biopsies.
Pediatr Dermatol
PMID:Fatal cytophagic histiocytic panniculitis. 1516 5

Caterpillars and moths (order Lepidoptera) are uncommonly recognized causes of adverse cutaneous reactions, such as localized stings, papular dermatitis, and urticarial wheals. These reactions are typically mild and self-limited; however, in South America, the sting of Lonomia caterpillars can cause a potentially fatal hemorrhagic diathesis related to massive fibrinolysis. In addition, ocular inflammation and prominent arthralgias have been reported to be caused by caterpillar exposures. Therapies for mucocutaneous reactions to Lepidoptera are largely empiric, with the exception of antivenin against Lonomia obliqua envenomation. Part II of this two-part series on caterpillars and moths reviews the varied symptoms caused by Lepidopteran exposures, reviews the differential diagnosis, and discusses appropriate treatment algorithms.
J Am Acad Dermatol 2010 Jan
PMID:Caterpillars and moths: Part II. Dermatologic manifestations of encounters with Lepidoptera. 2008 87

Hermansky-Pudlak syndrome (HPS) is a rare autosomal-recessive disorder characterized by oculocutaneous albinism, a hemorrhagic diathesis due to platelet dysfunction, and lysosomal ceroid accumulation that can cause a Crohn's-like granulomatous colitis and pulmonary fibrosis. We report peristomal and vulvar cutaneous involvement of the granulomatous colitis in HPS.
Pediatr Dermatol
PMID:Metastatic cutaneous involvement of granulomatous colitis in Hermansky-Pudlak syndrome. 2247 86