Leg ulcer presenting in a patient with congenital afibrinogenaemia.
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.
Kinebuchi
Akira
A
Department of Dermatology, Dokkyo University School of Medicine, 880 Kitakobayashi, Mibu, Tochigi 321-0293, Japan.
Ohtsuka
Tsutomu
T
Ishida
Shinnosuke
S
Otsuka
Shun
S
Abe
Takako
T
Yamakage
Akio
A
Yamazaki
Soji
S
Nakamura
Yuichiro
Y
Hirota
Ken
K
D000328:Adult; D000347:Afibrinogenemia / Q000097:blood / Q000150:complications / Q000151:congenital*; D001777:Blood Coagulation; D005340:Fibrinogen / Q000008:administration & dosage; D006801:Humans; D007871:Leg Ulcer / Q000209:etiology* / Q000628:therapy; D008297:Male
D005340:Fibrinogen