title: Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy.
abstract: Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis that affects children under 2 years of age and is clinically characterized by fever, extensive tender edema and large purpuric lesions that involve mainly the face, ears and limbs. AHEI typically exhibits an acute onset, with a short benign course followed by spontaneous and complete recovery. Visceral involvement is usually absent, and laboratory studies reveal no specific abnormalities. The pathogenic mechanisms underlying the development of AHEI remain unknown. We describe a patient with AHEI exhibiting transient renal involvement and hypocomplementemia. A 19-month-old Japanese boy was admitted to our hospital with a 2-day history of fever and purpura affecting his face, right ear and legs, and a 1-day history of painful edema of the right side of his face and his right lower leg. Laboratory studies revealed microscopic hematuria, proteinuria and hypocomplementemia affecting C4, C1q and CH50. A clinical diagnosis of AHEI was made, and the patient made a rapid and completely recovery without any specific therapy. We suggest that activation of the classical pathway of complement might be one of the pathogenic mechanisms underlying the development of AHEI.
authors: Department of Pediatrics, Niigata City General Hospital, 2-6-1 Shichikuyama Chuoku, Niigata, 950-8739, Japan. twata@hosp.niigata.niigata.jp
authors: Toru
authors: T
authors: Watanabe
authors: Yosuke
authors: Y
authors: Sato
mesh_terms: D003171:Complement Pathway, Classical; D003165:Complement System Proteins / Q000172:deficiency*; D004487:Edema / Q000150:complications / Q000473:pathology; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000473:pathology; D007223:Infant; D007674:Kidney Diseases / Q000209:etiology*; D008297:Male; D012867:Skin / Q000473:pathology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications* / Q000473:pathology
chemical_list: D003165:Complement System Proteins
references: J Allergy Clin Immunol. 1990 Oct;86(4 Pt 1):473-83
references: 2229809
references: J Am Acad Dermatol. 2002 Nov;47(5):801; author reply 801-2
references: 12399785
references: Clin Dermatol. 2002 Jan-Feb;20(1):2-3
references: 11849888
references: Eur J Dermatol. 2001 Jan-Feb;11(1):63-4
references: 11174143
references: J Am Acad Dermatol. 1999 Nov;41(5 Pt 2):837-9
references: 10534663
references: J Dermatol. 2002 May;29(5):290-5
references: 12081160
references: J Am Acad Dermatol. 1991 Jan;24(1):17-22
references: 1999524
references: Arch Fam Med. 2000 Jun;9(6):553-6
references: 10862218
references: J Am Acad Dermatol. 1990 Aug;23(2 Pt 2):347-50
references: 2394853
references: Arch Dis Child. 2005 Mar;90(3):316
references: 15723928
references: Pediatrics. 1993 Dec;92(6):865-7
references: 8233754
references: Pediatr Dermatol. 1995 Sep;12(3):224-7
references: 7501551
references: Pediatr Emerg Care. 1996 Apr;12(2):111-2
references: 8859921