title: The cutaneous manifestations in children with familial Mediterranean fever (recurrent hereditary polyserositis). A six-year study.
abstract: In a six-year study of 46 children with familial Mediterranean fever (recurrent hereditary polyserositis), 20 children (43 per cent) developed cutaneous manifestations. Ten children had 18 episodes of erysipelas-like erythema which proved to be specific for the disease. Twelve children (26 per cent) had 31 episodes of non-specific purpuric rash and six children (13 per cent) had nine episodes of Henoch-Schönlein purpura. Other manifestations included diffuse erythema of the face, and/or trunk, angioneurotic oedema, diffuse erythema of the palms and soles followed by mild desquamation of the skin, Raynaud's phenomenon and a subcutaneous nodule. The mean frequency of the cutaneous lesions was 1.6/patient/year before colchicine therapy and 0.4/patient/year after colchicine therapy (p = 0.0005). The high incidence of cutaneous manifestations and their response to colchicine strongly suggest that skin involvement is an integral part familial Mediterranean fever.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, University of Kuwait.
authors.forename: H A
authors.initials: HA
authors.lastname: Majeed
authors.forename: Z
authors.initials: Z
authors.lastname: Quabazard
authors.forename: Z
authors.initials: Z
authors.lastname: Hijazi
authors.forename: S
authors.initials: S
authors.lastname: Farwana
authors.forename: F
authors.initials: F
authors.lastname: Harshani
mesh_terms: D002648:Child; D002675:Child, Preschool; D004487:Edema / Q000473:pathology; D004890:Erythema / Q000473:pathology; D010505:Familial Mediterranean Fever / Q000473:pathology*; D005260:Female; D005528:Foot; D006225:Hand; D006801:Humans; D007223:Infant; D007866:Leg; D008297:Male; D011693:Purpura / Q000473:pathology; D011928:Raynaud Disease / Q000473:pathology; D012867:Skin / Q000473:pathology*