Juvenile hyaline fibromatosis: a case report.
Juvenile hyaline fibromatosis (JHF) is a rare, autosomal recessively inherited disorder characterized histologically by deposition of hyaline, collagen like substance aberrantly synthesized by the cells of the connective tissue and deposited within many organs, typically within the skin, gingiva, joints and bones. We report this rare case of Juvenile hyaline fibromatosis in a young boy who presented clinically with multiple papulonodular skin lesions, non tender soft tissue masses over the scalp, face, anterior chest wall, back, periarticular regions of the extremities with restricted mobility of joints and gingival hypertrophy. Calcifications were seen within the tumor shadows in the skull X-Rays. Histopathological study revealed characteristic features consistent with Juvenile hyaline fibromatosis. We report this case in view of its rarity.
Swaroop
Duddi Sreehari Krishna
DS
Department of Pathology, Narayana Medical College and Hospital, Nellore, Andhra Pradesh. drkrishu@yahoo.com
Vydehi
Beemaraju Venkata
BV
Bai
Sarala Jyothi
SJ
D002648:Child; D005350:Fibroma / Q000175:diagnosis* / Q000473:pathology*; D005886:Gingival Hypertrophy / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D006818:Hyalin / Q000378:metabolism*; D008297:Male; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000175:diagnosis / Q000473:pathology