Polyfibromatosis is a rare fibrosing condition characterized by fibromatosis in different body areas and by keloid formation, and which can be associated with arthropathy and osteolysis. Familial occurrence has been described, but the cause remains unknown. Here, we describe a patient with characteristics of polyfibromatosis with arthropathy who had in addition severe conjunctival fibrosis, distinctive face, gingival overgrowth, and pigmented keloids. We discuss the resemblances and differences with polyfibromatosis and descriptions of other, similar patients. We conclude that at present it remains uncertain whether the patient has a variant of polyfibromatosis or a separate entity.

Arthropathy, osteolysis, keloids, relapsing conjunctival pannus and gingival overgrowth: A variant of polyfibromatosis?

RONGIOLETTI, FRANCO;
2013-01-01

Abstract

Polyfibromatosis is a rare fibrosing condition characterized by fibromatosis in different body areas and by keloid formation, and which can be associated with arthropathy and osteolysis. Familial occurrence has been described, but the cause remains unknown. Here, we describe a patient with characteristics of polyfibromatosis with arthropathy who had in addition severe conjunctival fibrosis, distinctive face, gingival overgrowth, and pigmented keloids. We discuss the resemblances and differences with polyfibromatosis and descriptions of other, similar patients. We conclude that at present it remains uncertain whether the patient has a variant of polyfibromatosis or a separate entity.
Arthropathy; Conjunctival pannus; Distinctive face; Joint contractures; Keloid; Polyfibromatosis; Abnormalities, multiple; Arthrography; Cleft palate; Comparative genomic hybridization; Conjunctival diseases; Contracture; Cytogenetic analysis; Diagnosis, differential; Fibroma; Fibromatosis, gingival; Fibrosis; Foot joints; Gingival Ooergrowth; Hand joints; Humans; Hydrocephalus; Joint diseases; Keloid; Limb deformities, congenital; Male; Middle aged; Osteolysis; Genetics; Genetics (clinical)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11584/202106
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