Noninclusion-body infantile digital fibromatosis: a lesion heralding terminal osseous dysplasia and pigmentary defects syndrome.
This report describes the histologic and immunohistochemical features of a peculiar type of digital fibroma that shares some clinical and microscopic features with the more common inclusion-body type infantile digital fibromatosis. However, this type does not exhibit inclusion bodies and its cells are reactive for vimentin but not for actin. Significantly, it presents in combination with a constellation of other clinical findings, i.e., mainly positional and bone abnormalities of the fingers and toes, and skin pigmentary defects. Thus, noninclusion-body digital fibromatosis may represent the first clue for the diagnosis of the so-called terminal osseous dysplasia and pigmentary defects syndrome.
Drut
Ricardo
R
Department of Pathology, Hospital de Niños Superiora Sor María Ludovica, La Plata, Argentina.
Pedemonte
Luis
L
Rositto
Alicia
A
D015415:Biomarkers / Q000378:metabolism; D001848:Bone Diseases, Developmental / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D005350:Fibroma / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D005385:Fingers / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D010859:Pigmentation Disorders / Q000378:metabolism / Q000473:pathology*; D011859:Radiography; D012878:Skin Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D013577:Syndrome
D015415:Biomarkers