Juvenile Hyaline Fibromatosis



Juvenile Hyaline Fibromatosis


Elizabeth A. Montgomery, MD










Low magnification shows juvenile hyaline fibromatosis of the face in the deep dermis image. There is a tumor-free (grenz) zone image between the epidermis and the lesional tissue.






Higher magnification shows scattered lesional cells in homogeneous-appearing dense eosinophilic stroma. The cells are small and uniform, arranged in short curved cords image. Some nuclei appear in spaces image.


TERMINOLOGY


Abbreviations



  • Juvenile hyaline fibromatosis (JHF)


Synonyms



  • Related disorder: Infantile systemic hyalinosis (ISH)



    • Suggested synonym: Hyaline fibromatosis syndrome


Definitions



  • Rare autosomal recessive disorder (often found in consanguineous populations) characterized by multiple skin papules and early onset

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Juvenile Hyaline Fibromatosis
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