Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”.
Infantile systemic hyalinosis and juvenile hyaline fibromatosis are very rare recessive autosomal hereditary diseases that arise from mutation of gene ANTXR2 (anthrax toxin receptor-2), also known as gene CMG2 (capillary morphogenesis gene2), located in chromosome 4q21.
Follicular mucinosis is a rare inflammatory disorder of the skin characterized histologically by accumulation of mucin in the follicular epithelium.
Rosai- Dorfman Disease is a rare benign histiocytic disorder in which patients present with painless cervical lymphadenopathy, fever and leukocytosis. The classical findings include emperipolesis and histiocytes staining positive for S100 protein and CD68 and negative for CD1a.
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Aggressive angiomyxoma is a locally aggressive mesenchymal tumour.
This tumour usually occurs in women of reproductive age and is located in the perineal or pelvic region.
Histologically, this enigmatic soft tissue tumour is characterized by a prominent myxoid matrix and numerous thin-walled blood vessels.
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