These cells may show high rates of replicating as judged by their mitotic index but these mitoses are normal in appearance. The tumor tissues often contain red blood cells, lymphocytes and giant osteoclast-like giant cells and may contain sites of bone-like tissue. NF is sometimes classified into three subtypes based on its predominant histopathological pattern: myxoid or reactive (type I), cellular (type II), and fibrous (type III). These patterns appear related to the duration of the lesion with the myxoid variant tending to have the shortest duration and the cellular and fibrous variants tending to have progressively longer durations.Immunohistochemical analyses indicate that the cells in NF usually express smooth muscle actin, muscle specific actin, and vimentin proteins but generally do not express CD34, S-100 protein, desmin, trypsin, factor VIII, F4/80 (also termed macrophage-specific antigen), or HLA-DR1 proteins. Uncommonly, the cells in NF tumors also express the CD68 (a histiocyte-specific marker) protein. The histopathology and expressions of marker proteins in cranial fasciitis tumors are similar to NF although its tumors tend to be more organized and have higher levels of inflammatory cell infiltrates, vascularity, and involvements of underlying bone than NF.The histopathology and expressions of marker proteins in intravascular fasciitis tumors are also similar to NF. In comparison to NF, however, its spindle-shaped tumor cells are more often arranged in a storiformed (i.e. whirled) or haphazard pattern and have vesicle-containing nuclei with prominent nucleoli.
Several methods can be used for genetic testing that can help identity this condition,
Molecular genetic tests (or gene tests) study single genes or short lengths of DNA to identify mutations and variations that can help lead to a genetic disorder. Chromosomal genetic tests help investigate whole chromosomes or long lengths of DNA to see if there are bigger genetic changes, for example, an extra copy of a chromosome that can help cause a genetic condition. Biochemical genetic tests study the activity level and amount level of proteins; abnormalities in either can indicate changes to the DNA and result in a genetic disorder. Management
Although there has been no conclusive evidence on medications to manage the disorder, many organizations help with several aspects on living with the chronic condition, not only for the affected but also for the loved ones around them. With services like Global Genes helping contribute to this. References
Further reading
Itin P., Pittelkow M., Trichothiodystrophy: Review of sulfur-deficient brittle hair syndromes and association with the ectodermal dysplasias[1]. Journal of the American Academy of Dermatology, Volume 22, Issue 5, Pages 705–717
Rare Disorders: All you need to know. Trichothiodystrophy[2]. Copyright © 2005 Rare-Disorders.com
Sabinas brittle hair syndrome is a form of nonphotosensitive trichothiodystrophy; Brittle hair and mental deficit at NIHs Office of Rare Diseases


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