Emperipolesis: an additional common histopathologic finding in H syndrome and Rosai-Dorfman disease

The American Journal of Dermatopathology
Isabel ColmeneroL Requena

Abstract

H syndrome is a recently described autosomal recessive disorder characterized by indurated, hyperpigmented, and hypertrichotic cutaneous plaques, mainly involving the lower abdomen and lower extremities. Associated systemic manifestations include hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and hyperglycemia. H syndrome is caused by mutations in the gene SLC29A3, which encodes hENT3, a member of the human equilibrative nucleoside transporter family. Histopathologically, cutaneous lesions of H syndrome consist of dermal and subcutaneous fibrosis with inflammatory infiltrate mostly composed of large histiocytes, some plasma cells, and scattered lymphoid aggregates. Recently, histopathologic and immunohistochemical studies have demonstrated that the immunophenotype of the histiocytes infiltrating the skin of a patient with H syndrome is similar to that of the lesions of Rosai-Dorfman disease. Furthermore, mutations in SLC29A3 gene have also been demonstrated in patients described as having an inherited form of Rosai-Dorfman disease, named Faisalabad histiocytosis or familial Rosai-Dorfman disease. We describe emperipolesis in the cutaneous lesions of a patient with H syndrome, further supporting the...Continue Reading

References

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Citations

Feb 20, 2014·Ophthalmic Genetics·Vered Molho-PessachAbraham Zlotogorski
May 11, 2016·Ultrastructural Pathology·Jacques GilloteauxJack L Summers
Mar 4, 2017·International Journal of Dermatology·Jinjing JiaYan Zheng
Mar 14, 2018·Clinical Case Reports·David Dias-PolakEmily Avitan-Hersh
May 5, 2016·Revista chilena de pediatría·Hugo Hernán Abarca BarrigaNélida Aviles Alfaro
Jul 24, 2014·Clinical Reviews in Allergy & Immunology·Qi MiaoXiong Ma
Oct 5, 2016·Pediatric Dermatology·Georgios XirotagarosAntonio Torrelo
Jul 22, 2017·The Journal of Biological Chemistry·Md Fazlur RahmanRajgopal Govindarajan
Jun 2, 2018·Head and Neck Pathology·Vincent CracoliciDaniel T Ginat
Jul 5, 2019·Nature Communications·Sreenath NairRajgopal Govindarajan
Oct 20, 2020·Giornale Italiano Di Dermatologia E Venereologia : Organo Ufficiale, Società Italiana Di Dermatologia E Sifilografia·Irene Latour-Álvarez, Antonio Torrelo

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