<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Introduction. Indeterminate cell histiocytosis is a rare proliferative disorder of indeterminate dendritic cells, reported in only 50 case reports so far. It is clinically presented as yellow, red or brown papules and nodules that appear in otherwise healthy adult individuals. Indeterminate cells are distinct dendritic cells of the skin that have ultrastructural similarities to the epidermal Langerhans cells but do not contain the characteristic Birbeck’s granules and they are also langerin-negative, unlike LCH. Indeterminate cell histiocytosis is an exceptional entity with variable clinical, histopathologic or immunohistochemical findings, sharing morphologic and immunophenotypic features with both Langerhans- and non-Langerhans cell histiocytoses. Case Report. We present a case of indeterminate histiocytosis in a 77-year-old man with 3-year history of asymptomatic, multiple reddish and brown papules and nodules over the entire body, including the oral mucosa. Skin biopsy was done, and histopathological analysis with immunohistochemistry was performed. The positive ICH staining of cells for CD68, CD1a, and S-100 enabled us to diagnose ICH in our patient. Also, BRAF V600E mutation was detected in tumor tissue. The treatment was started with methotrexate that was effective for 6 months, but due to the disease recurrence, further therapy with thalidomide was advised, without effect. Conclusion. Indeterminate histiocytosis is a rare disease, therefore no standardized treatment has been established and the treatment options are limited.</p></abstract>

Introduction. Indeterminate cell histiocytosis is a rare proliferative disorder of indeterminate dendritic cells, reported in only 50 case reports so far. It is clinically presented as yellow, red or brown papules and nodules that appear in otherwise healthy adult individuals. Indeterminate cells are distinct dendritic cells of the skin that have ultrastructural similarities to the epidermal Langerhans cells but do not contain the characteristic Birbeck’s granules and they are also langerin-negative, unlike LCH. Indeterminate cell histiocytosis is an exceptional entity with variable clinical, histopathologic or immunohistochemical findings, sharing morphologic and immunophenotypic features with both Langerhans- and non-Langerhans cell histiocytoses. Case Report. We present a case of indeterminate histiocytosis in a 77-year-old man with 3-year history of asymptomatic, multiple reddish and brown papules and nodules over the entire body, including the oral mucosa. Skin biopsy was done, and histopathological analysis with immunohistochemistry was performed. The positive ICH staining of cells for CD68, CD1a, and S-100 enabled us to diagnose ICH in our patient. Also, BRAF V600E mutation was detected in tumor tissue. The treatment was started with methotrexate that was effective for 6 months, but due to the disease recurrence, further therapy with thalidomide was advised, without effect. Conclusion. Indeterminate histiocytosis is a rare disease, therefore no standardized treatment has been established and the treatment options are limited.

Indeterminate Cell Histiocytosis – Case Report and Review of Literature

Serbian Journal of Dermatology and Venereology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

{"article-title":"Indeterminate Cell Histiocytosis \u2013 Case Report and Review of Literature"}

Introduction. Indeterminate cell histiocytosis is a rare proliferative disorder of indeterminate dendritic cells, reported in only 50 case reports so far. It...

Indeterminate Cell Histiocytosis – Case Report and Review of Literature

Published Online: Dec 05, 2018

Page range: 18 - 24

DOI: https://doi.org/10.2478/sjdv-2018-0004

Keywords
Histiocytosis, Dendritic Cells, Langerhans Cells, Skin Neoplasms, Rare Diseases, Treatment Outcome

© 2018 Aleksandra R. Vojvodić, et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Indeterminate Cell Histiocytosis – Case Report and Review of Literature

Published Online: Dec 05, 2018

Page range: 18 - 24

DOI: https://doi.org/10.2478/sjdv-2018-0004

KeywordsHistiocytosis, Dendritic Cells, Langerhans Cells, Skin Neoplasms, Rare Diseases, Treatment Outcome

© 2018 Aleksandra R. Vojvodić, et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
Histiocytosis, Dendritic Cells, Langerhans Cells, Skin Neoplasms, Rare Diseases, Treatment Outcome