Indeterminate Cell Histiocytosis – Case Report and Review of Literature
| Autor: | Željko Mijušković, Aleksandra Vojvodic, Lidija Kandolf Sekulović |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
medicine.medical_specialty
integumentary system business.industry skin neoplasms rare diseases Dermatology medicine.disease 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine RL1-803 030220 oncology & carcinogenesis langerhans cells treatment outcome Medicine dendritic cells Indeterminate Cell Histiocytosis histiocytosis business |
| Zdroj: | Serbian Journal of Dermatology and Venereology, Vol 10, Iss 1, Pp 18-24 (2018) |
| ISSN: | 2406-0631 1821-0902 |
| DOI: | 10.2478/sjdv-2018-0004 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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