Facial cutaneous Rosai-Dorfman disease: a case report
Abstract Background Rosai-Dorfman disease (RDD) is a form of non-Langerhans cell histiocytosis in which the activated histiocytes of the lymph nodes and other organs begin to accumulate following excessive production. Bilateral, massive, and painless lymphadenopathy are classic presentations. System...
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| Format: | Article |
| Language: | English |
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BMC
2024-03-01
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| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-024-04410-9 |
| _version_ | 1797233553088446464 |
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| author | Tanapong Wongrat Siripan Sangmala |
| author_facet | Tanapong Wongrat Siripan Sangmala |
| author_sort | Tanapong Wongrat |
| collection | DOAJ |
| description | Abstract Background Rosai-Dorfman disease (RDD) is a form of non-Langerhans cell histiocytosis in which the activated histiocytes of the lymph nodes and other organs begin to accumulate following excessive production. Bilateral, massive, and painless lymphadenopathy are classic presentations. Systemic RDD is already known to be a rare condition, but isolated cutaneous RDD is extremely rare. We presented a rare and unusual presentations of a disease. Case presentation A 35-year-old Thai female with a 6-month history of a small acne-like lesion that rapidly progressed to 5 cm tumor-like lesions on the face within 3 months. Tissue histology showed a dense dermal infiltration of histiocytes with emperipolesis phenomenon. Immunohistochemistry was positive for S100 protein and CD68 and negative for CD1a. Oral prednisolone (50 mg/day) was initiated with a favorable outcome at the one-month follow-up. However, prednisolone yielded a partial response at 2-month follow-up, leading to application of another modality. Conclusion Although cutaneous Rosai-Dorfman disease is considered benign and well medical responded disease, patients with atypical presentation and rapid growing lesion may necessitate aggressive multimodal treatment. |
| first_indexed | 2024-04-24T16:18:00Z |
| format | Article |
| id | doaj.art-c1d01bd7e6a04287a25bd2e2befce270 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-04-24T16:18:00Z |
| publishDate | 2024-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-c1d01bd7e6a04287a25bd2e2befce2702024-03-31T11:21:56ZengBMCJournal of Medical Case Reports1752-19472024-03-011811510.1186/s13256-024-04410-9Facial cutaneous Rosai-Dorfman disease: a case reportTanapong Wongrat0Siripan Sangmala1Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityDivision of Dermatology, Department of Internal Medicine, Faculty of Medicine, Prince of Songkla UniversityAbstract Background Rosai-Dorfman disease (RDD) is a form of non-Langerhans cell histiocytosis in which the activated histiocytes of the lymph nodes and other organs begin to accumulate following excessive production. Bilateral, massive, and painless lymphadenopathy are classic presentations. Systemic RDD is already known to be a rare condition, but isolated cutaneous RDD is extremely rare. We presented a rare and unusual presentations of a disease. Case presentation A 35-year-old Thai female with a 6-month history of a small acne-like lesion that rapidly progressed to 5 cm tumor-like lesions on the face within 3 months. Tissue histology showed a dense dermal infiltration of histiocytes with emperipolesis phenomenon. Immunohistochemistry was positive for S100 protein and CD68 and negative for CD1a. Oral prednisolone (50 mg/day) was initiated with a favorable outcome at the one-month follow-up. However, prednisolone yielded a partial response at 2-month follow-up, leading to application of another modality. Conclusion Although cutaneous Rosai-Dorfman disease is considered benign and well medical responded disease, patients with atypical presentation and rapid growing lesion may necessitate aggressive multimodal treatment.https://doi.org/10.1186/s13256-024-04410-9Cutaneous Rosai-DorfmanSkinHematologyHistiocytosisCase report |
| spellingShingle | Tanapong Wongrat Siripan Sangmala Facial cutaneous Rosai-Dorfman disease: a case report Journal of Medical Case Reports Cutaneous Rosai-Dorfman Skin Hematology Histiocytosis Case report |
| title | Facial cutaneous Rosai-Dorfman disease: a case report |
| title_full | Facial cutaneous Rosai-Dorfman disease: a case report |
| title_fullStr | Facial cutaneous Rosai-Dorfman disease: a case report |
| title_full_unstemmed | Facial cutaneous Rosai-Dorfman disease: a case report |
| title_short | Facial cutaneous Rosai-Dorfman disease: a case report |
| title_sort | facial cutaneous rosai dorfman disease a case report |
| topic | Cutaneous Rosai-Dorfman Skin Hematology Histiocytosis Case report |
| url | https://doi.org/10.1186/s13256-024-04410-9 |
| work_keys_str_mv | AT tanapongwongrat facialcutaneousrosaidorfmandiseaseacasereport AT siripansangmala facialcutaneousrosaidorfmandiseaseacasereport |