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- Presentation
S049 - Clinicopathologic Self-assessment
Description
In this clinicopathologic self-assessment presentation, the speaker discusses two clinical cases involving skin lesions. The first case features a 57-year-old woman with an enlarging puritic pink lesion on her forehead, which is diagnosed as primary cutaneous CD4 positive small medium T cell lymphoproliferative disorder. This diagnosis was reached through histopathological examination and immunostaining, revealing a predominance of CD4+ T cells. The speaker explains this is a provisional entity linked to T-cell lymphomas, emphasizing its complex classification, indolent nature, and high remission rate, typically treated with clinical observation. The second case concerns an 83-year-old man with firm skin-colored papules on his hands, identified through histopathological analysis as related to a condition referred to as toxic hyaline, stemming from erythma elevatum diutinum (EED). The speaker outlines the intricacies of diagnosing this condition, the challenges associated with treatment, and emphasizes the rarity of encountering such cases. Both cases underscore the importance of accurate pathology in diagnosing skin lesions.
View moreConclusions
- The diagnosis for the first case is primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder, which is acknowledged as a provisional entity by the WHO.
- This disorder comprises approximately 2-3% of all cutaneous lymphomas and lacks a clear consensus on its definition and classification.
- Diagnosis of this disorder should ideally exclude other lesions and require specific clinical and histopathological features.
- The preferred treatment approach for this condition is clinical observation, with many patients experiencing resolution after biopsy.
- Five-year survival rates for this indolent disease are reported to be between 80-100%, indicating a generally favorable prognosis.
- In the second case of firm skin-colored papules, the potential diagnoses include primary cutaneous amyloidosis, hyaline globules, and lipoid proteinosis, with the specific diagnosis still uncertain based on histopathological findings.
- Hyaline globules are associated with several conditions, and the molecular basis of lipoid proteinosis involves mutations in extracellular matrix protein 1.
- Cerroni L. Skin Lymphoma: The Illustrated Guide. Fourth ed: Wiley-Blackwell; 2014.
- Choi M, Park SY, Park HS, Byun HJ, Cho KH. A Case of Primary Cutaneous CD4 Positive Small/medium T Cell Lymphoma. Ann Dermatol. 2011 Feb;23(1):76-80. doi: 10.5021/ad.2011.23.1.76. Epub 2011 Feb 28. PMID: 21738369; PMCID: PMC3120005.
- Besch-Stokes JG, Costello CM, Severson KJ, Bhullar P, Montoya J, Butterfield RJ, DiCaudo DJ, Comfere N, Sluzevich J, Rule W, Craig FE, Rosenthal A, Pittelkow MR, Mangold AR. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder: Diagnosis and management. J Am Acad Dermatol. 2022 May;86(5):1167-1169. doi: 10.1016/j.jaad.2021.04.067. Epub 2021 Apr 26. PMID: 33915243.
- Human Pathology Volume 25, Issue 12, December 1994, Pages 1283-1289
- Experimental Dermatology, Volume: 16, Issue: 11, Pages: 881-890, First published: 09 October 2007, DOI: 10.1111/j.1600-0625.2007.00608.x
- Am J Dermatopathol. 2024 Mar 1;46(3):181-182. doi:10.1097/DAD.0000000000002617