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Purpose of Review <p>This article provides a comprehensive overview of Histiocytoid Sweet Syndrome (HSS), a rare histopathological variant of Sweet Syndrome (SS). It discusses clinical manifestations, diagnostic challenges, pathogenesis, associations with other diseases, and therapeutic options. Given the rarity of HSS, this review highlights the importance of recognizing and differentiating it from other conditions to ensure appropriate management.</p> Recent Findings <p>The first official description of HSS as a histopathological variant of SS was established in 2005, in a series of 41 patients who were initially thought to have leukemia cutis, both due to their clinical presentation and histopathology results. After further investigation, leukemia cutis (LC) was excluded and HSS was described as a condition that requires thorough investigation. Immunohistochemical and molecular studies revealed essential in differentiating between HSS and LC, with HSS typically characterized by positivity for MPO, CD68, CD15, CD43, CD45, and Lysozyme and negativity for CD34 and CD117, thus ruling out LC. VEXAS syndrome is also commonly associated with HSS, and in case of suspicion, early genetic testing for mutations like UBA1 is necessary. Corticosteroids remain the first-line treatment, but alternative immunosuppressive agents may be necessary in some cases. Further research into genetic markers and cytokine involvement is necessary to improve diagnosis and management.</p> Summary <p>HSS has been found to have association with hematologic malignancies particularly myelodysplastic syndromes (MDS), autoimmune diseases, infections, certain medications, and genetic mutations. The review highlights the need for focused investigations of ongoing advances in immunohistochemistry and treatment approaches to continue to enhance our understanding and management of HSS.</p>

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A Comprehensive Review of Sweet Syndrome focused on the Histiocytoid Variant

  • 期刊论文

  • 发表日期：

    2025年4月25日

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  • Christèle Asmar,
  • Minahil Iqbal,
  • Ali Toufaily,
  • Nancy Emmanuel

摘要

Purpose of Review

This article provides a comprehensive overview of Histiocytoid Sweet Syndrome (HSS), a rare histopathological variant of Sweet Syndrome (SS). It discusses clinical manifestations, diagnostic challenges, pathogenesis, associations with other diseases, and therapeutic options. Given the rarity of HSS, this review highlights the importance of recognizing and differentiating it from other conditions to ensure appropriate management.

Recent Findings

The first official description of HSS as a histopathological variant of SS was established in 2005, in a series of 41 patients who were initially thought to have leukemia cutis, both due to their clinical presentation and histopathology results. After further investigation, leukemia cutis (LC) was excluded and HSS was described as a condition that requires thorough investigation. Immunohistochemical and molecular studies revealed essential in differentiating between HSS and LC, with HSS typically characterized by positivity for MPO, CD68, CD15, CD43, CD45, and Lysozyme and negativity for CD34 and CD117, thus ruling out LC. VEXAS syndrome is also commonly associated with HSS, and in case of suspicion, early genetic testing for mutations like UBA1 is necessary. Corticosteroids remain the first-line treatment, but alternative immunosuppressive agents may be necessary in some cases. Further research into genetic markers and cytokine involvement is necessary to improve diagnosis and management.

Summary

HSS has been found to have association with hematologic malignancies particularly myelodysplastic syndromes (MDS), autoimmune diseases, infections, certain medications, and genetic mutations. The review highlights the need for focused investigations of ongoing advances in immunohistochemistry and treatment approaches to continue to enhance our understanding and management of HSS.