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Superficial and Deep Cutaneous Involvement by RAS-Associated Autoimmunne Leukoproliferative Disease (RALD Cutis)

A Histologic Mimicker of Histiocytoid Sweet Syndrome

Tran, Tien Anh N. MD*; Grow, William B. MD; Chang, Chung-Che MD*

The American Journal of Dermatopathology: August 2019 - Volume 41 - Issue 8 - p 606–610
doi: 10.1097/DAD.0000000000001332
Extraordinary Case Report
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Abstract: RAS-associated autoimmune leukoproliferative disease (RALD) is a recently described noninfectious and nonmalignant clinical syndrome characterized by autoimmune disorders, massive splenomegaly, modest lymphadenopathy, and monocytosis. On the molecular level, RALD is defined by somatic mutations of either NRAS or KRAS gene in a subset of hematopoietic cells. To date, there is a dearth of well-documented histopathologic description of cutaneous involvement by RALD in the literature. In the current case report, a 43-year-old female patient with a history of RALD presented with clinical pictures of sepsis and an erythematous rash in the left lower extremity. Histologic examination revealed a dense perivascular and interstitial infiltrate of immature myeloid cells admixed with scattered neutrophils involving the dermis and subcutaneous adipose tissue, imparting a panniculitis-like histologic pictures. There was a strong angiocentric propensity of the immature hematopoietic cells as well as extensive extravasation of red blood cells, even in the subcutaneous adipose tissue. Immunohistochemically, the immature hematopoietic cells were positive for CD43, CD4, and CD68, but negative for CD34, CD117, and myeloperoxidase. Overall, the histologic and cytologic findings were highly reminiscent of histiocytoid Sweet syndrome. Review of the English literature revealed cutaneous involvements by RALD only in patients with KRAS mutation compared with none of its NRAS counterparts. However, larger clinicopathologic studies on cutaneous involvement by RALD are warranted. The term “RALD cutis” with its histologic and molecular features is suggested to serve as a potential groundwork for future studies of this rare phenomenon.

*Department of Pathology, Florida Hospital Orlando, Orlando, FL; and

Florida Hospital Cancer Center, Florida Hospital Orlando, Orlando, FL.

Correspondence: Tien Anh N. Tran, MD, Department of Pathology, Florida Hospital Orlando, 601 East Rollins Street, Orlando, FL 32803 (e-mail: tien.tran.md@flhosp.org).

The authors declare no conflicts of interest.

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