Plasmacytoid Dendritic Cell Dermatosis Associated to Myeloproliferative/Myelodysplastic Neoplasms. (25th December 2022)

• Record Type: Journal Article
• Authors: Machan, Salma; Alonso-Dominguez, Juan M.; Sánchez García, F. Javier; Nieves Salgado, Rocío; Soto, Carlos; Castro, Yolanda; Pajares, Raquel; Manso, Rebeca; Santonja, Carlos; Serrano del Castillo, Cristina; Piris, Miguel A.; Requena, Luis; Rodríguez Pinilla, Socorro M.
• Is Part Of: American journal of surgical pathology. Volume 46:Number 12(2022)
• Page Start: 1623
• Page End: 1632
• Publication Date: 2022-12-25
• Subjects: PDC -- CD123 -- myelodysplasia -- myeloproliferative neoplasm -- skin; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000001960
• Languages: English
• ISSNs: 0147-5185

Abstract

Abstract : Cutaneous lesions in the setting of myeloproliferative neoplasms and myelodysplastic syndromes are poorly understood. We report 6 patients with pruritic papular eruptions composed of mature T-lymphocytes with large clusters of CD123-positive cells. Double immunohistochemical studies demonstrated a lack of myeloid cell nuclear differentiation antigen in the CD123-positive cells, which expressed SPIB, confirming that they were mature plasmacytoid dendritic cells. Four patients were diagnosed with chronic myelomonocytic leukemia and 2 with myelodysplastic syndromes (AREB-I and myelodysplastic syndromes with 5q deletion, respectively). All patients had a long history of hematological alterations, mainly thrombocytopenia, preceding the cutaneous disorder. Nevertheless, the skin lesions developed in all cases coincidentally with either progression or full-establishment of their hematological disease. Most cutaneous lesions disappeared spontaneously or after corticosteroid treatment. Molecular studies performed in both bone marrow and cutaneous lesions in 2 patients demonstrated the same mutational profile, confirming the specific, neoplastic nature of these mature plasmacytoid dendritic cells-composed cutaneous lesions.