Unique Recognizable Histopathologic Variant of Palisaded Neutrophilic and Granulomatous Dermatitis that Is Associated With SRSF2-Mutated Chronic Myelomonocytic Leukemia: Case Report and Review of the Literature. (1st March 2022)

• Record Type: Journal Article
• Authors: Enescu, Christina D.; Patel, Avni; Friedman, Ben J.
• Is Part Of: American journal of dermatopathology. Volume 44:Number 3(2022)
• Page Start: e33
• Page End: e36
• Publication Date: 2022-03-01
• Subjects: palisaded neutrophilic and granulomatous dermatitis -- chronic myelomonocytic leukemia -- granulomatous dermatitis -- monocytosis -- SRSF2; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000002085
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract: Palisaded neutrophilic and granulomatous dermatitis (PNGD) represents a cutaneous histopathologic reaction spectrum associated with several underlying disorders. Few cases of PNGD have been associated with chronic myelomonocytic leukemia (CMML), a malignant hematopoietic disorder with features in between those of a myeloproliferative neoplasm and myelodysplastic syndrome. We present a patient with a generalized papular skin reaction involving the neck, chest, and shoulders with histomorphological features on the spectrum of PNGD. Subsequent laboratory workup demonstrated a persistent mild monocytosis, raising concern for CMML. The diagnosis was ultimately confirmed with a bone marrow biopsy and associated mutational analysis through next-generation sequencing which identified deleterious variants in SRSF2, IDH2, and ASXL1. The findings in this case strengthen the previously made association between PNGD and SRSF2-mutated CMML and may help better define a unique recognizable clinical–histopathological–molecular subtype for dermatopathologists.