Paraneoplastic Skin Findings in Patients with Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia
Deniz Peker, Lynn Moscinski, Rahel Mathew, Ling Zhang. H. Lee Moffitt Cancer Center, Tampa, FL
Background: Myelodysplastic syndromes (MDS) are clonal hematologic neoplasms which rarely involve the skin during their stable phase. Skin manifestations including leukemia cutis and other paraneoplastic lesions (generalized granuloma annulare, Sweet syndrome, pyoderma gangrenosum, folliculitis, follicular and papular mucinosis, blastic plasmacytoid dendritic neoplasm (BPDCN), and erythema nodosum) have occasionally been reported in hematologic malignancies. However, a large series of study of this rare phenomenon is lacking making it difficult to explore its relationship with MDS. Literature review is conducted.
Design: All skin biopsies from patients with the diagnosis of MDS and CMML were retrieved from our institution during 1/2003 to 12/2010. Cases with potential paraneoplastic lesions were identified. The corresponding bone marrow biopsies, laboratory studies and cytogenetics at the time of any skin eruption were reviewed. Clinical outcomes were correlated according to patients' chart reviews.
Results: 408 patients with MDS (306, 75%) and CMML (102, 25%) were reviewed and 9 cases with skin manifestations excluding leukemia cutis, drug eruption, infectious process, and graft versus host disease were identified. Four of 9 (44.4%) cases including pyoderma gangrenosum (n=1), Sweet's syndrome (n=1), and BPDCN (n=2) were confirmed to be paraneoplastic lesions in the presence of MDS (n=3) and CMML (n=1). Among these 4 cases, 2 patients presented with localized skin disease and 2 patients with BPDCN had disseminated lesions. All 4 patients showed disease progression to higher grade MDS or evolving AML within an average of 13 weeks (ranging from 2 to 28 weeks) of their cutaneous manifestation. Progression to a high grade disease was not observed in cases with florid folliculitis and septolobular panniculitis (n=2). The remaining 3 skin biopsies were diagnosed as granulomatous vasculitis, psoriasiform dermatitis (eczematous process) and atypical lymphohistiocytic infiltrate in CMML with lack of primary disease progression and were not considered paraneoplastic.
Conclusions: Recognition and tissue diagnosis of skin lesions in patients with MDS and CMML is crucial for identifying paraneoplastic lesions. Similar to literature reports, BPDCN, Sweet syndrome and pyoderma gangrenosum are often associated with disease progression. It is uncertain if florid folliculitis and septolobular panniculitis are pure inflammatory or paraneoplastic processes. Large case series are warranted to further explore the mechanism and prognostic significance of paraneoplastic skin lesions in MDS and CMML.
Tuesday, March 20, 2012 9:30 AM
Poster Session III # 238, Tuesday Morning