P92 Cutaneous manifestations of myelodysplastic syndrome: a systematic review

Abstract

Abstract A variety of associations between cutaneous manifestations and myelodysplastic syndrome (MDS) have been described, but the frequency is unknown. The presence of cutaneous manifestations may have therapeutic and prognostic significance, as the presence thereof has been reported to be associated with poor prognosis or acute myeloid leukaemia (AML) progression, or may precede the diagnosis of MDS [Farah C, Bulai Livideanu C, Jegu J et al. Prevalence and prognostic value of cutaneous manifestations in patients with myelodysplastic syndrome. J Eur Acad Dermatol Venereol 2010; 24:1171–5; Ghoufi L, Ortonne N, Ingen-Housz-Oro S et al. Histiocytoid Sweet syndrome is more frequently associated with myelodysplastic syndromes than the classical neutrophilic variant: a comparative series of 62 patients. Medicine (Baltimore) 2016; 95:e3033]. We aimed to determine the types and prevalence of cutaneous manifestations in MDS, treatment options and prognosis associated with specific cutaneous manifestations and MDS subtypes. PubMed, MEDLINE and Embase were searched from inception up to 26 January 2023 for studies reporting cutaneous manifestations of MDS in adults aged ≥ 18 years, as per the World Health Organization classification. Case reports and case series were included. The review was registered with PROSPERO (CRD42021293140). In total, 92 studies were included (case reports n = 71, case series n = 21), consisting of 140 patients [98 male, mean age 61.0 (SD 15.4)]. We identified six common cutaneous manifestations: neutrophilic, e.g. Sweet syndrome and pyoderma gangrenosum (n = 66); vasculitis, e.g. leucocytoclastic vasculitis and Behçet disease (n = 21); granulomatous, e.g. granuloma annulare and interstitial granulomatous dermatitis (n = 8); connective tissue disease (CTD), e.g. dermatomyositis (n = 7); panniculitis (n = 4); immunobullous (n = 2); and other (n = 30). Sweet syndrome (81.8%) and pyoderma gangrenosum (18.2%) were the commonest presentations of neutrophilic dermatosis. Cutaneous features either occurred at time of MDS diagnosis in 24.7%, preceded the diagnosis in 36.1% (mean 17 months; range 0.5–216), or after diagnosis in 39.2% (mean 20.6 msonths; range 1–132). Prognosis was poor (40.2% death) with 34.1% progressing to acute myeloid leukaemia (AML), 50% of which had neutrophilic dermatosis. Death occurred in 38.5% of neutrophilic dermatoses (median time from onset of cutaneous manifestation: 12 months), 50% of vasculitis (7.5 months), 62.5% of granulomatous (15.5 months), 14.3% of CTD (7 months), and none reported in immunobullous and panniculitis. Granulomatous skin lesions had the poorest prognosis, 60% of which had progressed to AML. This systematic review highlights the range of cutaneous manifestations associated with myelodysplastic syndrome. Of note, we found that the majority of cases progressing to AML were associated with neutrophilic dermatoses overall; however, granulomatous skin lesions had the poorest prognosis with the highest proportion progressing to AML. We speculate that these manifestations may confer an increase in risk of transformation, compared with other cutaneous changes.