Myelodysplasia Cutis

Abstract

Context.— Myelodysplasia cutis is an emerging concept in cutaneous neoplasia. Many of these cases were previously included under the umbrella of histiocytoid Sweet syndrome. However, with the advent of next-generation sequencing, cutaneous involvement by myelodysplastic syndrome is being increasingly recognized. Objective.— To review histiocytoid Sweet syndrome and myelodysplasia cutis and discuss our current understanding of these entities. Additionally, to discuss how next-generation sequencing can be applied in the evaluation of cutaneous infiltrates of immature histiocytoid cells. Data Sources.— The English-language literature from 2005 to 2023 on the topic of histiocytoid Sweet syndrome and myelodysplasia cutis was reviewed. Conclusions.— Biopsy specimens showing infiltrates of histiocytoid, immature myeloid cells may represent cutaneous involvement by myelodysplastic syndrome. Close clinical correlation is recommended in these cases. Recent studies suggest that next-generation sequencing is useful in separating myelodysplasia cutis from true histiocytoid Sweet syndrome. This distinction has important implications for patients.

Cytophagic histiocytic panniculitis leading to a diagnosis of acute myeloid leukemia with monocytic differentiation: A case report and literature review

“…Myelodysplasia cutis does not include the numerous blast or blast forms seen in leukemia cutis, but rather maturing myeloid forms, including those with a pince-nez or pseudo-Pelger-Huët anomaly. 5 Moreover, hemophagocytic lymphohistiocytosis (HLH) should be on the differential diagnosis, though it can have a variable and nonspecific presentation because prompt diagnosis and treatment are critical. A diagnosis of HLH is made by the presence of at least five of the eight following criteria: fever >38.5 C, splenomegaly, peripheral blood cytopenia, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis in the bone marrow, spleen, lymph node, or liver, low or absent natural killer cell activity, serum ferritin >500 ng/ mL, and elevated soluble CD25 two standard deviations above ageadjusted norms.…”

“…An additional diagnosis to consider is myelodysplasia cutis, which occurs in patients with known MDS who develop cutaneous involvement by atypical, immature, but non‐blastic myeloid cells. Myelodysplasia cutis does not include the numerous blast or blast forms seen in leukemia cutis, but rather maturing myeloid forms, including those with a pince‐nez or pseudo‐Pelger–Huët anomaly 5 . Moreover, hemophagocytic lymphohistiocytosis (HLH) should be on the differential diagnosis, though it can have a variable and non‐specific presentation because prompt diagnosis and treatment are critical.…”

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

“…Different histopathological patterns have been described in the classically considered reactive dermatoses in the setting of MDS/MPN. Since the description of shared molecular alterations, as well as a clinical course related to the hematological disease (persistence of skin lesions and response to the treatment of the myeloid neoplasm), a new term, myelodysplasia cutis (MDS-cutis), has emerged [7]. Below we will detail the main clinical, histopathological and molecular characteristics of these dermatoses, including forms with rapid response to oral corticosteroids and more persistent forms with shared molecular alterations with BM that could be discussed in what is described as MDS-cutis.…”

“…The following supporting information can be downloaded at: https: //www.mdpi.com/article/10.3390/cancers15245888/s1, Table S1: MDS/MPN related dermatoses with indolent clinical course with main clinical, epidemiological, immunophenotypic and genetic features; Table S2: Cutaneous processes related to either MDS/MPN and aggressive clinical behavior with main with main clinical, epidemiological, immunophenotypic and genetic features. References [6,7,12,13,15,16,24,25,29,30,35,[37][38][39][45][46][47][51][52][53][56][57][58][64][65][66][67][68][69][70][71][72][73]75,77,79] are cited in the supplementary materials.…”