Disseminated granulomatous skin lesions associated with myelodysplastic syndrome treated successfully with tranilast: a case report and review of the literature

Yoneta, Kensuke; Fujimoto, Noriki; Teramura, Kazuya; Takayama, Shojiro; Tanaka, Toshihiro

doi:10.1684/ejd.2016.2812

Cited by 11 publications

(12 citation statements)

References 10 publications

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“…In a French monocentric prospective study of 157 patients with primary MDS followed up for a median of 44 months, the prevalence of skin manifestations were close to 10% [80]. Skin lesions in patients with MDS include neutrophilic dermatosis such as SS and PG, leucocytoclasic vasculitis, cutaneous manifestations related to systemic diseases already mentioned (such as Behçet's-like syndrome and systemic vasculitis), and less frequently cutaneous granulomatosis [81,82]. Other skin lesions including cutaneous infections, drug adverse reactions and leukemia cutis (blastic myeloid cell infiltration of the skin) could be observed and must be distinguished from SIADs.…”

Section: Skin Manifestationsmentioning

confidence: 96%

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Jachiet

Fenaux

Sevoyan

et al. 2021

Hemato

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Systemic auto-inflammatory or autoimmune diseases (SIADs) develop in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). With or without the occurrence of SIADs, the distribution of MDS subtypes and the international or CMML-specific prognostic scoring systems have been similar between MDS/CMML patients. Moreover, various SIADs have been described in association with MDS, ranging from limited clinical manifestations to systemic diseases affecting multiple organs. Defined clinical entities including systemic vasculitis, connective tissue diseases, inflammatory arthritis and neutrophilic diseases are frequently reported; however, unclassified or isolated organ impairment can also be seen. Although the presence of SIADs does not impact the overall survival nor disease progression to acute myeloid leukemia, they can help with avoiding steroid dependence and make associated adverse events of immunosuppressive drugs challenging. While therapies using steroids and immunosuppressive treatment remain the backbone of first-line treatment, increasing evidence suggests that MDS specific therapy (hypomethylating agents) and sparing steroids may be effective in treating such complications based on their immunomodulatory effect. The aim of this review was to analyze the epidemiological, pathophysiological, clinical and therapeutic factors of systemic inflammatory and immune disorders associated with MDS.

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Section: Skin Manifestationsmentioning

confidence: 96%

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Jachiet

Fenaux

Sevoyan

et al. 2021

Hemato

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“…17 In the few previous reports of disseminated cutaneous granulomatous lesions in MDS, papules and nodules were observed in all cases, with pruritus present only in 2 of them. 18 Four of 6 patients mentioned by Yoneta et al experienced an acute transformation to acute myeloid leukemia. It seems that chemotherapy is also effective for the skin eruptions, and in 1 case, the authors suggested that tranilast was effective in the treatment of the skin lesions.…”

Section: Discussionmentioning

confidence: 98%

“…It seems that chemotherapy is also effective for the skin eruptions, and in 1 case, the authors suggested that tranilast was effective in the treatment of the skin lesions. 18…”

Section: Discussionmentioning

confidence: 99%

“…It seems that chemotherapy is also effective for the skin eruptions, and in 1 case, the authors suggested that tranilast was effective in the treatment of the skin lesions. 18 The inflammatory infiltrate in the skin of otherwise asymptomatic individuals containing the pathogenic mutation in granuloma-forming histiocytes can be related to the socalled clonal hematopoiesis of indeterminate potential. [19][20][21] We propose that the eventual development of full diagnostic criteria for CMML in these patients supports the hypothesis that these skin lesions are an initial manifestation of the disease rather than a reactive finding.…”

Section: Discussionmentioning

confidence: 99%

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Granulomatous Dermatitis Heralding Myelodisplastic/Myeloproliferative Neoplasms. Neoplastic or Reactive Cells? A Study of 2 Cases

Prieto‐Torres¹,

Santonja

Chamizo

et al. 2022

The American Journal of Dermatopathology

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:Skin manifestations in the context of underlying hematological malignancies are well known and not an infrequent clinical finding. They can represent specific neoplastic infiltrates or be considered as reactive. In the latter group, where granulomatous dermatitis is included, controversy has emerged recently. According to newly reported data, the histiocytes comprising these granulomata can carry the same molecular alterations found in the primary process. Moreover, the skin manifestations in these patients are sometimes the initial clue for the diagnosis of the underlying malignancy. We present here 2 cases with granulomatous skin infiltrates preceding the diagnosis of myelodysplastic/myeloproliferative neoplasms. In one of them, the same IDH2 mutation was detected in granulomatous lesions on the skin and in the bone marrow. This was performed by pyrosequencing instead of next-generation sequencing, with improved cost-effectiveness.

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“…In the setting of MDS/CMML, systemic inflammatory and autoimmune diseases (SIADs) occur in 10 to 30% patients, mostly neutrophilic diseases, connective tissue disorders, arthritis, and vasculitis ( 3 – 7 ). More than half of patients with SIADs have skin involvement, consisting in neutrophilic dermatosis and skin vasculitis, less frequently cutaneous granulomatosis ( 8 – 10 ). Other skin lesions including cutaneous infections, drug adverse reactions, and leukemia cutis (blastic myeloid cell infiltration of the skin) can be observed and must be distinguished from SIADs ( 11 ).…”

Section: Introductionmentioning

confidence: 99%

Myeloid Clonal Infiltrate Identified With Next-Generation Sequencing in Skin Lesions Associated With Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia: A Case Series

et al. 2021

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BackgroundMyelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with cutaneous manifestations. Next-generation sequencing (NGS) is a tool capable of identifying clonal myeloid cells in the skin infiltrate and thus better characterize the link between hematological diseases and skin lesions.ObjectiveTo assess whether skin lesions of MDS/CMML are clonally related to blood or bone marrow cells using NGS.MethodsComparisons of blood or bone marrow and skin samples NGS findings from patients presenting with MDS/CMML and skin lesions in three French hospitals.ResultsAmong the 14 patients recruited, 12 patients (86%) had mutations in the skin lesions biopsied, 12 patients (86%) had a globally similar mutational profile between blood/bone marrow and skin, and 10 patients (71%) had mutations with a high variant allele frequency (>10%) found in the myeloid skin infiltrate. Mutations in TET2 and DNMT3A, both in four patients, were the most frequent. Two patients harbored a UBA1 mutation on hematopoietic samples.LimitationsLimited number of patients and retrospective collection of the data. Blood and skin sampling were not performed at the exact same time point for two patients.ConclusionSkin lesions in the setting of MDS/CMML are characterized by a clonal myeloid infiltrate in most cases.

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Disseminated granulomatous skin lesions associated with myelodysplastic syndrome treated successfully with tranilast: a case report and review of the literature

Cited by 11 publications

References 10 publications

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Granulomatous Dermatitis Heralding Myelodisplastic/Myeloproliferative Neoplasms. Neoplastic or Reactive Cells? A Study of 2 Cases

Myeloid Clonal Infiltrate Identified With Next-Generation Sequencing in Skin Lesions Associated With Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia: A Case Series

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