Scleromyxedema: long‐term follow‐up after high‐dose melphalan with autologous stem cell transplantation

Chockalingam, Ramya; Duvic, Madeleine

doi:10.1111/ijd.13315

Cited by 17 publications

(10 citation statements)

References 16 publications

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“…Multiple cases of SMX treated with autologous SCT have been reported since the first report of a complete remission was published in 2001 51 . STC was used with thalidomide and IVIG, 39 steroids, melphalan, or other chemotherapies 51‐60 . It is hypothesized that cellular components of the bone marrow and secondary lymphatic organs are a source of the circulating pro‐fibrogenic factors involved in SMX.…”

Section: Discussionmentioning

confidence: 99%

“…Improvement in clinical symptoms and variable effects on monoclonal gammopathy levels was shown in several studies. Although melphalan was often considered a first‐line treatment for SMX, as a single agent or in combination therapy, 29,30,39,51‐57,59,60,67 the potential for drug‐related serious adverse events like hematologic malignancy and septic complications limits the use of this agent. Chlorambucil has been reported to be moderately myelosuppressive and leukemogenic and is a potentially a less toxic agent 68 …”

Section: Discussionmentioning

confidence: 99%

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Scleromyxedema treatment: a systematic review and update

2020

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Importance Scleromyxedema is a chronic disease with high morbidity and mortality and no definitive therapeutic guidelines. Objective To review all available data on the efficacy and the safety of the available treatments of scleromyxedema and suggest a possible therapeutic approach. Evidence Review We performed a systematic literature review in Pubmed/Medline, Embase, and Cochrane collaboration databases, searching for all articles since 1990 on the treatments of scleromyxedema, with no limits on participant age, gender, or nationality. Findings Ninety-seven studies were included in this systematic review, of which one prospective, two retrospective, 70 case reports/case series, and 24 letters/correspondence/ clinical image. Intravenous immunoglobulin (IVIG) was the most used first-line therapy based on its efficacy and its generally well-tolerated nature; most patients require continued treatment to remain in remission. Thalidomide and systemic glucocorticoids were mostly considered as second-line therapies and were given alone or in association with IVIG. Patients with severe or refractory disease were treated with autologous bone marrow transplantation, melphalan, or bortezomib with dexamethasone. Conclusions and Relevance Consideration of patient comorbidities, disease distribution, clinician experience, and treatment accessibility is mandatory in every therapeutic approach of scleromyxedema.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Scleromyxedema treatment: a systematic review and update

2020

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“…Although the clinical picture of pachyderma is relatively unequivocal, there is no clear histological definition of pachyderma, as the thickening may have various origins. Tumoral infiltration of neoplastic lymphocytes seems the main reason for pachyderma in FMF [ 11 ] and MF, tumoral infiltration of leukemic cells in LC [ 15 ], granulomatous infiltration in the case of leprosy, lymphatic and edematous stowage in patients with lymphatic insufficiency [ 1 ] or scleromyxedema [ 7 ], and dense lymphocytic infiltration in the case of prurigo and Norwegian scabies [ 6 ]. Granulomatous slack skin may also present a pachydermic aspect, particularly in the skin folds.…”

Section: Discussionmentioning

confidence: 99%

“…Pachyderma is defined as substantially thickened skin with deep folds, as observed in pachyderm animals such as elephants, rhinoceros, and hippopotami. Pachyderma in humans may be observed in several medical conditions – among others, during chronic severe lymphatic insufficiency with lymphedema [ 1 ], pachydermoperiostosis (Touraine-Solente-Golé syndrome) [ 2 ], keratitis-ichthyosis-deafness syndrome [ 3 ], congenital or secondary cutis verticis gyrata [ 4 , 5 ], crusted scabies [ 6 ], severely infiltrated scleromyxedema [ 7 ], especially of the face, and lepromatous leprosy, presenting as leonine facies. White sponge nevus is considered a manifestation of pachyderma of the intraoral mucosa.…”

Section: Introductionmentioning

confidence: 99%

Pachyderma in Primary Cutaneous NK and T-Cell Lymphoma and Leukemia Cutis

Lebas

Chián²,

Nikkels‐Tassoudji

et al. 2017

Case Rep Dermatol

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Background: Pachyderma is defined as severely thickened skin with deep folds and is occasionally observed with primary cutaneous NK and T-cell lymphoma (pCNKTCL), primary cutaneous B-cell lymphoma (pCBCL), and leukemia cutis (LC). Aim: To describe the clinical, histological, and therapeutic particularities of a series of pCNKTCL, pCBCL, and LC patients with pachyderma. Results: In a series of pCNKTCL (n = 70), pCBCL (n = 12), and LC (n = 2) patients followed up during 9 years, 6 cases of pachyderma were observed. Pachyderma occurred on the arms (n = 2), thighs (n = 1), forehead (n = 1), and face (n = 2). The mean age of the patients was 69 years (51–82). The stages were erythrodermic (T4) mycosis fungoides (MF) (n = 1), folliculotropic MF (FMF) (n = 2), classic (T2) MF (n = 2), and chronic myeloid leukemia (n = 1). The erythrodermic MF patient with acute pachyderma on the right arm responded rapidly to oral steroids. The other cases were indolent, appeared progressively, and were highly treatment resistant. Histology revealed dense dermal neoplastic infiltration. The immunohistological profile of the pachydermic lesions was similar to common MF and LC. Conclusion: Pachyderma is an atypical manifestation of MF and LC and may occur on the face (FMF) or the extremities (MF). The rapidly appearing pachyderma may be transitory and responds readily to oral steroids.

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“…Multiple regimens were used. These included the use of melphalan chemotherapy with autologous bone marrow transplant [15], and thalidomide with prednisolone [16]. High dose IVIG has recently become a more popular option and has been a first line strategy used in the recently reported cases [17].…”

Section: Fig 1 Infiltrated Plaques On the Thighmentioning

confidence: 99%

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report

et al. 2020

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Background Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Some atypical forms of the disease were reported in the literature, but none were reported in acute leukemia. Case presentation Herein, we report a case of a 21 years old female patient, known case of acute lymphoblastic leukemia (ALL), who developed numerous hyper-pigmented erythematous papules and plaques, mainly over her thighs, lower abdomen, and sub-mammary flexures. Histopathology of skin lesions confirmed the diagnosis of atypical scleromyxedema. Her symptoms significantly improved with the use of high dose intravenous immunoglobulin (IVIG). Conclusions Despite that scleromyxedema is associated with many hematologic disorders, it is very rarely associated with acute lymphoblastic leukemia, and a high index of suspicion is needed for diagnosis. IVIG remains a reasonable management of such a disabling disease.

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Scleromyxedema: long‐term follow‐up after high‐dose melphalan with autologous stem cell transplantation

Abstract: High-dose melphalan with autologous stem cell transplantation appears to be a safe and effective long-term treatment in patients with scleromyxedema. However, further studies are required to investigate this in larger groups of patients.

Cited by 17 publications

References 16 publications

Scleromyxedema treatment: a systematic review and update

Scleromyxedema treatment: a systematic review and update

Pachyderma in Primary Cutaneous NK and T-Cell Lymphoma and Leukemia Cutis

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report

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