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

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. Histolo...

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Bibliographic Details
Published in:BMC dermatology 2020-12, Vol.20 (1), p.18-18, Article 18
Main Authors: Rabee, Hadi, Tayem, Leeda, Gharbeyah, Mohammad, Abugaber, Dina
Format: Article
Language:English
Subjects:
Acute lymphocytic leukemia
Biopsy
Bone marrow
Cardiomyopathy
Case Report
Case reports
Chemotherapy
Fever
Gastric cancer
Health aspects
Immunoglobulins
Intensive care
Leukemia
Lymphoma
Patients
Sepsis
Skin
Skin diseases
Stem cells
Thyroid diseases
Transplants & implants
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Summary: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. 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). 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.
ISSN:1471-5945
1471-5945
DOI:10.1186/s12895-020-00118-7