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Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review

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Abstract

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations include a combination of nodules affecting peri-articular (especially interphalangeal joints) and head and neck areas; and linearly arranged ivory white papules over an erythematous indurated skin. Despite a benign course, an abrupt onset of symptoms with extensive cutaneous involvement often leads to parental anxiety, overenthusiastic evaluation and sometimes aggressive treatment. A peculiar cutaneous distribution in SHJCM including nodular lesions and periorbital edema, arthritis and arthralgia in a few cases, may simulate juvenile dermatomyositis. It is, therefore, important for dermatologists and pediatricians to be aware of this entity. In this report, we describe two cases of SHJCM and briefly review similarly reported cases in children.

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Author notes

  1. Anuradha Bishnoi, Ankur Kumar Jindal, and Gummadi Anjani contributed equally to the manuscript as first authors.

Authors and Affiliations

  1. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Anuradha Bishnoi, Keshavamurthy Vinay & Sunil Dogra

  2. Department of Pediatric Clinical Immunology and Rheumatology, Allergy Immunology Unit, Postgraduate Institute of Medical Education and Research, Advanced Pediatric Center, Chandigarh, 160012, India

    Ankur Kumar Jindal, Gummadi Anjani, Pratap Kumar Patra & Surjit Singh

  3. Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Debajyoti Chatterjee

  4. Dr AJ Kanwar Skin Clinic, Greater Kailash, New Delhi, 110048, India

    Amrinder J. Kanwar

Authors
  1. Anuradha Bishnoi
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  2. Ankur Kumar Jindal
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  3. Gummadi Anjani
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  4. Pratap Kumar Patra
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  5. Debajyoti Chatterjee
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  6. Keshavamurthy Vinay
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  7. Amrinder J. Kanwar
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  8. Sunil Dogra
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  9. Surjit Singh
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Contributions

AB, AJ, GA: Patient management, concept and draft of the manuscript, critical review of literature; PK: Patient management, critical review of literature; DC: Examination of histopathology slides; KV: Patient management, the concept of the manuscript, critical review of the manuscript for important intellectual content and final approval of the version to be published; AJK: Patient management and critical review of literature; SD, SS: patient management, critical review of the manuscript for important intellectual content. All co-authors take full responsibility for the integrity of the study and the final manuscript.

Corresponding author

Correspondence to Keshavamurthy Vinay.

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Bishnoi, A., Jindal, A.K., Anjani, G. et al. Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review. Rheumatol Int 40, 1911–1920 (2020). https://doi.org/10.1007/s00296-020-04578-5

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  • DOI: https://doi.org/10.1007/s00296-020-04578-5

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