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CC BY 4.0 · Journal of Child Science 2022; 12(01): e1-e4
DOI: 10.1055/s-0041-1741055
Case Report

The First Presentation of Localized Scleroderma at Birth: Scleroderma as a Differential Diagnosis of Congenital Skin Lesion

Elmira Hajiesmaeil Memar
1   Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran
2   Children's Medical Center, Pediatric Center of Excellence, Tehran, Iran
,
Moeinadin Safavi
2   Children's Medical Center, Pediatric Center of Excellence, Tehran, Iran
3   Department of Pathology, Tehran University of Medical Sciences, Tehran, Iran
,
Mohammad-Hassan Moradinejad
2   Children's Medical Center, Pediatric Center of Excellence, Tehran, Iran
4   Pediatric Rheumatology Society of Iran, Tehran, Iran
,
Vahid Ziaee 
1   Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran
2   Children's Medical Center, Pediatric Center of Excellence, Tehran, Iran
4   Pediatric Rheumatology Society of Iran, Tehran, Iran
5   Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Science, Tehran, Iran
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Funding None.
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Abstract

Localized scleroderma is an uncommon autoimmune disease characterized by fibrosis of the skin and underlying tissue without involvement of blood vessels or internal organs. It usually affects children during later childhood, and early presentation of localized scleroderma during infancy is rare. In the current study, we report a child with localized scleroderma-related presentations occurring at birth. A 2-day-old male neonate presented with a firm, erythematous, and slightly pigmented plaque on his left thigh, leading to a change in the diameter of the affected foot and contracture of the left knee. At the age of 7 months, he was referred to our rheumatology clinic with normal growth and development. Laboratory studies, including urine and blood high-performance liquid chromatography assay, antinuclear antibodies, antitopoisomerase I, and rheumatic factor, were in the normal range. No signs of ocular involvement were noted during ophthalmological consultation. Skin biopsy showed mild acanthosis and collagen bundles, which replaced the fat around the sweat glands. A final diagnosis of localized scleroderma was made. Treatment was started with oral prednisolone, oral methotrexate (MTX), and colchicine. The skin lesion stopped progressing after 3 months of treatment. Steroid was then tapered over 6 months, while MTX and colchicine were continued for 2 years. Localized scleroderma during early infancy is a rare disease, but it should be considered as a differential in infants with erythematous and firm lesions on their body at birth because early treatment can prevent future complications.

Keywords

localized scleroderma - linear scleroderma - diagnosis - early infancy - morphea

Authors' Contributions

E.H.M. performed data gathering and drafting of the manuscript. M.S. performed pathology study and contributed to interpretation of the pathologic findings. V.Z. provided the concept and case of need for the survey, clinical expertise, and interpretation of the clinical data and critical revision of the final draft of manuscript. All authors read and approved the final version of the manuscript.


Informed Consent

Informed consent was obtained from the patient's parent.




Publikationsverlauf

Eingereicht: 08. März 2021

Angenommen: 13. November 2021

Artikel online veröffentlicht:
10. Februar 2022

© 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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