Clinicohistopathological correlations in juvenile localized scleroderma: Studies on a subset of children with hypopigmented juvenile localized scleroderma due to loss of epidermal melanocytes - 28/07/11
Correspondence to: Amy E. Gilliam, MD, Dermatology Palo Alto Foundation Medical Group, 795 El Camino Real, Palo Alto, CA 94301.Abstract |
Background |
Localized scleroderma or morphea is a connective tissue disorder characterized by fibrosis of the skin and subcutaneous tissue. Excessive accumulation of collagen underlies the fibrosis, yet the pathogenesis is unknown. A subset of localized scleroderma/morphea, juvenile localized scleroderma (JLS), affects children and adolescents.
Objectives |
The clinical and microscopic features of JLS have not been fully characterized. The goal is to better characterize the microscopic features of JLS.
Methods |
We collected a distinctive data set of 35 children with JLS, 19 (54%) of whom presented with hypopigmented lesions, and performed a retrospective chart and pathology review. We had adequate tissue for immunostaining studies on 8 of these individuals.
Results |
We found that: (1) CD34 and factor XIIIa immunostaining, reported previously in adult morphea and scleroderma, when used with clinical information, is valuable for confirming a diagnosis of JLS; and (2) presence of hypopigmented lesions in JLS correlates with immunostaining studies. Decreased numbers of MelanA+ melanocytes were present at the dermoepidermal junction in lesional skin in two of 3 children with hypopigmented JLS and in two of 4 children with nonhypopigmented JLS.
Limitations |
The number of cases is small, a function of the small number of children who have biopsy specimens with material sufficient for multiple immunostaining procedures.
Conclusions |
These results provide a useful immunostaining method for confirmation of the diagnosis of JLS. They suggest a complex autoimmune phenotype in some children with JLS.
Le texte complet de cet article est disponible en PDF.Key words : CD34, dermatology, dermatopathology, factor XIIIa, hypopigmentation, immunostaining, juvenile localized scleroderma, morphea, pediatric
Abbreviations used : DEJ, FXIIIa, JLS, UCSF
Plan
| Supported in part by the Dermatology Foundation and the National Institutes of Health (NIH). Dr Amy E. Gilliam was supported by a Dermatology Foundation Medical Dermatology Career Development Award. Dr Anita C. Gilliam was supported by NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) R01 AR 049284 and the NIH NIAMS Case Skin Diseases Research Center P30-AR. The sponsors had no role in the design and conduct of the study; in the collection, analysis, and interpretation of data; or in the preparation, review, or approval of the manuscript. |
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| Conflicts of interest: None declared. |
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| Dr Sung is currently affiliated with the Department of Dermatology, the Permanente Medical Group, Walnut Creek, CA; Dr Chen with the Department of Dermatology, University of California at Irvine; and Dr Amy E. Gilliam (Dermatology) and Anita C. Gilliam (Dermatology and Dermatopathology), Palo Alto Foundation Medical Group, Palo Alto, CA. |
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| Reprints not available from the authors. |
Vol 65 - N° 2
P. 364-373 - août 2011 Retour au numéro
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