A Case of Morphea (Localized Scleroderma)

Authors

Keywords:

morphea, scleroderma, pansclerotic

Abstract

A 48-year-old, single woman initially presented with a solitary, rounded lesion in the gluteal region that, over the course of one year, progressed to involve the inguinal area, abdomen, and both upper and lower extremities, ultimately affecting a large proportion of the body. Lesions began as inflammatory erythematous plaques and subsequently evolved into shiny, sclerotic plaques with eventual central hypopigmentation and atrophy. Clinical and histopathological findings were consistent with localized scleroderma (morphea) of a generalized/pansclerotic phenotype. The disease course was accompanied by hair loss and weight gain and was associated with psychosocial deterioration. Systemic immunomodulatory therapy with methotrexate plus systemic corticosteroid (prednisone) was initiated; following treatment, the patient developed depressive symptoms attributed in part to medication-related adverse effects, and psychiatric management with sertraline combined with a low-dose antipsychotic (aripiprazole) resulted in clinical improvement. Neurological imaging and cardiac and basic pulmonary assessments were largely unremarkable, and no further pulmonary investigations were performed in the absence of respiratory symptoms. This case highlights the potential for extensive cutaneous and extracutaneous involvement in generalized morphea and underscores the importance of multidisciplinary follow-up, proactive management of treatment-related psychiatric morbidity, and routine psychosocial assessment to optimize adherence and quality of life.

References

1.Peterson LS, Nelson AM, Su WP, Mason T, O’Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997;24(1):73–80.

2.Fett N, Werth VP. Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis. Vol. 64, Journal of the American Academy of Dermatology. Mosby Inc.; 2011. p. 217–28.

3.O’Brien JC, Rainwater YB, Malviya N, Cyrus N, Auer-Hackenberg L, Hynan LS, et al. Transcriptional and Cytokine Profiles Identify CXCL9 as a Biomarker of Disease Activity in Morphea. Journal of Investigative Dermatology. 2017;137(8):1663–70.

4.Mirizio E, Liu C, Yan Q, Waltermire J, Mandel R, Schollaert KL, et al. Genetic Signatures From RNA Sequencing of Pediatric Localized Scleroderma Skin. Front Pediatr. 2021;9.

5.Chen HW, Zhu JL, Martyanov V, Tsoi LC, Johnson ME, Barber G, et al. Gene Expression Signatures in Inflammatory and Sclerotic Morphea Skin and Sera Distinguish Morphea from Systemic Sclerosis. Journal of Investigative Dermatology. 2023;143(10):1886-1895.e10.

6.Baghdassarian H, Blackstone SA, Clay OS, Philips R, Matthiasardottir B, Nehrebecky M, et al. Variant STAT4 and Response to Ruxolitinib in an Autoinflammatory Syndrome. New England Journal of Medicine. 2023;388(24):2241–52.

7.Jacobe H, Ahn C, Arnett FC, Reveille JD. Major Histocompatibility Complex Class I and Class II Alleles May Confer Susceptibility to or Protection Against Morphea: Findings From the Morphea in Adults and Children Cohort. Arthritis & Rheumatology. 2014;66(11):3170–7.

8.Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe HT. Distinct Autoimmune Syndromes in Morphea. Arch Dermatol. 2009;145(5).

9.Grabell D, Hsieh C, Andrew R, Martires K, Kim A, Vasquez R, et al. The role of skin trauma in the distribution of morphea lesions: A cross-sectional survey of the Morphea in Adults and Children cohort IV. J Am Acad Dermatol. 2014;71(3):493–8.

10.Teske NM, Jacobe HT. Morphea (Localized Scleroderma). In: Scleroderma. Cham: Springer International Publishing; 2024. p. 123–47.

11.Prasad S, Zhu JL, Schollaert-Fitch K, Torok KS, Jacobe HT. An Evaluation of the Performance of Current Morphea Subtype Classifications. JAMA Dermatol. 2021;157(4):399.

12.Chen HW, Walker AM, Stein AB, Chen SL, Schollaert-Fitch KL, Torok KS, et al. Clinical Characteristics Associated with Musculoskeletal Extracutaneous Manifestations in Pediatric and Adult Morphea: A Prospective, Cohort Study. Journal of Investigative Dermatology. 2023;143(10):1955-1963.e3.

13.Zulian F. New developments in localized scleroderma. Curr Opin Rheumatol. 2008;20(5):601–7.

14.Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol. 2007;56(2):257–63.

15.Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, et al. Ocular involvement in children with localised scleroderma: a multi-centre study. British Journal of Ophthalmology. 2007;91(10):1311–4.

16.Niklander S, Marin C, Martinez R, Esguep A. Morphea en coup de sabre: An unusual oral presentation. J Clin Exp Dent. 2017;0–0.

17.Dehen L, Roujeau JC, Cosnes A, Revuz J. Internal Involvement in Localized Scleroderma. Medicine. 1994;73(5):241–5.

18.Elinkichari D, Tabka M, Souissi A, Alaoui F, Chelly I, Haouet S, Mokni M. Localized scleroderma with pulmonary arterial hypertension and pulmonary interstitial fibrosis in a patient with positive Th/to antibodies: Case report and review of literature. Clin Case Rep. 2023 May 16;11(5):e7183.

19.Dahl J, Ormstad H, Aass HC, Malt UF, Bendz LT, Sandvik L, Brundin L, Andreassen OA. The plasma levels of various cytokines are increased during ongoing depression and are reduced to normal levels after recovery. Psychoneuroendocrinology. 2014 Jul;45:77-86.

20.Yang Y, Gu K, Li J. Relationship between serum inflammatory cytokines and suicide risk in patients with major depressive disorder. Front Psychiatry. 2024 Jun 27;15:1422511.

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Published

24.09.2025 — Updated on 30.12.2025

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