Pansclerotic morphea: A case report
DOI:
https://doi.org/10.66344/jpad.v31i3.1664Keywords:
Adult, Generalized pansclerotic morphea, steroid pulse therapyAbstract
Generalized disabling pansclerotic morphea is a rare and mutilating form of morphea involving the dermis, subcutaneous tissue, fat, muscle, and even bone. It most often occurs in young children but adults can also be affected. We report a case of rapidly progressing and disabling adult onset generalized pansclerotic morphea, which responded well to methotrexate and steroid pulse therapy.
References
1. Singh A, Singhal K, Choudary S, Bisati S, Arora M. Adult onset unilateral disabling pansclerotic morphea. Indian J Dermatol. 2014;59(3):316.
2. Padmavathy L, Rao LL. Unilateral linear pansclerotic morphea affecting face and limbs. Indian J Dermatol Venereol Leprol. 2005;71:192–4.
3. Maragh SH, Davis MD, Bruce AJ, Nelson AM. Disabling pansclerotic morphea: clinical presentation in two adults. J Am Acad Dermatol.2005;53(2 Suppl 1):S115-9. doi: 10.1016/j.jaad.2004.10.881. PMID: 16021158.
4. Orteu CH. Morphea and Allied Scarring and Sclerosing Inflammatory Dermatoses. In: Griffiths CE, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Wiley Blackwell; 2016. p. 1561.
5. Heck J, Olk J, Kneitz H, Hamm H, Goebeler M. Long-standing morphea and the risk of squamous cell carcinoma of the skin. J Dtsch Dermatol Ges.2020;18(7):669-73.
6. Bielsa I, Ariza A. Deep morphea. Semin Cutan Med Surg.2007;26(2):90–5.
7. Forsea AM, Cretu AN, Ionescu R, et al. Disabling pansclerotic morphea of childhood–unusual case and management challenges. J Med Life.2008;1:348–54.
8. Orteu CH. Morphea and Allied Scarring and Sclerosing Inflammatory Dermatoses. In: Griffiths CE, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Wiley Blackwell; 2016. p. 1562.
9. Florez-Pollack S, Kunzler E, Jacobe HT. Morphea: Current concepts. Clin Dermatol. 2018;36(4):475-86.
doi: 10.1016/j.clindermatol.2018.04.005. Epub 2018 Apr 10. PMID: 30047431.
10. Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. Clin Rev Allergy Immunol. 2017;53(3):306-36.
doi: 10.1007/s12016-017-8625-4. PMID: 28712039.
11. Khatri AM, McLemore M, Malhotra P. Morphea (Localized Scleroderma) Presenting With Recurrent Skin and Soft Tissue Infections: A Diagnostic Dilemma. Cureus.2020;12(5): e8067. www.ncbi.nlm.nih.gov/pmc/articles/PMC7290118/#.
12. Albuquerque JV de, Andriolo BNG, Vasconcellos MRA, Civile VT, Lyddiatt A, Trevisani VFM. Interventions for morphea. Cochrane Database Syst Rev.2019;7. Art. No.: CD005027. DOI: 10.1002/14651858.CD005027.pub5
13. Kreuter, Alexander & Gambichler, Thilo & Breuckmann, Frank & Rotterdam, Sebastian & Freitag, Marcus & Stuecker, Markus & Hoffmann, Klaus & Altmeyer, Peter. Pulsed High-Dose Corticosteroids Combined With Low-Dose Methotrexate in Severe Localized Scleroderma. Arch Dermatol.2005;141:847-52.
14. Qayoom S, Rather S, Manzoor S, Sameen F. Evaluation of corticosteroid and methotrexate therapy in the treatment of moderate to severe localized scleroderma (Morphoea) –A 5 year study in a teaching hospital of North India. Int J Contem Med Res.2019;6(5):E1-E6.
15. Werth VP, 2020. Morphea; Treatment & Management. [online] Emedicine.medscape.com. Available at: https://emedicine.medscape.com/article/1065782-treatment?src=mbl_msp_android&ref=share
16. Roldan R, Morote G, Castro Mdel C, Miranda MD, Moreno JC, Collantes E. Efficacy of bosentan in treatment of unresponsive cutaneous ulceration in disabling pansclerotic morphea in children. J Rheumatol.2006;33(12):2538-40. Erratum in: J Rheumatol.2007;34(8):1786. Dosage error in published abstract; MEDLINE/PubMed abstract corrected. PMID: 17143989.
17. Wollina U, Buslau M, Heinig B, Petrov I, Unger E, Kyriopoulou E, Koch A, Köstler E, Schönlebe J, Haroske G, Doede T, Pramatarov K. Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma. Int J Low Extrem Wounds. 2007;6(4):291-8.
doi: 10.1177/1534734607308731. PMID: 18048875.
Downloads
Published
Issue
Section
License
Copyright (c) 2021 Anum Sadia, Anum Sadia
This work is licensed under a Creative Commons Attribution 4.0 International License.
Submission declaration
Authors retain the copyright to their work and grant the 'Journal of Pakistan Association of Dermatologists (JPAD)' the right of first publication under a Creative Commons Attribution 4.0 International (CC BY 4.0) license. This license allows others to share, adapt, and reuse the work for any purpose, including commercial use, as long as appropriate credit is given to the original authors and the journal.
By submitting a manuscript, authors confirm that the work has not been published previously (except as an abstract, lecture, or academic thesis), is not under review elsewhere, and has been approved by all authors and relevant authorities. Once accepted, the article will be openly accessible under the CC BY 4.0 license, ensuring wide dissemination and reuse with proper attribution.
