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Pansclerotic morphea in childhood: a case report

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Abstract

Scleroderma can be differentiated into progressive systemic and circumscript forms. The extensive form with lethal outcome is known from case reports of children and adolescents. The present case report concerns a boy who died at 16 years of age. In the 5th year of life, he experienced weight loss and developed multiple, firm, partially atrophic plaques in the skin of the extremities. These plaques gradually became confluent and extended over the whole torso and head. Plaque ulceration resulted in massive mutilations to the body. Later the patient's cachexia worsened and he developed keratose, moderately differentiated squamous cell carcinoma of the right leg. The prognosis of pansclerotic morphea for children is worse than for adults. No successful therapy is known.

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References

  1. Ozkan S, Atebey N, Fetil E, Erkizan V, Gunes AT (2000) Evidence for borrelia burgdorferi in morphea and lichen sclerosus. Int J Dermatol 39:278–283

    Google Scholar 

  2. De Vito JR, Merogi AJ, Vo T Boh EE, Fung HK, Freeman SM, Cockerell C, Stewart K, Marrogi AJ (1996) Role of borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus : a PCR study of 35 cases. J Cutan Pathol 23:350–358

    PubMed  Google Scholar 

  3. Diaz-Perez JL, Connolly SM, Winkelmann RK (1980) Disabling pansclerotic morphea in children. Arch Dermatol 116:169–173

    Article  CAS  PubMed  Google Scholar 

  4. Gruss C, Stücker M, von Kobyletzki G, Schreiber D, Altmeyer P, Kerscher M (1997) Low dose UVA1 phototherapy in disabling pansclerotic morphea of childhood. Br J Dermatol 136:293–294

    CAS  Google Scholar 

  5. Stücker M, Schreiber D, Gruss C, Freitag M, von Kobyletzki G, Kerscher M, Altmeyer P (1999) Schwerer Verlauf einer mutilierenden pansklerotischen zirkumskripten Sklerodermie im Kindesalter. Hautarzt 50:131–135

    Article  PubMed  Google Scholar 

  6. Todd DJ, Askari A, Ektaish E (1998) PUVA therapy for disabling pansclerotic morphea in childhood. Br J Dermatol 138:2081–2082

    Article  Google Scholar 

  7. Ferrandiz C, Henkes J, Gonzalez J, Peyri J (1981) Incapacitating pansclerotic morphea in childhood. Med Cutan Ibero Lat Am 9:377–382

    CAS  PubMed  Google Scholar 

  8. Wollina U, Looks A, Uhlemann C, Wollina K (1999) Panclerotic morphea of childhood—follow-up over 6 years. Pediatr Dermatol 16:245–247

    Article  CAS  PubMed  Google Scholar 

  9. Parodi PC, Riberti C, Draganic Stinco D, Patrone P, Stinco G (2001) Squamous cell carcinoma arising in a patient with long-standing pansclerotic morphea. Br J Dermatol 144:417–419

    Article  CAS  PubMed  Google Scholar 

  10. Scharfetter-Kochanek K, Goldermann R, Lehmann P (1995) PUVA-therapy in disabling pansclerotic morphea of children. Br J Dermatol 132:830–831

    PubMed  Google Scholar 

  11. Kerscher M, Dirschka T, Volkenadt M (1995) Treatment of localized scleroderma by UVA 1 phototherapy. Lancet 346:1166

    CAS  PubMed  Google Scholar 

  12. Kerscher M, Volkenandt M, Meurer M, Lehmann P, Plewig G, Rocken M (1994) Treatment of localized scleroderma by PUVA bath photochemotheraoy. Lancet 343:1233

    CAS  Google Scholar 

  13. Wollina U, Looks A, Schneider R, Maak B (1998) Disabling morphoea of childhood—beneficial effect of intravenous immunoglobulin therapy. Clin Exp Dermatol 23:292–293

    Article  CAS  PubMed  Google Scholar 

  14. Wollina U, Karte K, Herold C, Looks A (2000) Biosurgery in wound healing—the renaissance of maggot therapy. J Eur Acad Dermatol Venereol 14:285–289

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Abteilung Kinderchirurgie, Klinikum der FSU Jena, Bachstrasse 18, 07740, Jena, Germany

    T. Doede, F. Schier & M. Bondartschuk

  2. Klinik für Dermatologie und Dermatologische Allergologie, Erfurter Strasse 35, 07740, Jena, Germany

    U. Wollina

  3. Institut Pathologie, Klinikum der FSU Jena, Bachstrasse 18, 07740, Jena, Germany

    W. Hindermann

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  1. T. Doede
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  2. U. Wollina
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  3. W. Hindermann
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  4. F. Schier
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  5. M. Bondartschuk
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Correspondence to T. Doede.

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Doede, T., Wollina, U., Hindermann, W. et al. Pansclerotic morphea in childhood: a case report. Ped Surgery Int 19, 406–408 (2003). https://doi.org/10.1007/s00383-003-1020-7

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  • DOI: https://doi.org/10.1007/s00383-003-1020-7

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