medigraphic.com
ISSN 0210-5187 (Electronic)

2009, Number 4

<< Back Next >>

Med Cutan Iber Lat Am 2009; 37 (4)

Disabling pansclerotic morphoea of children: a new case report

Galán GM, Rodríguez BA, Vázquez BMC, Jiménez PR, Collantes EE, Moreno GJC

Language: Spanish
References: 27
Page: 175-179
PDF size: 263.40 Kb.


ABSTRACT

Panesclerotic morphea of the childhood is a rare variant of localiced scleroderma that appears typically in the pediatric age, it is characterized by the fast progression of deep cutaneous fibrosis that reaches fascia and muscle, determining the appearance of articular contractures in flexion and cutaneous ulceration. Although the features of systemic scleroderma are generally absent, this process affects severely the quality of life, the reason why the prognosis is bad. In some cases the inmunosupressive agents can delay the course of the disease. Recently cases of improvement of this disorder with phototerapia have been communicated. We described a new case of this entity.


REFERENCES

  1. Bielsa I. Esclerodermia. Colagenosis en la infancia.En: Dermatología Pediátrica de Fonseca E. Madrid.Aula Médica 2001: 1178-1200.

  2. Ferrándiz C, Henkes J, Gonzalez J, Peyrí J. Morfea panesclerótica de la infancia. Med Cutan Iber Lat Am 1981; 9: 377-82.

  3. Díaz-Pérez JL, Connoly SM, Winkelmann RK. Disabling pansclerotic morphea of children. Arch Dermatol 1980; 116: 169-73.

  4. Maragh SH, Davis MD, Bruce AJ, Nelson AM. Disabling pansclerotic morphoea: Clinical presentation in two adults. J Am Acad Dermatol 2005; 53: S115-20.

  5. Roudinesco, Vallery-Radot P. Sclérodermie mutilante progressive. Bull Soc Fr Dermatol Syphyol 1923; 30: 151-4.

  6. Falanga V. Fibrosing conditions in childhood. Adv Dermatol 1999; 6: 145-58.

  7. Debidayal, Singh S, Kumar L, Radotra BD. Disabling pansclerotic morphea of childhood and hypogammaglobulinemia: a curious association. Rheumatol Int 2002; 21: 158-60.

  8. Ochs HD, Winkelstein J. Disorders of the Bcell system. En: Stiehm (ed) Immunologic disorders in infants and children, 4th edn. Saunders. Philadelphia, 1996; 296-338.

  9. Bourgeois-Drouin C, Touriane R. Sclérodermie en plaques. Perturbations inmunologiques et viscerales. Ann Med Int 1978; 129:107-12.

  10. Hatamochi A, Ono M, Arakawa M. Análisis of collagen gene expresión by cultured fibroblasts in morphoea. Br J Dermatol 1992; 126: 216-21.

  11. Lacour JP, Vitetta A, Chiquet-Ehrismann R, Pisani A, Ortonne JP.Increased expression of tenascin in the dermis in scleroderma. Br J Dermatol 1992; 127: 328-34.

  12. Krieg T, Meuer M. Systemic scleroderma. Clinical and pathophysiologic aspects. J Am Acad Dermatol 1988; 18: 457-81.

  13. Krieg T, Braun-Falco O, Perlish JS, Fleischmayer R. Collagen synthesis in generalized morphea. Arch Dermatol Res 1983; 275: 393-6.

  14. Parodi PC, Riberti C, Draganic Stinco D, Patrone P, Stinco G. Squamous cell carcinoma arising in a patient with long-standing pansclerotic morphea [letter]. Br J Dermatol 2001; 144: 417-9.

  15. Bielefeld P. Sclerodermie sistemique and maladie malignant: revision de la literature. Rev Med Interne 1991; 12: 350-4.

  16. Winkelmann RK, Flach DB, Unni KK. Lung cancer and scleroderma. Arch Dermatol Res 1988; 280: S15-8.

  17. Wollina U, Buslau M, Weyers W. Squamous cell carcinoma in pansclerotic morphea of childhood. Pediatr Dermatol 2002; 19: 151-4.

  18. Wollina U. Looks A, Uhlemann C, Wollina K. Pansclerotic morphea of chilhood-follow-up over 6 years. Pediatr Dermatol 1999; 16: 245-47.

  19. Scharffetter-Kochanek K, Goldermann R, Lehmann P, Holzle E, Goerz G. PUVA therapy in disabling pansclerotic morphoea of children. Br J Dermatol 1995; 132: 830-1.

  20. Kerscher M, Volkenandt M, Meuer M, Lehmann P, Plewig G, Rocken M. Treatment of localised scleroderma with PUVA bath photochemotherapy. Lancet 1994; 343: 1233.

  21. Kerscher M, Dirschke T,Volkenandt M.Treatment of localised scleroderma by UVA1 phototherapy. Lancet 1995; 346: 1166.

  22. Gruss C, Stucker M, Kobyletzki G, Schreiber D, Altmeyer P, Kerscher M. Low dose UVA1 phototherapy in disabling pansclerotic morphoea of childhood. Br J Dermatol 1997; 136: 293-4.

  23. Scharffetter K, Wlaschek M, Hofg A, Bolsen K, Schothorst A, Goerz G, Krieg T, Plewig G. UVA radiation induces collagenasa in human termal fibroblasts in vitro and in vivo. Arch Dermatol Res 1991; 283: 506-11.

  24. Herrmann G, Wlaschek M, Lange TS, Prenzel K, Goerz G, Scharffetter-Kochanek K. UVA irradiatin stimulates the synthesis of various matrixmetalloproteinases (MMPs) in cultured human fibroblasts. Exp Dermatol 1993; 2: 92-7.

  25. Petersen MJ, Hansen C, Craigs S. Ultraviolet A irradiation stimulates collagenase production in cultured human fibroblasts. J Invest Dermatol 1992; 99: 440-4.

  26. Wlaschek M, Heinen G, Postwig A, Schwartz A, Krieg T, Scharffetter-Kochanek K. UVAinduced autocrine stimulation of fibroblastsderived collagenase/MMPs-1 by interrelated loops of interleukin-1 and interleukin- Photochem Photobiol 1994; 59: 550-6.

  27. Todd DJ, Askari A, Ektaish E. PUVA therapy for disabling pansclerotic morphoea of children. Br J Dermatol 1998; 138: 201-2.




2020     |     www.medigraphic.com