Localized Scleroderma: Clinical Spectrum and Therapeutic Update

Overview

Journal An Bras Dermatol

Specialty Dermatology

Date 2015 Feb 13

PMID 25672301

Citations 90

Authors

Mariana Figueiroa Careta

Ricardo Romiti

Affiliations

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Abstract

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

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References

Buense R, Duarte I, Bouer M . Localized scleroderma: assessment of the therapeutic response to phototherapy. An Bras Dermatol. 2012; 87(1):63-9. DOI: 10.1590/s0365-05962012000100007. View

De P, Lloyd H, Rashid A, Anstey A . Morphoea presenting shortly after the onset of Schmidt's syndrome. Clin Exp Dermatol. 2000; 25(2):168-9. DOI: 10.1046/j.1365-2230.2000.00602.x. View

Rai R, Handa S, Gupta S, Kumar B . Bilateral en coup de sabre-a rare entity. Pediatr Dermatol. 2000; 17(3):222-4. DOI: 10.1046/j.1525-1470.2000.01757.x. View

Higashi Y, Kanekura T, Fukumaru K, Kanzaki T . Scleroderma en coup de sabre with central nervous system involvement. J Dermatol. 2000; 27(7):486-8. View

Inigo F, Arroyo O, Fernandez M, Ysunza A . Restoration of facial contour in Romberg's disease and hemifacial microsomia: experience with 118 cases. Microsurgery. 2000; 20(4):167-72. DOI: 10.1002/1098-2752(2000)20:4<167::aid-micr4>3.0.co;2-d. View

Komocsi A, Tovari E, KOVACS J, Czirjak L . Physical injury as a provoking factor in three patients with scleroderma. Clin Exp Rheumatol. 2000; 18(5):622-4. View

Hulshof M, Bouwes Bavinck J, Bergman W, Masclee A, Heickendorff L, Breedveld F . Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. 2000; 43(6):1017-23. DOI: 10.1067/mjd.2000.108369. View

Stone J, Franks A, Guthrie J, Johnson M . Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry. 2001; 70(3):382-5. PMC: 1737241. DOI: 10.1136/jnnp.70.3.382. View

Jappe U, Holzle E, Ring J . [Parry-Romberg syndrome. Summary and new knowledge based on an unusual case]. Hautarzt. 1996; 47(8):599-603. DOI: 10.1007/s001050050475. View

10.

Blaszczyk M, Janniger C, Jablonska S . Childhood scleroderma and its peculiarities. Cutis. 1996; 58(2):141-4, 148-52. View

11.

Kerscher M, Meurer M, Sander C, Volkenandt M, Lehmann P, Plewig G . PUVA bath photochemotherapy for localized scleroderma. Evaluation of 17 consecutive patients. Arch Dermatol. 1996; 132(11):1280-2. View

12.

DeFelipe J, Segura T, Arellano J, Merchan A, DeFelipe-Oroquieta J, MARTIN P . Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. Epilepsia. 2001; 42(9):1198-203. DOI: 10.1046/j.1528-1157.2001.45800.x. View

13.

Nadeau S . Neurologic manifestations of connective tissue disease. Neurol Clin. 2002; 20(1):151-78, vi. DOI: 10.1016/s0733-8619(03)00057-4. View

14.

Lee H, Kim M, Ha S, Kim J . Two cases of morphea associated with Hashimoto's thyroiditis. Acta Derm Venereol. 2002; 82(1):58-9. DOI: 10.1080/000155502753600920. View

15.

Park J, Lee C . Concurrent development of dermatomyositis and morphoea profunda. Clin Exp Dermatol. 2002; 27(4):324-7. DOI: 10.1046/j.1365-2230.2002.10493.x. View

16.

Orozco-Covarrubias L, Ridaura-Sanz C, Carrasco Daza D, Ruiz-Maldonado R . Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them?. J Eur Acad Dermatol Venereol. 2002; 16(4):361-6. DOI: 10.1046/j.1468-3083.2002.00442.x. View

17.

Grosso S, Fioravanti A, Biasi G, Conversano E, Marcolongo R, Morgese G . Linear scleroderma associated with progressive brain atrophy. Brain Dev. 2003; 25(1):57-61. DOI: 10.1016/s0387-7604(02)00147-x. View

18.

Marzano A, Menni S, Parodi A, Borghi A, Fuligni A, Fabbri P . Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol. 2003; 13(2):171-6. View

19.

Unterberger I, Trinka E, Engelhardt K, Muigg A, Eller P, Wagner M . Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids. J Neurol Neurosurg Psychiatry. 2003; 74(5):661-4. PMC: 1738439. DOI: 10.1136/jnnp.74.5.661. View

20.

Feghali-Bostwick C, Medsger Jr T, Wright T . Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies. Arthritis Rheum. 2003; 48(7):1956-63. DOI: 10.1002/art.11173. View