2019, Number 2
Next >>
Acta Pediatr Mex 2019; 40 (2)
Morphea in children: a retrospective study of its clinical characteristics and extracutaneous manifestations
Rodríguez-Salgado P, Hernández-Zepeda C, Saez-de Ocariz M, Yamazaki- Nakashimada MA, García-Romero MT
Language: English
References: 22
Page: 51-58
PDF size: 444.61 Kb.
ABSTRACT
Background: Morphea is an inflammatory disease with diffuse thickening and hardening
of the skin. It is common in children and it has been reported that as many as 40%
have extracutaneous articular and neurological manifestations, vascular and ocular
disorders, and gastrointestinal or respiratory symptoms.
Rationale: There are no studies of associated extracutaneous manifestations in Mexican
children with morphea.
Objective: Describe clinical characteristics, frequency of extracutaneous manifestations,
and treatment of pediatric patients with morphea who received care at the Instituto
Nacional de Pediatría.
Material and Methods: A retrospective study of patients with morphea, 1 to 18
years of age, who received care between January 1, 2000, and December 31, 2015.
Results: Thirty-two patients with morphea were included, half (n = 16) with the linear
subtype, followed by plaque-type morphea in 7 (22%). Extracutaneous manifestations
were reported in 23 (72%) patients; the most common were: musculoskeletal in 23
(72%), neurological in 9 (28%), and ophthalmological in 3 (9%). Twenty-three patients
(72%) required systemic treatment. Nineteen patients (60%) required orthopedic and
surgical treatment for complications. The clinical evolution was toward remission in
72% of the patients (n = 23).
Conclusions: In this series of 32 cases of children with morphea, 72% had extracutaneous
manifestations, principally musculoskeletal and neurological. It is important to
conduct targeted questioning, and if necessary perform paraclinical studies, to identify
extracutaneous manifestations which may be associated with morphea, especially when
it is linear or extensive.
REFERENCES
Yu BD, Eisen AZ. Scleroderma. In: Freedberg IM, Eisen AZ, Wolff K, et al. ed. Fitzpatrick’s Dermatology in general medicine, vol II. 6ta edición. Nueva York, EEUU: McGraw- Hill, 2003: 1709-1717.
Röcken M, Ghoreschi K. Morphea and Lichen Sclerosus. In: Bolognia J, Jorizzo J, Rapini R. ed. Dermatology. 2a ed. España: Mosby Elsevier; 2008: 1469-1476.
Weibel L, Harper J. Morphea (Localized Scleroderma). In: Irvine A, Hoeger PH, Yan AC. Ed. Harper's textbook of pediatric dermatology. 3ra ed. NJ: Wiley-Blackwell. 2011: 1651-58.
Careta M, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. Anais Brasileiros de Dermatologia. 2015;90(1): 62-73.
García de la Peña, P. Esclerodermia localizada. Protoc diagn ter pediatr. 2014;1:10: 1-6.
Behman R, Kliegman R, Jenson H, Agud Aparicio J. ed. Nelson, tratado de pediatría. 2ª ed. Madrid: McGraw-Hill, 2002: 765-766.
Garcia-Romero MT, Laxer R, Pope E. Localized Scleroderma: Symptoms, Diagnosis and Treatment. In: De Winter, R. ed. Scleroderma. Symptoms, Diagnosis and Treatment. NY: Nova Science Publishers, Inc, 2013: 87-103.
Arenas R. Dermatología. Atlas, diagnóstico y tratamiento. 4ª ed. México: McGraw-Hill; 2015, pag 169-174.
Alfaro Cordero, K. Morfea: El desequilibrio entre la producción y la destrucción del colágeno. Revista médica de Costa Rica y Centroamérica LXXIII. 2016; 619: 351-354.
Bielsa Marsol, I. Update on the Classification and Treatment of Localized Scleroderma. Actas Dermo-Sifiliográficas (English Edition). 2013;104(8): 654-666.
Warner Dharamsi J, Victor S, Aguwa N, et al. Morphea in Adults and Children Cohort III. JAMA Dermatology. 2013; 149(10): 1159.
Christen-Zaech S, Hakin MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): Review of 136 patients. J Am Acad. Dermatol. 2008; 59:385-96.
Noguera M, Sauro de Carvalho M, Lombardi V. Morfea. Arch. Argent. Dermatol. 2012;62: 77-84.
Atzeni F, Bardoni A, Cutolo M et al. Localized and systemic forms of scleroderma in adults and children. Clin Exp Rheumatol. 2006; 24 (suppl.40):S36-45
Zulian F, Vallongo C, Woo P, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005; 52: 2873-81
Pequet MS, Holland KE, Zhao S, et al. Risk factors for morphoea disease severity: A retrospective review of 114 pediatric patients. Br J Dermatol. 2014; 170(4): 895-900.
Chiu YV, Vora S, Kwon EK, Maheshwari M. A significant proportion of children with morphoea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol. 2012; 29(6):738-48.
Uziel Y, Feldman BM, Krafchik BR, Yeung RS, Laxer RM. Methotrexate and corticosteroids therapy for pediatric localized scleroderma. J Pediatr. 2000; 136: 91-95.
Weibel L, Sampaio MC, Visentin MT, et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol. 2006; 155(5):1013-20.
Kreuter A, Altmeyer P, Gambichler T. Treatment of localized scleroderma depends on the clinical subtype. British Journal of Dermatology. 2007; 156(6): 1363-1364.
Bielsa Marsol, I. Update on the Classification and Treatment of Localized Scleroderma. Actas Dermo-Sifiliográficas (English Edition). 2013; 104(8), 654-666.
Martini G, Campus S, Bernd R, et al. Tocilizumab in two children with pansclerotic morphoea: a hopeful therapy for refractory cases? Clin Exp Rheumatol. 2017; 35 Suppl 106(4):211-213. Epub 2017 Sep 29. PMID: 28980909