Localized scleroderma: assessment of the therapeutic response to phototherapy

Abstract

BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES -This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS -Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS -Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS -The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound. Keywords: PUVA therapy; Phototherapy; Scleroderma, localized; Ultrasonography; Ultrasound, high-intensity focused, transrectal Resumo: FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS: Este trabalho teve como objetivo avaliar o tratamento da esclerodermia cutânea com fototerapia. MÉTODOS: Foram selecionados pacientes com diagnóstico de esclerodermia cutânea para o tratamento com fototerapia, os quais foram classificados de acordo com o tipo clínico e o estágio evolutivo das lesões. Utilizou-se o exame clínico e a ultrassonografia da pele como metodologia para demonstrar os resultados obtidos com o tratamento proposto. RESULTADOS: Foi observado o início da melhora clínica das lesões com média de 10 sessões de fototerapia. A palpação clínica mostrou amolecimento em todas as lesões estudadas, com redução nos escores de avaliação estabelecidos. No exame de ultrassom, a maioria das lesões avaliadas mostrou diminuição da espessura da derme, e apenas cinco mantiveram sua medida. Não se observou diferença na resposta ao tratamento de acordo com o tipo de fototerapia instituída. CONCLUSÕES: O tratamento proposto foi efetivo em toda...

Post Trauma Morphea - A Brief Case Report

“…Many treatments has been explained and employed in the literature such as phototherapy and intralesional steroids (Buense et al 2012).…”

Phototherapy in Scleroderma

“…The mean thermography and joint passive range of motion both increased after treatmentUnknownMorita et al [75]Prospective uncontrolled study83Morphea (63), systemic sclerosis (15)UVA5, 10, and 20 J/cm 2 100, 200, 4006 + weeks20Clinical improvement. No difference between the groupsNoEl-Mofty et al [51]Prospective uncontrolled study12MorpheaUVA20 J/cm 2 3 times a week4006 + weeks2090% cure of early lesions, 50% cure of “late” lesionsNoEl-Mofty et al [12]Prospective uncontrolled study11SclerodermaOral + topical PUVA, Narrowband UVBUnavailableUnavailableUnavailableMean 10Most lesions had a decreased dermal thickness on ultrasound at 12 weeksUnknownBuense et al [56]Prospective uncontrolled study12Systemic sclerosisPUVA, bath or oralUnavailableMedian cumulative exposure 68.25UnavailableMedian 24Improvement in 11 patientsNoUsmani et al [29]Prospective uncontrolled study4Localized sclerodermaPUVA cream4 times a week. Maximum single dose of 3.5 J/cm 2 89.5 (range 67.5–121)Unavailable30Decrease in dermal thickeningNoGrundmann-Kollmann et al [76]Prospective uncontrolled study4Systemic sclerosisPUVA oral3 times a week for 10 weeksMean of 70.5 (...…”

“…Two months afterwards there was less induration of her plaque, decreased tenderness, and improved range of motion of the left arm [55]. Eleven patients that underwent phototherapy treatment (seven treated with PUVA and four treated with narrowband UVB) for an average of ten sessions experienced a 48% improvement of their localized scleroderma as indicated by a clinical pinching test [56]. Additionally, the ultrasound examination showed a dermal thickness reduction ranging from 20% to 100% [56].…”

“…Eleven patients that underwent phototherapy treatment (seven treated with PUVA and four treated with narrowband UVB) for an average of ten sessions experienced a 48% improvement of their localized scleroderma as indicated by a clinical pinching test [56]. Additionally, the ultrasound examination showed a dermal thickness reduction ranging from 20% to 100% [56]. There was no correlation between the type of phototherapy and clinical response rate [56].…”

Trauma Induced Localized Scleroderma

“…Many treatments has been explained and employed in the literature such as phototherapy and intralesional steroids [9] . The troublesome skin disease of morphea can happen months to years after the incident, and is usually accompanied with considerable morbidity and ache, and in addition the worrisome cosmetic aspects.…”