Superficial Morphea: Clinicopathological Characteristics and a Novel Therapeutic Outcome to Excimer Light Therapy

Abstract

Introduction. Superficial morphea (SM) is an uncommon entity that was described in the literature without definitive correlation to localized scleroderma (LS) or other atrophoderma diseases. Aim. To demonstrate the clinicopathological features of SM and evaluate the efficacy of different therapeutic modalities in its management. Patients and methods. A total of 28 patients with SM were studied during the period from 2010 to 2015. Clinicopathological features and therapeutic outcomes were recorded and analyzed. Results. Clinically, SM was predominant in females (71.4%) with an average onset at 33 years of age and an average duration of 15 months. It was commonly presented as asymptomatic, darkly pigmented, and multiple and slightly indurated patches. The lesions were mostly ill-defined, large-sized, and located more on the trunk. Histologically, thickening of collagen fibers was observed either localized to the papillary dermis only (38.9%) or extended into the upper reticular dermis (61.1%). Elastic fibers were generally diminished in the upper reticular dermis while the number of fibroblasts and basal melanin pigmentation were increased in the majority of cases (92.9% and 96.4%, respectively). The most commonly associated diseases were diabetes mellitus (50%) and hepatitis C virus (HCV) infection (42.8%), and their incidence was significantly higher than that in patients with LS. Excimer light showed promising effective results in the treatment of most cases (78.9%) while the response to other modalities such as topical corticosteroid alone or in combination with tacrolimus or treatment with UVA1 alone was less effective (7.1%, 23.1%, and 5%, respectively). Conclusion. Our results proposed that SM is a distinctive clinicopathological variant and not a stage in the spectrum of LS. The novel response of SM to excimer light and not for UVA1 therapy also suggests the different therapeutic outcome of SM from LS. Although SM has a significant association with DM and HCV infection, they seem not to affect the course of the disease.

Superficial morphea: A rare condition and report of three unique cases

“…The major defining characteristics of superficial morphea include bilateral atrophic plaques or patches in the intertriginous areas and/or trunk and histologically thickened collagen in the superficial dermis with presence of elastic fibers. 2 Differential diagnosis includes classic morphea, lichen sclerosus (LS), and idiopathic atrophoderma of Pasini and Pierini (IAPP). IAPP commonly coexists with morphea and is believed to be an abortive scleroderma in which sclerosis fails to form.…”

Morfea superficial

“…Las enfermedades sistémicas asociadas con mayor frecuencia a la morfea superficial son la diabetes mellitus (50%), la hepatitis C (42,8%) y la hipertensión arterial (21,4%), las cuales se descartaron en el caso comentado 2 . Como se describe en la bibliografía, el examen histopatológico es fundamental para el diagnóstico.…”

“…Se ha observado un aumento de estas fibras en las zonas en las que se evidencia su ausencia. También se supone que el incremento del factor de crecimiento transformante beta, producido como consecuencia de la respuesta inflamatoria ocasionada por la lesión microvascular, puede contribuir a la variación de las fibras colágenas, con el incremento de su producción y la disminución de su destrucción por las metaloproteinasas [2][3][4][5] .…”

“…Esta última se diferenciaría de la morfea superficial por tratarse de placas con un límite "en acantilado" y por afectar a los niños y los adolescentes. Además, en el estudio histológico presenta hialinización del colágeno dérmico, anormalidad en las fibras elásticas e inflamación activa en las lesiones en regresión 2,3,6,7 Por último, en el liquen escleroso y atrófico se destaca la presencia de lesiones epidérmicas además de los cambios dérmicos, la esclerosis de la dermis papilar y el infiltrado en banda en la unión dermoepidérmica 1,3 .…”

Small-sized shiny sclerotic plaques: Think early morphea

“…The early lesions of morphea clinically present as erythematous patches and plaques, while fully developed lesions are asymmetric sclerotic plaques. 2 Although morphea lesions are generally reported to be relatively large (>5 cm), 3 the clinical features of small-sized lesions (<5 cm) have been poorly discussed. In this study, we report two cases of small-sized superficial circumscribed morphea which probably represent the early lesion of the disease, and discuss the importance of early recognition of morphea.…”