Forsea Ana-Maria, Cretu Aura-Nicoleta, Ionescu Ruxandra, Giurcaneanu Calin
Carol Davila University of Medicine and Pharmacy, Department of Oncologic Dermatology and Allergology, Elias University Emergency Hospital, Bucharest.
J Med Life. 2008 Jul-Sep;1(3):348-54.
Morphea, also known as localized scleroderma is a chronic disease of unknown etiology, characterized by fibrous deposition and obliteration of vessels in the skin. This disease has a wide clinical spectrum, ranging from mild hyperpigmented plaques to severe, invalidating generalized and pansclerotic forms. Disabling pansclerotic morphea of childhood is a rare and debilitating variant of localized scleroderma, characterized by a rapid progression of deep cutaneous fibrosis that involves the dermis and the subcutaneous adipose tissue but also fascia, muscles, and bone. Contractures and musculoskeletal atrophy develop and the disease has an invalidating and even fatal course. We present an unusual case of severe morphea in a 19-year-old girl, with a polymorphous clinical picture consisting of plaques, linear and pansclerotic, circumferential lesions, with symmetric, invalidating involvement of all limbs and explosive evolution with centripetal progression. This case emphasizes the unpredictable character of morphea evolution, the possible severe prognosis and the therapeutic challenges raised by the generalized, disabling forms of this disease.
硬斑病，也称为局限性硬皮病，是一种病因不明的慢性疾病，其特征是皮肤中纤维沉积和血管闭塞。这种疾病临床谱广泛，从轻度色素沉着斑到严重的、导致残疾的全身性和泛硬化型。儿童致残性泛硬化性硬斑病是局限性硬皮病的一种罕见且使人衰弱的变体，其特征是深部皮肤纤维化迅速进展，累及真皮、皮下脂肪组织，还包括筋膜、肌肉和骨骼。挛缩和肌肉骨骼萎缩出现，疾病呈致残甚至致命病程。我们报告一例19岁女孩的严重硬斑病罕见病例，其临床表现多样，包括斑块、线状和泛硬化性、环形病变，四肢均对称受累并致残，呈向心性进展的爆发性演变。该病例强调了硬斑病演变的不可预测性、可能的严重预后以及这种疾病全身性、致残性形式所带来的治疗挑战。
