Disabling pansclerotic morphea of childhood--unusual case and management challenges.
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.
Forsea
Ana-Maria
AM
Carol Davila University of Medicine and Pharmacy, Department of Oncologic Dermatology and Allergology, Elias University Emergency Hospital, Bucharest.
Cretu
Aura-Nicoleta
AN
Ionescu
Ruxandra
R
Giurcaneanu
Calin
C
D000203:Activities of Daily Living; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D003286:Contracture / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D006233:Persons with Disabilities; D018450:Disease Progression; D004359:Drug Therapy, Combination; D004547:Elastic Tissue / Q000473:pathology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010789:Phototherapy / Q000379:methods; D026741:Physical Therapy Modalities; D010840:Phytosterols / Q000627:therapeutic use; D011379:Prognosis; D012594:Scleroderma, Localized / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology* / Q000628:therapy*; D012720:Severity of Illness Index; D012867:Skin / Q000473:pathology*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use; D055815:Young Adult
D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D010840:Phytosterols; D014665:Vasodilator Agents