A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting “stuck” with upward gaze. Maddox rod test revealed a subtle intermittent right hypotropia and right enophthalmos (figure 1). Asymptomatic T2 hyperintensities were noted on MRI (figure 2). Scalp morphea was confirmed pathologically. Formal rheumatologic evaluation w...

Generalized morphea is a disease characterized by wide-spread sclerosis of the skin. A 39-year-old man presented with history of multiple pigmented and bound-down plaques on the body along with mucosal involvement. Dermatological examination showed multiple indurated and sclerosed plaques with follicular plugging in few of them and gross thickened eroded and glazed tongue. The constellation of ...

Morphea and systemic sclerosis are fibrosing disorders of the skin that share common inflammatory and immunologic pathways that are responsible for the vascular changes, increased collagen production, and extracellular matrix proliferation seen in both conditions. Recent advances in molecular biology techniques have furthered our knowledge of the potential underlying pathogenic mechanisms and o...

Juvenile Localized Scleroderma (JLS), is characterised by presence of areas of skin thickening, which is relatively benign, but if deeper tissues such as muscle and bone are involved severe deformities may develop. Mycophenolate Mofetil (MMF) is increasingly utilised both for treatment of systemic sclerosis and immune-mediated skin diseases such as psoriasis, graft-versus-host and lichen planus...

OBJECTIVES To evaluate the esophageal involvement in patients with juvenile localized scleroderma (JLS). METHODS A cohort of patients with JLS underwent esophageal stationary manometry to evaluate esophageal motility and lower esophageal sphincter (LES) function, distal esophagus 24-hour pH-monitoring to detect gastroesophageal reflux (GER) and upper gastrointestinal (GI) endoscopy to evaluat...

Annular lichenoid dermatitis of youth (ALDY) is a more recently described inflammatory disease of the skin of unknown etiology with clinical similarities to morphea. The authors clinically, histopathologically, and immunohistochemically investigated 14 biopsies from 12 patients in western Austria with this disease. There were 6 female and 6 male patients with solitary (n = 7) and multiple lesio...

BACKGROUND There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). Monitoring is done almost exclusively by clinical assessment. Our aim was to determine whether serum concentrations of TGFbeta1 are a good biomarker of disease activity in children with LS. METHODS 55 pediatric patients with LS were divided into sub-types accordin...