Juvenile localized scleroderma (JLS) includes several subtypes including plaque morphea, linear scleroderma and the en coup de sabre type which affects face and head. The latter variety may involve the eye and the brain with various appearance and clinical complications.We describe the case of a 6-year-old boy who presented partial complex seizures, with status epilepticus, four months before t...

Skin blood flow was measured by the clearance of radioactive xenon ((133)Xe) injected intracutaneously in eight patients with scleroderma and nine control subjects under conditions of controlled temperature and humidity. Scleroderma patients, on being cooled 1 hr at 18 degrees C, had a rate constant of (133)Xe clearance from the dorsal finger skin which was 0.04 +/-0.07 min(-1) (mean +/-SD), co...

Diffuse panbronchiolitis (DPB) is a distinct clinicopathologic entity, which is characterized by chronic recurrent sinopulmonary infection and inflammation. We describe 3 patients with rheumatoid arthritis (RA) associated with DPB and consider that DPB is one of the bronchopulmonary manifestations associated with RA.

Renal manifestations occur frequently in scleroderma (SSc). Commonest is a reduction in renal function due to chronic disease but most clinically important is the scleroderma renal crisis (SRC). This life-threatening complication occurs in up to 15% of the cases of dcSSc. Mortality is reduced by use of angiotensin converting enzyme (ACE) inhibitors. Renal outcome can be assessed by quantifying ...

ER: extended release HDACi: Histone deacetylase inhibitor TSA: trichostatin A TGF-b: Transforming growth factor beta INTRODUCTION Scleroderma is an autoimmune connective tissue disease that involves the skin and internal organs for which there are few reliably effective treatments. The cutaneous manifestations of scleroderma include fibrosis and sclerodactyly, calcinosis cutis, digital ulcers, ...

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 ...

Introduction Although there is no universally agreed classification of scleroderma, the following is most generally accepted (Rowell, 1977): (i) morphoea (localized or generalized); (ii) progressive systemic sclerosis; (iii) pseudo scleroderma; (iv) occupational scleroderma. Progressive systemic sclerosis usually begins in middle age, but there is a wide range in the age of onset. Rodnan (1963)...

Linear atrophoderma of Moulin (LAM) is an acquired skin condition that manifests in early childhood and adolescence. It likely represents a form of cutaneous mosaicism that presents with linear, hyperpigmented and atrophic lesions appearing on the trunk and limbs. Its clinical appearance varies and may closely resemble that of atrophoderma of Pasini and Pierini (APP) and linear scleroderma. LAM...