Scleroderma (systemic sclerosis) is a connective tissue disorder which involves skin, gastrointestinal tract, kidneys and lungs with considerable morbidity. Scleroderma renal crisis is a well-known severe form of renal involvement, characterised by the presence of high blood pressure with variable degrees of renal insufficiency. We report a case of scleroderma renal crisis diagnosed on the basi...

General examination of the eye was carried out in 22 patients with systemic lupus erythematosus (SLE) and in 10 with scleroderma. 3 of the SLE and 2 of the scleroderma patients had keratoconjunctivitis sicca. Fluorescein angiography showed abnormalities of the retinal vasculature in one of a subgroup of 12 SLE patients and one of 10 scleroderma patients. None of the 12 SLE patients had abnormal...

Exaggerated acute and late toxicities following radiotherapy have been reported in patients with pre-existing connective tissue diseases, such as systemic lupus scleroderma. Behcet’s disease (BD) is a relapsing multisystem characterized by vasculitis the mucocutaneous, ocular, gastrointestinal, respiratory, neurologic, urogenital, articular, cardiovascular systems. Data concerning relationship ...

INTRODUCTION Treprostinil diethanolamine is an innovative salt form of the prostacyclin analogue, treprostinil sodium, developed as an oral sustained release (SR) osmotic tablet. The availability of a formulation permitting convenient systemic delivery might have applicability to scleroderma vascular complications. We evaluated pharmacokinetics and perfusion in scleroderma patients with digital...

It currently is believed that scleroderma is a complex polygenic disease that occurs in genetically predisposed individuals who have encountered specific environment exposures and/or other stochastic factors. The nature of these genetic determinants and how they interact with environmental factors are areas of active investigation. This article discusses the evidence that supports a strong gene...

In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy), who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea) and myositis has not been described.

Capillary microscopy was performed on 19 patients with eosinophilic fasciitis. These patients were compared with 13 individuals with progressive systemic sclerosis (scleroderma). Capillary patterns were normal in 16 of 19 (84%) eosinophilic fasciitis patients; 3 exhibited either borderline or nonspecific changes, and none showed a definite scleroderma pattern. In contrast, characteristic nailfo...

Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global...