Subungual onycholemmal cyst of the toenail mimicking subungual melanoma.

This report highlights a rare case of a woman with horizontal ridging and tenderness of the right great toenail associated with dyspigmentation of 5 years' duration. Histopathology revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus-catagen cyst admixed with the presence of both an intermittent, focal granular layer and an eosinophilic cuticle surrounding pink, laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC). It is a rare and distinctive nail abnormality occurring in the dermis of the nail bed. We present a case of an SOC in the toenail mimicking subungual malignant melanoma, which may be an underrecognized and common entity that must be considered when discussing tumors of the nail unit, especially subungual melanoma.

A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study.

OBJECTIVES: In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. METHOD: We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. RESULTS: 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p<0.001), a higher frequency of digital ulcers (OR: 1.28 (1.11 to 1.47); p<0.001) and pulmonary hypertension (OR: 3.01 (1.47 to 6.20); p<0.003). In the longitudinal analysis (n=4499), after a mean follow-up of 4.9 (±2.7) years, male sex was predictive of new onset of pulmonary hypertension (HR: 2.66 (1.32 to 5.36); p=0.006) and heart failure (HR: 2.22 (1.06 to 4.63); p=0.035). 908 deaths were recorded, male sex predicted deaths of all origins (HR: 1.48 (1.19 to 1.84); p<0.001), but did not significantly account for SSc-related deaths. CONCLUSIONS: Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decision-making process.

Acute retinal artery occlusion in systemic sclerosis: a rare manifestation of systemic sclerosis fibroproliferative vasculopathy.

OBJECTIVES: To describe three patients with systemic sclerosis (SSc) who developed acute unilateral blindness in the absence of any common etiologic factor for blindness. In one patient, the affected eye required enucleation and was examined histopathologically. METHODS: Following identification of the first patient with retinal artery occlusion at the Scleroderma Center of Thomas Jefferson University, every patient evaluated at the Center from May 2001 to December 2010 was prospectively assessed for the development of acute unilateral blindness. Two additional cases were identified. Here, we describe the clinical features, laboratory and ancillary examinations of the three patients with SSc who developed acute unilateral blindness and present the histopathological examination of one eye enucleated from one of the patients. RESULTS: Clinical and angiographic studies were consistent with acute retinal artery occlusion. The histopathological studies showed severe retinal ischemic atrophy and concentric narrowing and fibrosis of small retinal vessels. CONCLUSIONS: These findings suggest that acute retinal artery occlusion in these patients is a manifestation of the fibroproliferative vasculopathy characteristic of SSc.

Assessment of tissue fibrosis in skin biopsies from patients with systemic sclerosis employing confocal laser scanning microscopy: an objective outcome measure for clinical trials?

OBJECTIVES: To obtain an objective, unbiased assessment of skin fibrosis in patients with SSc for use in clinical trials of SSc disease-modifying therapeutics. METHODS: Skin biopsies from the dorsal forearm of six patients with diffuse SSc and six healthy controls, and skin biopsies from the forearm of one patient with diffuse SSc before and following 1 year treatment with mycophenolate mofetil were analysed by confocal laser scanning microscopy (CLSM) with specific antibodies against collagen types I and III or fibronectin. The integrated density of fluorescence (IDF) was calculated employing National Institutes of Health-ImageJ software in at least four different fields per biopsy spanning the full dermal thickness. RESULTS: The intensities of collagen types I and III and fibronectin IDF were 174, 147 and 139% higher in SSc skin than in normal skin, respectively. All differences were statistically significant. The sum of the IDF values obtained for the three proteins yielded a comprehensive fibrosis score. The average fibrosis score for the six SSc samples was 28.3 x 10(6) compared with 18.6 x 10(6) for the six normal skin samples (P < 0.0001). Comparison of skin biopsies obtained from the same SSc patient before treatment and after 12 months of treatment with mycophenolate mofetil showed a reduction of 39% in total fibrosis score after treatment. CONCLUSIONS: CLSM followed by quantitative image analysis provides an objective and unbiased assessment of skin fibrosis in SSc and could be a useful end-point for clinical trials with disease-modifying agents to monitor the response or progression of the disease.