Xanthoma disseminatum (XD) is a rare, nonLangerhans cell histiocytosis characterized by multiple red to brown papules and nodules involving the skin and mucosal membranes.1,2 XD is very rare, with approximately 100 cases reported in the literature. 2-5The onset of XD ranges from as early as 8 months to as late as 85 years, 3 and is commonest in young adult men, with a male : female ratio of 2.4 : 1.4 XD appears to occur sporadically, with no known hereditary factors identified.2,4 XD is a disease with a chronic and progressive course. It presents a therapeutic challenge. We present a patient with the typical clinical and histological features of XD who was successfully treated with cladribine.A 19-year-old woman presented an 8-year history of an asymptomatic eruption, which had first appeared slowly on the anterior surface of the chest, and then progressed to involve the face, neck, arms and other flexural surfaces. Her medical history was otherwise unremarkable.Physical examination revealed yellow to red-brown coloured papules and nodules on the patient's face, neck and shoulder, the anterior and posterior surface of her chest, and the mucosal surfaces, along with verrucous tumours on the flexural surfaces of the inguinal area, popliteal fossae and axillary folds (Fig. 1a).Laboratory investigations, including full blood count, erythrocyte sedimentation rate, levels of C-reactive protein, urea and electrolytes, lipid profile, protein electrophoresis, urinalysis, 24-hour urine protein test, and chest radiography were all normal.Biopsies taken from the oral mucosa, anterior surface of the chest and inguinal region showed histopathological changes characteristic of XD (Fig. 2), thus the diagnosis of XD was made.We treated the patient with cladribine (2-chlorodeoxyadenosine) 0.14 mg/kg/day for 5 days each week, repeated every month. 4 After 7 months of treatment, many of the papular lesions had disappeared, leaving slightly hyperpigmented macules, but the rapid flattening process that took place in flexural areas resulted in an appearance of the loose skin (Fig. 1b). Cladribine therapy was well-tolerated, with few adverse effects. No new lesions developed during 2 years of follow-up.XD is characterized by multiple, asymptomatic, yellowish or red to brown papules, nodules and tumours. These often initially appear in flexural sites such as the axillae. The lesions tend to have a symmetrical
Aim: The primary objective of this study was to assess the ultrasonographic signs of subclinical enthesitis in patients with psoriasis. Secondary objective was to examine the associations between the clinical assessments of enthesitis, severity of psoriasis, and the ultrasonographic signs of enthesitis. Method: This study included 30 patients with psoriasis who did not have clinically detectable arthritis or enthesitis and 30 healthy volunteers as a control group. In the patient group, PASI, NAPSI, MASES, and SPARCC scores were calculated, and in the control group, MASES and SPARCC scores were calculated. Acute, chronic, and total enthesitis scores were calculated by ultrasonographic examination of the enthesis points that are assessed during calculation of SPARCC score, performed by a researcher blinded to the clinical assessments. Result: In the ultrasonographic assessment, total enthesitis score was significantly higher in the patient group compared with the control group (P = .04). There was no significant difference between the groups regarding acute or chronic enthesitis scores. NAPSI, PASI, MASES, or SPARCC scores did not show correlation with the ultrasonographically acute, chronic, or total enthesitis scores. There was a low-level correlation between MASES and SPARCC scores in the patient group, which was statistically significant (P = .03). No significant correlation was found between other clinical scores. There was no significant difference between patient and control groups in terms of MASES and SPARCC scores. Conclusion: Entheseal changes may be frequently observed in patients with psoriasis who are asymptomatic. Musculoskeletal ultrasonography (MUS) may be utilized to detect such abnormalities at the early period.
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