Oral NSM is rare and may represent a diagnostic challenge for pathologists. To confirm the diagnosis of NSM, the evaluation of S-100 protein expression or other neural marker is essential. The use of the terms NSM and neurothekeoma as synonymous or as variants of the same tumor should be avoided, because they are clearly distinct lesions.

A paired comparison of the efficacy of a topically applied fluoridated corticosteroid, 0.2% fluocinolone acetonide in a cream base (Synalar-HP®, Syntex) and the base alone was carried out in 28 patients with alopecia areata and alopecia totalis. A satisfactory to excellent therapeutic response was obtained in 17/28 patients (61%), with the corticosteroid cream treatment. The patient’s age and the duration of the alopecia appeared to be important factors in the outcome. Although relapses were encountered in 11/17 patients (63%) during the course of treatment or within 3 months after treatment was discontinued, 12/17 (71%) of those who responded did retain sufficient regrowth for the result to be considered satisfactory. The only troublesome complication was the development of a localized acneform eruption in 5/28 patients (18%) between the ages of 15 and 25 years. None of the patients developed clinical evidence of systemic absorption.

BackgroundSkin neurofibromas represent one of the main clinical manifestations of neurofibromatosis 1, and their number varies greatly between individuals. Quantifying their number is an important step in the methodology of many clinical studies, but counting neurofibromas one by one in individuals with thousands of tumors is arduous, time-consuming, and subject to intra and interexaminer variability. We aimed to evaluate the efficacy of a new methodology for skin neurofibromas quantification using paper frames.MethodsThe sample comprised 92 individuals with NF1. Paper frames, with a central square measuring 100 cm2, were placed on the back, abdomen and thigh. Images were taken, transferred to a computer and two independent examiners counted the neurofibromas. The average number of neurofibromas/100 cm2 of skin was obtained from the mean of the three values. The differences in the quantity of neurofibromas counted by two examiners were evaluated with Intraclass correlation coefficient (ICC), paired t-test, Bland-Altman and survival-agreement plots. To evaluate the predictive value of the method in obtaining the total number of neurofibromas, 49 participants also had their tumors counted one by one. Reproducibility was assessed with Pearson’s correlation coefficients and simple linear regression model.ResultsThere was excellent agreement between examiners (ICC range 0.992-0.997) and the total number of skin neurofibromas could be predicted by the adhesive frames technique using a specific formula (P < 0.0001).ConclusionsIn this article we describe a reliable, easy and rapid technique using paper frames to quantify skin neurofibromas that accurately predicts the total number of these tumors in patients with NF1. This method may be a useful tool in clinical practice and clinical research to help achieve an accurate quantitative phenotype of NF1.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-014-0202-9) contains supplementary material, which is available to authorized users.

The β2-microglobulin amyloidosis is a newly recognized type of systemic amyloidosis, which occurs in patients with chronic renal failure undergoing long-term hemodialysis. The dialysis-related amyloidosis (DRA) of the tongue is an uncommon and late complication, which occurs in patients undergoing chronic hemodialysis for more than 20 years. The aim of this paper is to report two cases of lingual amyloidosis associated with long-term hemodialysis. Two patients were referred to the Oral Medicine Clinic due to the presence of multiple nodules on the tongue and macroglossia. The patients had history of chronic renal failure and had been dialysed for more 20 years with bio-incompatible cellulose membrane. Biopsies of the lesions were performed and the histopathological features and Congo red stain revealed oral amyloidosis. Both patients remain in clinical follow-up after 12 and 6 months, respectively. Recognition of the amyloidosis tongue lesions can minimize the complications caused by this condition such as obstruction of the upper airways, making it imperative to carry out early treatment. Furthermore, in patients with oral amyloidosis, lesions in areas of trauma may ulcerate and cause pain and dysphagia becoming necessary periodic surgical removal and regular follow-up of these patients.

No abstract