[LINEAR MYCOSIS FUNGOIDES IN A BLASCHKOID DISTRIBUTION].

INTRODUCTION: Mycosis fungoides (MF) is the most common type of primary cutaneous T cell lymphoma. Many clinicopathological variants of MF have been described in the literature, though only a few presented in a segmental pattern. There are several unique patterns of distribution of skin diseases, one of which is the Blaschko Lines. Congenital skin diseases develop in a Blaschkoid pattern due to mosaicism. In contrast, according to Happle, the development of acquired skin diseases in a similar pattern is explained by superimposed segmental manifestation - a process which involves mosaicism overlapping a preexisting congenital mutation. The theories by which previous case reports explained the segmental appearance of MF did not cover the molecular basis for their development. We report a case of a patient who presented with MF in a unique segmental distribution consistent with the Blaschko lines. The patient was found to have an acquired mosaic mutation in GNAS gene exclusively in the involved skin which represents a superimposed segmental manifestation according to Happle's theory. This case demonstrates the hidden potential of these rare cases which allows a better understanding of the pathogenesis by which acquired diseases develop. This is a basis for further research that could help identify new therapeutic targets for MF and other diseases that share its genetic etiology.

The Added Value of Patch Testing Beyond the Baseline Tray.

BACKGROUND: The patch test is the standard for diagnosing allergic contact dermatitis. Standardized trays allow the examination of the most prevalent allergens, whereas customized trays are more appropriate for addressing specific allergens and require expertise. They are therefore usually performed in specialized clinics. METHODS: We assessed the results of 4355 patch tests performed between 2012 and 2020 in a contact dermatitis clinic located in a large tertiary medical center. All patients were tested using the European baseline series and additional trays as clinically indicated. We assessed the frequency of relevant positive reactions outside the European baseline series. We then examined the added value and number of tests (NNTs) that need to be performed to elicit one relevant positive reaction per tray and common allergens. RESULTS: Nine hundred fifty-four patients (21.9%) had 1 or more positive relevant reactions; 43.3% tested positive for an allergen outside the European baseline series (OEBS). The acrylate and fragrance trays were highly represented among the positive and relevant reactions OEBS with NNTs of 4.4 and 6.8, respectively. 2-Hydroxyethyl methacrylate is the most prevalent allergen OEBS and is considered a marker for acrylate sensitivity with a high rate of cross-reactions and concordance rate of 85%, justifying its addition to the EBS in 2018. Other highly represented allergens include chloramphenicol, 2-hydroxyethyl acrylate, and Amerchol L-101, a lanolin derivative. The cosmetics and textile trays, although often tested, have relatively low added values of 3.7% and 2.3%, respectively. Surprisingly, the cutaneous adverse drug reaction series tray (CAD-1000) yielded no positive reactions, whereas testing the patients' medication yielded positive results in 10.9% of the cases. CONCLUSIONS: Expanded patch testing is crucial to accurately diagnose allergic contact dermatitis and almost doubles the number of patients with relevant positive reactions. Acrylate sensitivity is an emerging epidemic with a high positive reaction rate and low NNT, as is sensitivity to the allergens in the fragrance tray. 2-Hydroxyethyl methacrylate is a reliable marker for acrylate sensitivity with a concordance rate of 85%. Chloramphenicol is a common culprit and should be added to the standard tray in countries with a high usage rate. A low NNT was also observed when testing the patients' own cosmetics and medications; this should, therefore, be encouraged. The textile tray yielded a relatively high NNT; however, it should be performed when clinically indicated in the absence of a reliable marker in the EBS.

Patch testing versus interferon-gamma release assay in evaluation of drug eruptions.

Delayed-onset T-cell-mediated cutaneous adverse drug reactions are an uncommon but potentially serious result of medication exposures. Identification of culprit medications is crucial, but clinical diagnosis is often difficult. Patch tests and interferon-gamma release assays (IFNγ-RA) were previously reported as potentially useful ancillary tests, while rechallenges remain the reference standard test. We compared the number of positive test results with patch testing and IFNγ-RA for drugs implicated as possible causes of cutaneous reactions. Fifty-one patients with a suspected cutaneous drug eruption underwent patch testing and IFNγ-RA for suspected drugs. Participants were followed up at least 9 months after the onset of the rash with results compared with the clinical diagnosis. Forty-two patients presented with morbilliform/eczematous eruptions; five were diagnosed with fixed drug eruption (FDE) and four with erythema multiforme. None had positive patch testing to the drugs tested. A total of 8/51 (15.6%) patients had positive reaction by the IFNγ-RA, and an additional 11 (21.6%) patients had borderline results. Positive or borderline results were more likely in patients with FDE (80%) than morbilliform/eczematous eruptions (30.9%) or erythema multiforme (25%). Our study emphasizes the necessity of additional effective ancillary tests in the evaluation of drug eruptions and supports the use of IFNγ-RA for drug testing as a tool for identifying medications associated with cutaneous drug eruptions.