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BackgroundEuropean guidelines propose a 0 center dot 5 mg kg(-1) per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients.MethodsIn a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0 center dot 5 mg kg(-1) per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0 center dot 1 mg kg(-1) per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score.ResultsIn total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80 center dot 9 (SD 9 center dot 1) years. Control of disease activity was achieved at day 21 in 119 patients [62 center dot 6%, 95% confidence interval (CI) 55 center dot 3-69.5]; 18 of 24 patients (75%, 95% CI 53 center dot 3-90 center dot 2), 75 of 110 patients (68 center dot 8%, 95% CI 59 center dot 2-77 center dot 3) and 26 of 56 patients (46.4%, 95% CI 33 center dot 0-60 center dot 3) had mild, moderate and severe BP, respectively (P = 0 center dot 0218). A total of 30 patients died during the study. The overall Kaplan-Meier 1-year survival was 82 center dot 6% (95% CI 76 center dot 3-87 center dot 4) corresponding to 90 center dot 9%, 83 center dot 0% and 80 center dot 0% rates in patients with mild, moderate and severe BP, respectively (P = 0 center dot 5). Thresholds of 49 points for BPDAI score and 70 points for Karnofsky score yielded maximal Youden index values with respect to disease control at day 21 and 1-year survival, respectively.ConclusionsA 0 center dot 5 mg kg(-1) per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.

Background Subungual squamous cell carcinoma (SU-SCC) is the most common malignant tumour of the nail unit. Intraoperative nail dermoscopy has been described only for pigmented tumours, onychomatricoma and glomus tumours. Aim To establish a description of intraoperative dermoscopic features of SU-SCC. Methods A single-centre retrospective cohort of 53 SU-SCC cases over a 5-year period was reviewed by six examiners who individually scored 31 intraoperative dermoscopic features as present or absent. For each feature, the frequency and interobserver agreement was evaluated, then the data were compared and a consensus was reached. Results No feature had perfect or substantial interobserver agreement. Regarding anatomy and architecture, most tumours involved both the nail bed and nail matrix (n = 34, 64.2%) and had nonparallel lateral side edges (n = 36, 67.9%). Regarding vascular features, several different patterns were found: dotted vessels (n = 49, 92.5%), irregular vessels (n = 47, 88.7%), curved vessels (n = 46, 86.8%), sagittal vessels (n = 45, 84.9%), milky-red areas (n = 42, 79.2%), linear and regular vessels (n = 30, 56.6%), coiled and hairpin vessels (n = 23, 43.4%), and arborizing vessels (n = 16, 30.2%). Pigmented dermoscopy structures included dotted purpura, grey granulation and splinter haemorrhages, which were found in 49 (20.8%), 9 (17%) and 9 (17%) cases, respectively. Other dermoscopic signs were pink background, translucent structureless area, whitish scaly areas, distal plug, yellowish scales and dots, and 'digitiform' proximal edge, which were found in 49 (84.9%), 49 (84.9%), 43 (81.1%), 37 (69.8%), 28 (52.8%) and 22 (41.5%) cases, respectively. Conclusion Analysis of this first large series of SU-SCC studied by intraoperative dermoscopy suggests that it gives useful information to better approach the diagnosis and to target biopsies.

We report the case of a 60-year-old woman who underwent 18F-FDG PET/CT to evaluate a metastatic breast carcinoma. Follow-up 18F-FDG PET/CT showed progressive disease with 18F-FDG increased in primary tumor, axillary lymph nodes, and pleural and bone diffuse metastases but also a concomitant uptake in multiples joints. The anatomopathological analysis from skin biopsy revealed a multicentric reticulohistiocytosis, considered paraneoplastic in the context. Second follow-up PET/CT after treatment showed a decrease of 18F-FDG uptake in previously affected joints, consistent with the symptoms evolution. 18F-FDG PET/CT could be helpful in the detection and the evaluation of such rare systemic disorder.

Introduction Les patients ayant une maladie bulleuse auto-immune (MBAI) etant a risque d’infection (âge, traitement corticoide et/ou immunosuppresseur), nous avons evalue le risque de la COVID-19 et de ses formes severes chez les patients ayant une MBAI. Materiel et methodes Cette etude retrospective multicentrique (49 services de dermatologie) a inclus tous les cas connus de COVID survenus entre janvier et juin 2020 en France metropolitaine. Les cas ont ete classes en certains (PCR + ), probables (PCR− ou non faite, TDM pulmonaire evocateur) ou possibles (PCR et TDM negatifs ou non faits mais signes cliniques et/ou cas contacts). L’incidence cumulee des cas de COVID a ete calculee a partir des cas certains et hospitalises de patients MBAI suivis dans chaque service, puis comparee a celle dans la population generale sur la meme periode (donnees Sante Publique France) apres standardisation indirecte sur l’âge et la region. L’incidence et le pronostic ont ete analyses pour l’ensemble des MBAI et pour le sous-groupe ayant recu du rituximab (RTX) dans les 9 derniers mois. Resultats Incidence : 59 cas de COVID-19 ont ete recenses (âge : 73,1 ± 16,2 ans) parmi 5180 patients suivis pour une MBAI : 29 (49 %) correspondaient a des cas certains, 8 (14 %) probables et 22 (37 %) possibles. Les MBAI etaient une PB (n = 21, 36 %), une P muqueuse (PM) (PC/EBA, n = 19, 32 %), un pemphigus (n = 18, 31 %) et 1 P gestationnelle (n = 1, 2 %). Le ratio d’incidence standardise (RIS) etait de 0,42 [IC95 % : 0,20–0,80] p = 0,005 pour les PB, 1,02 [0,37–2,26], p = 0,91 pour les pemphigus et de 1,18 [0,55–2,23], p = 0,62 pour les PM. Parmi les 516 patients ayant recu du RTX, on comptait 22 cas possibles probables ou certains d’infection COVID correspondant a un RI = 5,37 [3,15–8,96], parmi lesquels 13 cas probables ou certains (RI = 4,90 [2,43–9,40]) par rapport aux patients n’ayant pas recu de RTX, et un RI de formes certaines et hospitalisees de 3,62 [1,29–8,85]. Pronostic 30 cas (51 %) ont ete hospitalises pour COVID et 15 (25 %) sont decedes (RR = 1,63 [0,83–2,55] p = 0,13 par rapport a la population generale ajustee a l’âge et a la region. Un patient MBAI avait 3,4 [2,2–5,1] fois plus de risque de deceder sur la periode epidemique s’il contractait la COVID que s’il ne la contractait pas. L’âge moyen des patients ayant une forme severe (hospitalisation ou deces) de COVID etait plus eleve que celui de ceux ayant une forme non severe (77,9 ± 11,2 vs 65,0 ± 19,3, p = 0,006). Les RR de forme severe des PM/pemphigus avec COVID probable ou certaine traites par RTX (âge moyen 68,8 ± 14,9) etaient respectivement de 0,77 [0,45–1,33] p = 0,33 et de 0,51 [0,08–2,56] p = 0,40 par rapport aux PM/pemphigus avec COVID non traites par RTX (âge moyen 76,7 ± 9,1). Discussion La COVID entraine une forte surmortalite chez les patients MBAI. L’incidence semble particulierement elevee parmi les patients ayant recu du RTX, sans que l’on puisse conclure a une plus grande severite de la COVID-19 chez ces patients.

BACKGROUND: Several dermoscopic and in vivo reflectance confocal microscopy (RCM) diagnostic criteria of lentigo maligna (LM)/lentigo maligna melanoma (LMM) have been identified. However, no study compared the diagnostic accuracy of these techniques. OBJECTIVE: We evaluated the diagnostic accuracy of dermoscopy and RCM for LM/LMM using a holistic assessment of the images. METHODS: A total of 223 facial lesions were evaluated by 21 experts. Diagnostic accuracy of the clinical, dermoscopic and RCM examination was compared. Interinvestigator variability and confidence level in the diagnosis were also evaluated. RESULTS: Overall diagnostic accuracy of the two imaging techniques was good (area under the curve of the sROC function: 0.89). RCM was more sensitive (80%, vs. 61%) and less specific (81% vs. 92%) than dermoscopy for LM/LMM. In particular, RCM showed a higher sensitivity for hypomelanotic and recurrent LM/LMM. RCM had a higher interinvestigator agreement and a higher confidence level in the diagnosis than dermoscopy. CONCLUSION: Reflectance confocal microscopy and dermoscopy are both useful techniques for the diagnosis of facial lesions and in particular LM/LMM. RCM is particularly suitable for the identification of hypomelanotic and recurrent LM/LMM.

Summary Background New primary melanomas arising in patients with stage IV melanoma and receiving BRAF inhibitors have recently been reported. This raises the question of the nature of the earliest cellular events identifiable within pre-existing moles. Objectives To use reflectance confocal microscopy (RCM) to investigate changing moles in patients using vemurafenib. Methods In the first part of the study 23 lesions were examined by RCM before excision (performed because of digital dermoscopy changes) and histopathological examination. In the second part, 10 randomly chosen lesions in two patients were examined before and after 3 months of vemurafenib treatment. Results The first step permitted the highlighting of an unusual RCM pattern identified in five lesions characterized by areas of marked atypia in otherwise nondysplastic lesions. In the second step, four initially nondysplastic lesions developed focal or multifocal areas of marked atypia under treatment, which were not always correlated with digital dermoscopy changes, but did correlate with histopathology. All four lesions were finally diagnosed as melanomas. Conclusions Although the clinical relevance of such findings remains questionable, RCM allowed us to observe, at the cellular level, the earliest events occurring within vemurafenib-induced changing moles. Moreover, repeated RCM examinations permitted to confirm that microscopic marked atypia that led to the histopathological diagnosis of melanoma appeared under treatment and were not pre-existing.

in situ or minimally invasive melanoma can be treated by nonamputative wide excision of the nail unit followed by a skin graft, performed by a dermatologic surgeon, with an excellent cosmetic and functional result that does not impair the prognosis.1-2 We previously published a preliminary study showing a good correlation between perioperative Reflectance Confocal Microscopy (RCM) and histopathology of the nail matrix in case of subungual melanoma (SUM)3. Since then, we used perioperative in vivo RCM in our centre, after informed consent of patient, during surgical exploration of 30 consecutive cases of suspicious MS between 1/11/2012 and 31/03/2016. This study was approved by the institutional review board of our centre (n°0180) and followed the ethical principles of the Declaration of Helsinki. This article is protected by copyright. All rights reserved.

SummaryBackground Amelanotic nail tumours are difficult to diagnose. Dermoscopy is an accessible tool successfully used in diagnosis of amelanotic or melanotic skin tumours. We have previously shown the usefulness of dermoscopy in the preoperative diagnosis of onychomatricoma (OM). In this study, we completed this work by identifying additional intraoperative criteria to better establish the initial diagnosis of this tumour. Aims Evaluation of intraoperative dermoscopy in a small series of OM cases in order to define relevant diagnostic criteria. Methods In total, 10 patients with OM diagnosed in our centre were enrolled in the study. Six trained dermoscopists individually evaluated each criterion, then the data were compared and a consensus reached after discussion between the observers. For each criterion, we analysed its frequency and its interobserver accordance. We defined three architectural criteria (the ‘Sagrada Familia’ sign, digitations and the ‘mirror sign’), and three vascular criteria (sagittal vessels, dotted vessels and irregular vessels). Results The Sagrada Familia sign, digitations and mirror sign were found in 100%, 90% and 70% of the cases, respectively, with high interobserver agreement. The vascular criteria were less regularly observed: sagittal, dotted and irregular vessels were respectively found in 80%, 70% and 50% of the OM cases, and were more difficult to assess, as shown by the lower interobserver agreement rates. Conclusion Intraoperative dermoscopy of the nail matrix and bed offers useful information for the diagnosis and management of OM. Larger comparative studies should be performed to evaluate the true benefit of this approach.

Summary Background Dermoscopy is now recognized as an essential tool for discriminating melanoma from other pigmented lesions, as corroborated by several robust meta-analyses. Although it is considered to be widely used in European countries, no published data on this topic are available to date, unlike in Australia and the U.S.A. Objectives To describe and quantify the use and learning of dermoscopy among French private practice dermatologists. Methods A questionnaire of 19 items regarding demographic characteristics, dermoscopy use and training, and physician’s judgment on dermoscopy was mailed to all French private practice dermatologists. Only questionnaires with an answer to the key item, ‘Do you use dermoscopy?’ were taken into account. Results Of 3179 mailed questionnaires, 1611 were returned and 1576 were analysable (49·6%). Most respondents declared using dermoscopy (94·6%), using their dermoscope several times a day (82·7%) and/or for the diagnosis of nonpigmented lesions (87·7%). Physicians learned dermoscopy mainly through books (75·8%) and/or conferences (88·6%); 12·8% reported a dedicated university degree. Dermoscopy helps to detect melanoma earlier and to perform fewer biopsies according to 86·6% and 74·6%, respectively. On multivariate analysis, female sex and age under 45 years were significantly associated with higher utilization rate of dermoscopy [odds ratio 1·89, 95% confidence interval (CI) 1·15–3·10; and 2·85, 95% CI 1·14–7·11, respectively]. Conclusions This is the first published nationwide survey of dermoscopy practice in Europe. Despite potential classification and/or selection bias, the particularly high penetration rate found in our study suggests that dermoscopy is now widely accepted by French private practice dermatologists for the routine management of both pigmented and nonpigmented lesions.

Summary Background Although dermoscopy of the nail plate is helpful to discriminate between benign and malignant causes of nail pigmentations, there remain ambiguous cases in which a matricial biopsy is required. When a subungual melanoma is diagnosed histopathologically, a complementary surgical treatment is performed secondarily, the duration of postoperative disability being accordingly prolongated. Objectives The purpose of our study was to evaluate the feasibility of an intraoperative diagnosis by reflectance confocal microscopy (RCM). Patients and methods Our series included nine consecutive patients who underwent a matricial biopsy for an acquired melanonychia (one benign lentigo and eight melanomas). RCM examination was performed in vivo on the nail matrix after reclination of the nail plate, and/or ex vivo on the fresh tissue biopsy. RCM data were compared with histopathology. Results There was a good correlation between confocal and histopathological features. Seven melanoma cases were unequivocally diagnosed intraoperatively according to the confocal features, whereas the lentigo was correctly classified as a benign lesion according to RCM. The remaining lesion could not be unequivocally classified by RCM and corresponded histopathologically to an early melanoma that required immunostaining to be diagnosed. Conclusions Intraoperative RCM examination of the nail matrix is an efficient diagnostic approach of melanonychia striata that permits an extemporaneous diagnosis of malignancy and therefore a one-step surgical treatment of in situ or minimally invasive melanoma, reducing dramatically the duration of postoperative disability.

Summary Background Lentigo maligna melanoma (LMM) is the most common subtype of melanoma on the face. Its presentation may be quite subtle, particularly in early stages, and delayed diagnosis is common. Few dermoscopic studies have been performed and the main dermoscopic features of LMM were defined by Stolz and coworkers in 2000. Objectives To investigate classical as well as new dermoscopic features in a large series of LMM in a white-skinned population, in order to evaluate their diagnostic value. Methods One hundred and twenty-five consecutive histopathology-proven LMMs were analysed retrospectively based on medical records, clinical and dermoscopic photographs by three independent observers for the presence of 19 predefined criteria. Results At least one of the classical Stolz criteria was present in 87% of cases (hyperpigmented follicular opening, annular-granular pattern, pigmented rhomboidal structures, obliterated hair follicles). Three original criteria were also present at a relatively high frequency: increased density of the vascular network (58%), red rhomboidal structures (40%), target-like patterns (41%). Darkening at dermoscopic examination (when compared with naked-eye examination) was observed in 25% of lesions. Classical dermoscopic features of extrafacial melanoma (atypical pigment network, irregularly distributed globules, dots, streaks and pseudopods) and vertical growth phase-associated dermoscopic criteria (ulceration, blue papular areas and black structureless areas) were rarely seen. A large number of colours, pigmented rhomboidal structures, obliterated hair follicles and red rhomboidal structures were significantly more frequent in invasive LMMs. In contrast, in situ melanomas were more often associated with one or two colours and few distinctive dermoscopic features. Conclusions We present herein, in a large series of LMM, confirmation of the diagnostic value of the classical Stolz dermoscopic criteria and describe four additional original criteria, mainly vascular. A correlation between the presence of some dermoscopic features and thicker tumoral invasion has also been demonstrated.

Background The ‘dermoscopic island’ is a term that was recently proposed to design an area of a pigmented lesion with a uniform dermoscopic pattern different from the remainder of the lesion. The positive predictive value of this sign for the diagnosis of melanoma is about 50%. Objective The purpose of our study was to see if reflectance confocal microscopy (RCM) permitted to accurately distinguish between nevi and melanoma in such lesions. Methods Five lesions of five consecutive unselected patients, with a dermoscopic island but no feasible clear cut diagnosis on the basis of dermoscopy alone were examined by RCM before excision for histopathological evaluation. Results Two lesions corresponded to nevi, and three lesions were early melanomas arising on a benign naevus in one case, and on a dysplastic naevus in two cases. In all five cases, RCM permitted to make the correct diagnosis, with a very good correlation with conventional histopathology. Conclusion Reflectance confocal microscopy appears as a promising tool not only to enhance the early diagnosis of melanoma but also to avoid unnecessary excisions of lesions with a dermoscopic island.

Background: The naevoid basal cell carcinoma syndrome (NBCCS) is a therapeutic challenge due to the multiplicity of cutaneous tumours. Photodynamic therapy (PDT) is increasingly used as an alternative treatment for superficial and in some countries nodular basal cell carcinomas (BCC). Objective: To study the safety and efficiency of PDT in NBCCS. Methods: We reviewed retrospectively the evolution of 62 lesions from patients with multiple BCC treated with PDT. Results: The initial response rate (85.4%, 53/62) and recurrence rate (7.5%) appeared comparable to literature values in NBCCS and to those reported in the treatment of sporadic BCC. The clearance rate without recurrence was 79% (49/62), during a mean follow-up period of 13 months. The cosmetic outcome was excellent. Recurrences were found almost 2 years after treatment. Conclusion: PDT is a suitable therapeutic option in the management of NBCCS patients but requires a strict and long follow-up.

Resume Introduction Le syndrome d’hypersensibilite medicamenteuse est une reaction medicamenteuse grave potentiellement letale. Au cours de ces dernieres annees, l’hypothese d’un lien entre la survenue de ce syndrome et une reactivation virale HHV6 a ete soulevee. Observation Une femme âgee de 45 ans, traitee par sulfasalazine (Salazopyrine®) pour un rhumatisme inflammatoire, a presente un mois apres le debut du traitement un syndrome d’hypersensibilite medicamenteuse, complique d’une hepatite toxique severe. Le profil serologique HHV6 et la PCR sur serum etaient en faveur d’une primo-infection HHV6. Discussion Nous rapportons la deuxieme observation d’un syndrome d’hypersensibilite medicamenteuse associe a une seroconversion HHV6. L’hypothese d’un desordre immunitaire favorisant la replication virale pourrait expliquer cette association. Des serologies HHV6 systematiques au cours de ce syndrome pourraient permettre de confirmer ou non cette relation et, le cas echeant, de modifier la prise en charge therapeutique.

Gonorrhea is a common sexually transmitted infection, which can present as the ‘arthritisdermatitis syndrome’. Patients with systemic lupus erythematosus often develop disseminated neisserial infections, because of inherited and acquired complement deficiencies. Neisserial infection, and particularly gonococcemia, can mimic a lupus flare. We report one case of gonococcemia presenting as acral papulo-vesiculous lesions of the digits in a young woman with lupus. Lupus (2014) 23, 81–83.

Summary Background Desmoplastic melanoma (DM) is a rare variant of cutaneous melanoma. Its diagnosis is often delayed by an unusual clinical presentation. The dermoscopic features of DM have not yet been described. Objective To define the dermoscopic features of DM. Patients and methods A single-institution register-based retrospective study of six cases of histology-proven desmoplastic melanoma for which dermoscopy data were available. The criteria we studied included: network, dots and globules, streaks, regression features, ulceration, number of colours, blue/white veil, and vascular pattern. Results Only three cases exhibited one classical feature for a melanocytic lesion; other cases were recognized on the basis of the presence of figures of regression (all six), i.e. white scar-like areas and ‘peppering’ (three of six), multiple (> 4) colours (five of six), and of melanoma-related vascular patterns (five) such as linear-irregular vessels (four) and milky-red areas (two). Discussion We believe that dermoscopy could help in the accurate diagnosis of this rare neoplasm. In the absence of a pigmented network, attention should be given to the identification of features of regression and to melanoma-associated vascular patterns.

Summary Background Sentinel lymph node (SLN) positivity has been found to be strongly associated with a poor prognosis in melanoma. Objectives This large referral centre study was conducted: (i) to confirm the powerful prognostic value of SLN biopsy (SLNB); (ii) to correlate patient prognosis to the micromorphometric features of SLN metastasis in SLN-positive patients; and (iii) to correlate these micromorphometric features to the likelihood of positive completion lymph node dissection (CLND). Patients and methods SLNB was performed in 455 cases of primary melanoma between January 1999 and December 2004; for patients with positive SLN, the following micromorphometric features were registered: size of the largest metastasis (two diameters), depth of metastasis, number of millimetric slices involved, maximum number of metastases on a single section, presence of intracapsular lymphatic invasion and extracapsular spread. Kaplan–Meier survival curves were compared with the log-rank test; multivariate analysis was performed using a Cox regression model. Dependence of CLND status on micromorphometric features of SLN was assessed by the χ2 test and predictive values of the different features were evaluated by multivariate analysis using a logistic regression model. Results A positive SLN was identified in 98 of our 455 cases. Survival was significantly shorter in SLN-positive patients than in SLN-negative patients. Extracapsular invasion was found to be an independent prognostic factor of disease-free survival; ulceration of the primary and the maximum diameter of the largest metastasis were identified as independent predictive factors of disease-specific survival. Age and the lowest diameter of the largest metastasis were identified as independent predictive criteria of positive CLND, whereas depth of metastasis was not. Positivity of CLND was not significantly associated with a worse prognosis. Conclusions Our study confirms the previously demonstrated strong prognostic value of SLNB. It also confirms the relationship between tumour burden in the SLN (evaluated by the maximum diameter of the largest metastasis) and clinical outcome. We point out a new micromorphometric feature of SLN, which seems to be predictive of CLND status: the lowest diameter of the largest metastasis.

Lyme serology was positive for both IgG and IgM (ELISA, Enzygnost Borreliosis , Siemens, Dade Behring, Germany, and blot, Euroline WB , Euroimmun, Germany). A punch biopsy of a papule showed a dermal perifollicular ( fig. 2 ) – and occasionnally perineural – infiltrate of lymphocytes and plasma cells, consistent with a borrelial infection. Borrelia burgdorferi DNA was amplified from fresh tissue obtained from a skin biopsy performed on a peripilar papule, using a specific real-time PCR according to Makinen et al. [1] (culture not performed). The erythema resolved after a 3-week doxycycline treatment whereas arthralgia and dysesthesia persisted. Erythema chronicum migrans (ECM) is usually the earliest manifestation of Lyme borreliosis. It occurs a few days, sometimes a few weeks, after the tick bite. Lyme disease is caused by an infection with the spirochete B. burgdorferi, which is transmitted by ixodid ticks. ECM usually begins as a macule or papule at the site of the tick bite and evolves as a centrifugally growing annular erythematous plaque with central clearing. It is usually asymptomatic, but localized pruritus, pain, hyperesthesia or dysesthesia have occasionally been reported [2] . It spontaneously disappears within several weeks, occasionally several months; antibiotherapy shortens the evolution and prevents secondary dissemination which can cause neurological, rheumatological, cardiac, ophthalmological and late cutaneous complications [3] . Posttreatment assessment is clinical [4] . Numerous atypical presentations have been described for ECM: mini erythema migrans [5] , erythema with no central clearing, erythema with central induration [6] , vesicles [7] , ulceration, necrosis or purpura [1] , alternating bands of erythema, confluent red or red-blue lesion without central clearing, and even triangular, rectangular or linear lesion [6, 8] .