Search

Marina Talamonti,1 Filomena Russo,2 Giovanna Malara,3,4 Katharina Hansel,5 Manuela Papini,6 Angelo Cattaneo,7 Aurora Parodi,8 Andrea Chiricozzi,9,10 Piergiorgio Malagoli,11 Federico Bardazzi,12 Valeria Brazzelli,13 Paolo Dapavo,14 Paolo Gisondi,15 Cristina Zane,16 Concetta Potenza,17 Franca Cantoresi,18 Maria Concetta Fargnoli,19 Sara Trevisini,20 Pina Brianti,21 Leonardo Pescitelli,22 Giovanni Gigante,23 Marta Bartezaghi,23 Luisa Caputo,23 Elisabetta Aloisi,23 Antonio Costanzo24,25 On behalf of the SUPREME Study Group1Dermatology, University of Rome Tor Vergata, Rome, Italy; 2Department of Medical, Surgical and Neurological Science, Dermatology Section, University of Siena, S. Maria alle Scotte Hospital, Siena, Italy; 3Dermatology Unit, Hospital “Bianchi Melacrino Morelli”, Reggio, Calabria, Italy; 4Department of Dermatology, Papardo Hospital, Messina, Italy; 5Section of Dermatology, Department of Medicine and Surgery, University of Perugia, Perugia, Italy; 6Dermatologic Clinic of Terni, Department of Medicine and Surgery, University of Perugia, Perugia, Italy; 7U.O. Dermatologia, Fondazione IRCCS Ca’ Granda - Ospedale Maggiore Policlinico, Milano, Italy; 8Di.S.Sal. Section of Dermatology, Ospedale Policlinico San Martino, University of Genova, Genova GE, 16132, Italy; 9UOC di Dermatologia, Dipartimento di Scienze Mediche e Chirurgiche, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy; 10Dermatologia, Dipartimento di Medicina e Chirurgia Traslazionale, Università Cattolica del Sacro Cuore, Rome, Italy; 11Psocare Unit, I.R.C.C.S Policlinico San Donato, Milano, Italy; 12Dermatology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; 13Dermatology, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy; 14Department of Biomedical Science and Human Oncology, Second Dermatologic Clinic, University of Torino, Torino, Italy; 15Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy; 16Dermatology Department, ASST Spedali Civili di Brescia, University of Brescia, Brescia, Italy; 17Dermatology Unit “Daniele Innocenzi”, Department of Medico-Surgical Sciences and Biotechnologies, Faculty of Pharmacy and Medicine, Sapienza University of Rome – Polo Pontino, Latina, Italy; 18Dermatology Unit, Department of Medicine, University of Roma, Roma, Italy; 19Dermatology, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, L’Aquila, Italy; 20Department of Dermatology, University of Trieste, Trieste, Italy; 21Dermatology and Cosmetology Unit - San Raffaele Hospital, Milan, Italy; 22Department of Health Sciences, Dermatology Clinic, University of Firenze, Firenze, Italy; 23Novartis Farma SpA, Origgio, Italy; 24Unit of Dermatology, IRCCS Humanitas Research Hospital, Milano, Italy; 25Department of Biomedical Sciences, Humanitas University, Milano, ItalyCorrespondence: Marina Talamonti, Dermatology-Department of Systems Medicine, University of Rome Tor Vergata, PTV – Policlinico Tor Vergata, V.le Oxford 81, Rome, 00133, Italy, Tel +39 0620902743, Fax +39 0620902742, Email talamonti.marina@gmail.comPurpose: Secukinumab is a fully human monoclonal antibody that inhibits interleukin (IL)-17A approved for the treatment of moderate to severe plaque psoriasis in adults and children. We compared the efficacy and safety of secukinumab in patients aged < 65 years (adult patients) versus patients aged ≥ 65 years (elderly patients) in a post-hoc analysis of the SUPREME study.Patients and Methods: Patients with moderate to severe plaque psoriasis received subcutaneous secukinumab 300 mg per week for the first 5 weeks, then 300 mg per month. We compared the following outcomes in patients aged ≥ 65 years vs < 65 years: baseline characteristics; PASI50/75/90/100 response rates (improvements ≥ 50%/75%/90%/100% in Psoriasis Area and Severity Index (PASI) from baseline); changes in Dermatology Life Quality Index (DLQI); Hospital Anxiety and Depression Scale (HAD-A, HAD-D) score changes; treatment-emergent adverse events (TEAEs).Results: Secukinumab was slightly less effective in elderly patients than in adult patients (response rates at week 16: PASI90, 69.4% vs 80.9%, p = 0.4528; PASI100, 44.4% vs 56.7%, p = 0.8973). Elderly and adult patients showed a similar time course of changes in absolute PASI scores. Patients aged ≥ 65 years had a statistically significantly lower improvement in quality of life (mean DLQI reduction) than patients aged < 65 years at week 16 [− 5.4 (± 4.3) vs − 8.8 (± 6.9), p = 0.0065] and at week 24 [− 5.3 (± 4.4) vs − 9.2 (± 7.1), p = 0.0038]. Secukinumab treatment resulted in comparable mean reductions in anxiety and depression scores in both cohorts at 24 weeks [HAD-A, − 1.3 (± 3.3) vs − 2.1 (± 3.8), p = 0.9004; HAD-D, − 1.0 (± 3.3) vs − 1.5 (± 3.1), p = 0.4598]. The frequency of TEAEs in the two cohorts was similar (16.7% vs 14.6%, p = 0.7391).Conclusion: Secukinumab is a valid option for the management of moderate to severe psoriasis in elderly patients.Keywords: psoriasis, biologics, interleukin-17A, secukinumab, elderly

Luca Stingeni,1 Giovanna Malara,2,3 Andrea Conti,4 Luisa Di Costanzo,5 Carlo Giovanni Carrera,6 Martina Burlando,7 Piergiorgio Malagoli,8 Maria Letizia Musumeci,9 Federico Bardazzi,10 Valeria Brazzelli,11 Paolo Amerio,12 Clara De Simone,13,14 Sara Trevisini,15 Anna Balato,16 Matteo Megna,17 Francesco Loconsole,18 Catia De Felice,19 Marta Bartezaghi,20 Alice Rausa,20 Elisabetta Aloisi,20 Roberto Orsenigo,20 Antonio Costanzo21,22 On behalf of the SUPREME Study Group1Dermatology Section, Medical and Surgical Department, University of Perugia, Perugia, Italy; 2Dermatology Unit, Hospital “Bianchi Melacrino Morelli”, Reggio Calabria, Italy; 3Department of Dermatology, Papardo Hospital, Messina, Italy; 4Dermatologic Unit, Department of Surgery, Infermi Hospital, AUSL Romagna, Rimini, Italy; 5Department of Dermatology, “Gaetano Rummo” Hospital, Benevento UOC Dermatologia, AO G. Rummo, Benevento, Italy; 6U.O. Dermatologia, Fondazione IRCCS Ca’ Granda - Ospedale Maggiore Policlinico, Milano, Italy; 7IRCCS San Martino Polyclinic Hospital, Di.S.Sal. Section of Dermatology, Genoa, Italy; 8Dermatology Unit IRCCS Policlinico San Donato, Milan, Italy; 9Dermatology Clinic, University of Catania, Catania, Italy; 10Division of Dermatology, University Hospital Policlinico “S. Orsola-Malpighi”, Bologna, Italy; 11Institute of Dermatology, Foundation IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy; 12Dermatologic Clinic, G. D’Annunzio University, Chieti, Italy; 13Istituto di Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy; 14Fondazione Policlinico Universitario A. Gemelli – IRCCS, Rome, Italy; 15Dermatology Department, University of Trieste, Trieste, Italy; 16Dermatology Unit, University of Campania “Luigi Vanvitelli”, Naples, Italy; 17Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy; 18Department of Biomedical Sciences and Human Oncology, University of Bari, Bari, Italy; 19Department of Clinical Dermatology, Centre for the Study and Treatment of Psoriasis, San Gallicano Dermatological Institute, IRCCS, Rome, Italy; 20Novartis Farma SpA, Origgio, Italy; 21Unit of Dermatology, IRCCS Humanitas Research Hospital, Milan, Italy; 22Department of Biomedical Sciences, Humanitas University, Milan, ItalyCorrespondence: Luca Stingeni, Dermatology Section, Medical and Surgical Department, University of Perugia, Perugia, Italy, Tel +39075-5783881, Email luca.stingeni@unipg.itPurpose: Psoriasis, a common systemic inflammatory disorder, presents with gender-related differences in the quality of life (QoL) and treatment outcomes. This post hoc analysis from the Phase 3b SUPREME study explored gender-related differences in patient characteristics and efficacy of secukinumab 300 mg on Psoriasis Area and Severity Index (PASI) 75/90/100 and impact on QoL using the Dermatology Life Quality Index (DLQI) in patients with moderate to severe psoriasis through week 24.Patients and Methods: The proportion of patients achieving PASI 75/90/100 was computed using a nonresponder imputation approach. Differences between cohorts were analyzed using a logistic regression model. The mean change from baseline in DLQI was computed using the Wilcoxon test.Results: Among the 433 patients (males: 71.6%), females had a higher DLQI than males at baseline (13.1 vs 9.5; P< 0.0001). Males had a slightly higher response for PASI 90 than females at week 16 (80.7% vs 78.1%; P=0.0779) and 24 (83.2% vs 79.7%; P=0.0319). No differences were observed between genders in PASI 100/75 responses at week 24. Both genders showed an improvement in DLQI with secukinumab at week 24 (− 10.9 vs − 8.1, respectively, in females vs males; P=0.0004).Conclusion: In summary, secukinumab was effective in the treatment of psoriasis, irrespective of gender.Keywords: plaque psoriasis, PASI, patient-reported outcomes, Dermatology Quality of Life Index, Hospital Anxiety and Depression Scale

Adamantiades-Behçet disease (ABD) is a systemic disease with vasculitis, characterized by recurrent oral aphthosis and ocular, cutaneous, articular, vascular, cardiopulmonary manifestations and it is mainly found in the territories of the antique "silk road". ABD pathogenesis remains unknown although genetic, infectious and environmental factors seem to be implicated in the development of the disease, which is considered an auto-inflammatory condition. COVID-19 infection can present some symptoms, in particular at the level of oral and pulmonary mucosa, which require a differential diagnosis with ABD. Furthermore, the immunological alterations of this disease, and the drugs used for its treatment could influence the infection by COVID-19, and its clinical evolution. Nevertheless, vaccination anti-COVID-19 is recommended in ABD patients. The most commonly used diagnostic criteria for ABD are those established in 2014 by the International Team for the Revision of the International Criteria for BD (ITR-ICBD). Furthermore, criteria for disease severity according to the Overall Damage Index of Behçet's Syndrome (BODI) have recently been proposed in order to quantify the severity of the disease as well as the evolution during follow-up. In ABD patients it is mandatory to investigate on the presence of active/latent tuberculosis, because of the common organ involvement, such as eyes and bowel. ABD has a high morbidity and low mortality, sometimes linked to the rupture of an arterial aneurysm and/or neurological complications. This article is based on a general review on ABD ranging from the history of ABD to possible causes and clinical manifestations. A specific section has been dedicated to the COVID-19 pandemic.

Adamantiades-Behçet's disease (ABD) is a chronic, idiopathic, relapsing immune-mediate disease that may involve multiple organs. It is characterized by recurrent oral and genital ulcers, skin lesions, ocular, gastrointestinal, vascular, neurological and joint involvement. It can lead to significant morbidity and mortality. Due to its heterogeneity in clinical findings and physiopathology, its treatment can be various as ABD manifestations in different organs may differently respond to the same drug. The cornerstone of therapy for inducing remission is systemic corticosteroid, whereas immunomodulatory and immunosuppressive agents such as colchicine, azathioprine, cyclosporine-A, interferon-alpha, and cyclophosphamide are used as steroid-sparing agents and to prevent relapses. For aggressive, refractory or frequently relapsing cases, tumor necrosis factor (TNF) alpha inhibitors (infliximab, adalimumab, etanercept) have been reported beneficial. Herein, we describe our experience of 7 patients treated with TNF-alpha inhibitors with recommendations regarding treatment choice during the COVID-19 era.

Simple Summary Borreliae are spirochaetes, which represent a heterogeneous phylum within bacteria. Spirochaetes are indeed distinguished from other bacteria for their spiral shape, which also characterizes Borreliae. This review describes briefly the organization of the phylum Spirocheteales with a digression about its pathogenicity and historical information about bacteria isolation and characterization. Among spirochaetes, Borrelia genus is here divided into three groups, namely the Lyme group (LG), the Echidna-Reptile group (REPG) and the Relapsing Fever group (RFG). Borreliae Part 1 deals with Lyme group and Echidna-Reptile group Borreliae, while the subject of Borreliae Part 2 is Relapsing Fever group and unclassified Borreliae. Lyme group Borreliae is organized here in sections describing ecology, namely tick vectors and animal hosts, epidemiology, microbiology, and Borrelia genome organization and antigen characterization. Furthermore, the main clinical manifestations in Lyme borreliosis are also described. Although included in the Lyme group due to their particular clinical features, Borrelia causing Baggio Yoshinari syndrome and Borrelia mayonii are described in dedicated paragraphs. The Borrelia Echidna-Reptile group has been recently characterized including spirochaetes that apparently are not pathogenic to humans, but infect reptiles and amphibians. The paragraph dedicated to this group of Borreliae describes their vectors, hosts, geographical distribution and their characteristics. Abstract Borreliae are divided into three groups, namely the Lyme group (LG), the Echidna-Reptile group (REPG) and the Relapsing Fever group (RFG). Currently, only Borrelia of the Lyme and RF groups (not all) cause infection in humans. Borreliae of the Echidna-Reptile group represent a new monophyletic group of spirochaetes, which infect amphibians and reptiles. In addition to a general description of the phylum Spirochaetales, including a brief historical digression on spirochaetosis, in the present review Borreliae of Lyme and Echidna-Reptile groups are described, discussing the ecology with vectors and hosts as well as microbiological features and molecular characterization. Furthermore, differences between LG and RFG are discussed with respect to the clinical manifestations. In humans, LG Borreliae are organotropic and cause erythema migrans in the early phase of the disease, while RFG Borreliae give high spirochaetemia with fever, without the development of erythema migrans. With respect of LG Borreliae, recently Borrelia mayonii, with intermediate characteristics between LG and RFG, has been identified. As part of the LG, it gives erythema migrans but also high spirochaetemia with fever. Hard ticks are vectors for both LG and REPG groups, but in LG they are mostly Ixodes sp. ticks, while in REPG vectors do not belong to that genus.

Dear Editor, Grover's disease, also known as transient acantholytic dermatosis, is an idiopathic and acquired pruritic eruption of small vesicles and erythematous papules, classically on the central chest. The pathogenesis is not clearly defined, although heat sweating and occlusion have been interpreted as predisposing factors. We describe a case of monolateral acantholytic eruption with blaschkoid distribution in a patient treated with an orthopedic bandage for a shoulder injury. A 53-years-old man was referred to the dermatologic department because of an acute eruption of pruritic, discrete, pinkish grey papulovesicles, developed on the right side of the trunk 14 days after the positioning of an orthopedic bandage for a shoulder injury. This skin eruption followed a multilinear distribution along Blaschko lines (Figure 1, a, b). The medical history was unremarkable. The patient's family history was negative for similar dermatosis. Histopathological analysis showed acantholytic dyskeratosis with suprabasal cleft. A basket weave epidermic pattern with hyperkeratosis was observed, with a perivascular lymphocytic infiltration in the upper dermis (Figure 1, c, d). Given the clinical and histopathological features, a diagnosis of monolateral Grover's disease was established. The patient was treated with topical mometasone furoate 0.1% cream for 3 weeks with clinical improvement. Monolateral blaschkoid distribution in Grover's disease is extremely rare, with only 1 case reported in the literature (1). Two cases of zosteriform distribution have been described (2,3). The exact pathogenesis and the differential diagnosis with linear Darier's disease were the main topics of discussion, while the pathogenesis is still in debate. In our case, it appears that a shoulder bandage can cause an occlusive environment that can elicit the disease (4). Similar conditions were present in a previously described cases of bedridden patients (1). Some authors have postulated that such conditions may act as precipitating factors on a genetically predisposed epidermis (1). In monolateral and localized cases, postzygotic somatic mutations along Blasckho's lines or in dermatomes could be present. It is also important to distinguish this monolateral and blasckhoid Grover's disease from other acantholytic dermatoses. In this case, the differential diagnosis with an eruptive linear Darier's disease is very difficult (5). Histopathological analysis is not useful. Both monolateral and ordinary variants of Darier's disease can be elicited by environmental factors such as heat, sweating, or occlusion. The diagnosis of monolateral blaschkoid Grover's disease was preferred due to the patient's anamnesis (late-onset and no familiar history of similar dermatosis) and the clinical features (sparing of the head, extremities, and flexures). Since only additional genetic analysis could definitively resolve this question, it was performed. No mutation in genes coding the Ca2+ pump using genomic DNA from the patient's white blood cells or from a skin biopsy was found. A clinical anamnestic and genetic correlation is always crucial in these rare and unique acantholytic dermatoses.

Leukemia cutis (LC) is described as cutaneous infiltration by neoplastic leukocytes into the epidermidis, dermis, or subcutis, resulting in clinically various skin lesions. When the infiltrate is characterized by neoplastic granulocytic precursors, LC is defined as granulocytic sarcoma. Multiple, erythematous, and infiltrated papules and nodules localized on the legs, arms, and trunk are the most common clinical presentation. Here we report a case of granulocytic sarcoma in a patient with a previous diagnosis of acute myeloid leukemia currently in hematological remission.

In the present study, we describe the use of electrochemotherapy as alternative therapy for primary cutaneous marginal zone B-cell lymphomas in patients unsuitable for surgery or radiotherapy. Our experience refers to three patients with primary cutaneous marginal zone B-cell lymphomas related to Borrelia burgdorferi infection, treated with specific antimicrobial therapy and electrochemotherapy.

NONE

We report the occurrence of Staphylococcus lugdunensis cutaneous infection with sporotrichoid distribution of the left lower limb of a 60-year-old man. Recent studies have confirmed that Staphylococcus lugdunensis is a significant pathogen in causing skin and soft tissue infections that usually manifest in abscesses, surgical wound infections, and cellulitis. It used to be considered a skin commensal bacteria, but if unrecognized it can lead to fulminant endocarditis, meningitis, skin abscesses, peritonitis, and spondylodiscitis.

No abstract available

N/A

N/A

Background: Borrelia burgdorferi has been associated with primary cutaneous B-cell lymphoma, in particular with cutaneous marginal zone B cell lymphoma. Ixodes ticks, which are vectors of Borrelia burgdorferi, can also transmit many other microorganisms such as Anaplasma phagocytophilum and Rickettsia helvetica. Nowadays data on the frequency of these co-infections are still conflicting, but in our geographical area (north-eastern of Italy) they appear rather frequently. Aim: To investigate the presence of co-infections with Anaplasma phagocytophilum and Rickettsia helvetica, in patients with proven Borrelia burgdorferi-related cutaneous marginal zone B cell lymphoma. Methods: Four consecutive patients with a histopathological diagnosis of cutaneous marginal zone B cell lymphoma were investigated for the presence of Borrelia burgdorferi with conventional serology and with PCR, and for the presence of Anaplasma phagocytophilum and Rickettsia helvetica with conventional serology. Results: Serologic test for Borrelia burgdorferi was positive in 2 cases, while PCR assays for Borrelia burgdorferi performed on DNA from the biopsy and peripheral blood was positive in all four patients. Serologic analysis for Rickettsia helvetica was positive in one patient, while serologic analysis for Anaplasma phagocytophilum was positive in all patients. Conclusions: Our case series have revealed a co-infection between Borrelia burgdorferi and Anaplasma phagocytophilum in all patients with cutaneous marginal zone B cell lymphoma. Therefore more comprehensive analyses on the co-existence of Anaplasma phagocytophilum infection should be performed in patients with Borrelia burgdorferi-related cutaneous marginal zone B cell lymphoma.

The basal cell carcinoma (BCC) assessment in dermoscopy is based on the recognition of specific characteristics synthesized and described as classical and non-classical criteria, but which may not necessarily present all at the same time. Consequently, a deep knowledge in detecting the aforementioned dermoscopic criteria is crucial in diagnosis. The aim of the study was to evaluate which criteria are more frequently recognized among a group of low-experienced dermoscopists when confronted with the difficult diagnosis of BCC with a diameter lower than 5 mm. We examined 100 BCC finding that data displays a full agreement only for one classical criterion, the lack of pigmented network (Fleiss' κ = 1), while among other classical criteria only arborizing vessels and ulceration exhibit a good agreement among observers (Fleiss' κ > 0.40). Analyzing non-classical criteria, only blue-whitish veil and blue in-focus dots show a good agreement among low-experience observers (Fleiss' κ > 0.40). It is evident that in small size BCC classic dermoscopic criteria are often substituted by non-classical criteria, which represent the neoplasm's early phase. Thus, it is of importance, especially for low-experience dermoscopists, to analyze even the non-classical criteria in order to obtain a diagnosis of early BCC. © 2016 Japanese Dermatological Association

Chronic spontaneous urticaria (CSU) is perceived as a difficult to manage disease with negative impact on quality of life. The aim of this study was to highlight how to improve the care of people with CSU, using the methodology of narrative medicine. From June 2014 to March 2015, CSU-diagnosed patients and their physicians were asked to record their experiences of the condition in writing. Fourteen healthcare teams participated: 41% considered CSU as a challenge to overcome, while 22% experienced CSU as a big commitment. The number of professional involved was evaluated as insufficient in 11 hospitals. Seventy-five percent of the 190 Italian patients had visited 3 or more physicians before receiving a final diagnosis, with a perceived waste of time and resources. The therapeutic pathways were described as unsatisfactory in 83% of cases. As a result, anger and frustration were life-dominant emotions in 92% of patients. The critical points of the care pathway are related to organizational issues and lack of awareness.

Dermoscopy, in expert hands, increases accuracy, sensitivity and specificity in diagnosis of pigmented skin lesions of a single operator, compared with clinical examination. Simplified algorithmic methods have been developed to help less expert dermoscopists in diagnosis of melanocytic lesions. This study included 125 melanocytic skin lesions divided into melanocytic nevi, dysplastic nevi and thin melanomas (

NONE

We describe the case of an elderly woman who acquired a Mycobacterium marinum infection following skin exposure to the bacteria through a small wound on her right ring finger, obtained while preparing fish. The resultant sporotrichoid nodules of the right hand and the distal forearm, refractory to the initial therapy with doxycycline and rifampicin, were successfully treated with oral regimen of clarithromycin.

In the present study, we describe the use of electrochemotherapy as alternative therapy for primary cutaneous marginal zone B-cell lymphomas in patients unsuitable for surgery or radiotherapy. Our experience refers to three patients with primary cutaneous marginal zone B-cell lymphomas related to Borrelia burgdorferi infection, treated with specific antimicrobial therapy and electrochemotherapy.

Malignant melanoma presenting as a giant cutaneous mass is rarely observed in clinical practice. A few patients with giant melanoma have been reported, Herein, we document our experience with a patient with giant cutaneous melanoma of the abdomen and review the features of previously reported individuals.

Introduction : Nailfold videocapillaroscopy (NVC) is a non-invasive imaging technique widely used to investigate microvascular abnormalities in different connective tissue diseases (CTDs). Methods : We conducted a retrospective study where we analysed 415 patients submitted to NVC. Patients with scleroderma-like pattern were selected to investigate if there are specific NCV changes, which discriminate among the different CTDs. Ninety-one patients met this requirement and had a diagnosis of CTD. For each patient the following abnormalities were observed: enlarged and giant capillaries, oedema, loss and rarefaction of capillaries, long loops and minor dystrophies. Results : Multivariate analyses did not reveal any specific modification among the analysed co-variables for scleroderma (SS) and dermatomyositis (DM). For the others CTDs analysed in this study, logistic regression revealed that some of the capilloroscopic features could be indicative of specific diseases. Of note, the presence of megacapillaries with long loops in a scleroderma-like pattern seems to be highly indicative for a diagnosis of systemic lupus erythaematosus (SLE). Conclusions : Our data showed that in CTDs with a scleroderma-like pattern, the NVC variables alone are not able to discriminate for a specific diagnosis of CTD. Nevertheless, there are some NVC features, which could strongly address the differential diagnosis toward a specific CTD.