Adamantiades-Behçet's disease therapy: current treatment options and recommendations with regard 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.

The surgical treatment of non-metastatic melanoma in a Clinical National Melanoma Registry Study Group (CNMR): a retrospective cohort quality improvement study to reduce the morbidity rates.

BACKGROUND: Reproducible, high-quality surgery is a key point in the management of cancer patients. Quality indicators for surgical treatment of melanoma has been presented with benchmarks but data on morbidity are still limited. This study presents the quality indicators on morbidity after surgical treatment for non-metastatic skin melanoma in an Italian registry. METHODS: Data were extracted from the Central National Melanoma Registry (CNMR) promoted by the Italian Melanoma Intergroup (IMI). All surgical procedures (WE, SNLB or LFND) for non-metastatic skin melanoma between January 2011 and February 2017 were evaluated for inclusion in the study. Only centers with adequate completeness of information (> 80%) were included in the study. Short-term complications (wound infection, dehiscence, skin graft failure and seroma) were investigated. RESULTS: Wound infection rate was 1.1% (0.4 to 2.7%) in WE, 1.3% (0.7 to 2.5%) in SLNB and 4.1% (2.1 to 8.0%) in LFND. Wound dehiscence rate was 2.0% (0.8 to 5.1%) in WE, 0.9% (0.2 to 3.0%) in SLNB and 2.8% (0.9 to 8.6%) in LFND. Seroma rate was 4.2% (1.5 to 11.1%) in SLNB and 15.1% (4.6 to 39.9%) in LFND. Unreliable information was found on skin graft failure. CONCLUSIONS: Our findings contribute to available literature in setting up the recommended standards for melanoma centers, thus improving the quality of surgery offered to patients. A consensus on the core issues around surgical morbidity is needed to provide practical guidance on morbidity prevention and management.

Deep sedation for laser treatment of cutaneous vascular anomalies in children.

Pulse laser treatment is useful for aesthetic improvement in children with capillary vascular malformation and residual superficial haemangiomas. However, pulse laser treatment is painful, and repeated procedures are usually required to achieve a satisfactory clinical result. Evidence shows that analgesia in children undergoing pulse laser treatment may vary from general anaesthesia to no treatment at all. This report describes our institutional experience with deep sedation in children with capillary vascular malformation, undergoing laser pulse treatment.

Cerebrospinal Fluid Cytokine Expression Profile in Multiple Sclerosis and Chronic Inflammatory Demyelinating Polyneuropathy.

BACKGROUND: Cerebrospinal fluid (CSF) analysis in patients with particular neurologic disorders is a powerful tool to evaluate specific central nervous system inflammatory markers for diagnostic needs, because CSF represents the specific immune micro-environment to the central nervous system. METHODS: CSF samples from 49 patients with multiple sclerosis (MS), chronic inflammatory demyelinating polyneuropathy (CIDP), and non-inflammatory neurologic disorders (NIND) as controls were submitted to protein expression profiles of 47 inflammatory biomarkers by multiplex Luminex bead assay to investigate possible differences in the inflammatory process for MS and CIDP. RESULTS: Our results showed differences in CSF cytokine levels in MS and CIDP; in particular, IL12 (p40) was significantly highly expressed in MS in comparison with CIDP and NIND, while SDF-1α and SCGF-ß were significantly highly expressed in CIDP cohort when compared to MS and NIND. IL-9, IL-13, and IL-17 had higher expression levels in NIND if compared with the other groups. CONCLUSIONS: Our study showed that, despite some common pathogenic mechanisms, central and peripheral nervous system demyelinating diseases, such as MS and CIDP, differ in some specific inflammatory soluble proteins in CSF, underlining differences in the immune response involved in those autoimmune diseases.

Epidemiology of pyoderma gangrenosum: Results from an Italian prospective multicentre study.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterised by painful, necrotic ulcerations. PG is described as a rare disease: the world-wide incidence is estimated to be around 3 to 10 cases per million population per year. These estimations are based mostly on case reports and retrospective case series; there are no prospective, multicentre studies on the matter. The apparent rarity of PG is in contrast with our clinical perception as dermatologists: in our opinion, PG is not so uncommon. Therefore, we decide to investigate the epidemiology of PG in the Italian population and confirm our clinical suspicions that it is not an orphan disease. We enrolled all patients diagnosed with PG in 8 Italian Dermatological Departments from 1st October 2014 to 1st November 2015, and we recorded their features. Our data, collected from 64 patients, are in accordance with those of the published literature regarding the epidemiology and features of PG. In an Italian population of roughly 8 million inhabitants of 7 provinces, we found an incidence of 5.17 new cases per million population per year. Unlike our predictions before the study, we confirmed the world-wide incidence of PG. To our knowledge, this is the first observational, multicentre study on PG. We hope that it provides a stimulus for further researches on PG and for the creation of an Italian register.

Staphylococcus lugdunensis cutaneous infection with sporotrichoid distribution.

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.

Giant Centrifugal and Necrotizing Cutaneous Metastases of Melanoma: A Brief Review of the Literature and a Case Report.

BACKGROUND: Melanoma is a neoplasm derived from melanocytes of the skin and other sites. In the natural history of melanoma, cutaneous metastases occur relatively frequently and can arise in early or late stages of the disease. OBJECTIVE AND METHODS: Melanoma skin metastases have a variable appearance. Several clinical appearances are described in the literature. RESULTS AND CONCLUSION: We describe an unusual clinical pattern of cutaneous melanoma metastasis, defined as giant, centrifugal, and necrotizing, and we provide a brief review of the literature.

Sporotrichoid Mycobacterium marinum infection in an elderly woman.

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.

Cutaneous melanoma frequencies and seasonal trend in 20 years of observation of a population characterised by excessive sun exposure.

BACKGROUND: Cutaneous melanoma is an aggressive form of skin cancer. It has become an increasingly common neoplasm in the most developed countries, especially among individuals of European origin. PATIENTS AND METHODS: Anonymous data of patients with cutaneous melanoma were collected from the diagnostic database of the University Hospital of Trieste from 1 January 1990 to 10 December 2013. Our study is based on a population which was constant over the period of observation; it was also well-defined and characterised by unrestrained sun exposure. RESULTS: The number of cutaneous melanomas increased during the period of observation with a seasonality trend and gender related differences both for anatomical sites distribution and stage of the disease. Moreover, 6% of our cohort developed multiple melanomas. CONCLUSIONS: In a well-defined population devoted to excessive sun exposure the frequencies of skin melanomas roughly doubled from 1990 to 2013 following a seasonal trend. In that population, prevention efforts according to gender specific risk behaviour, as well as follow-up programmes both for evaluation of metastatic spreading and for early diagnosis of additional skin melanomas, are crucial due to gender specific differences and to the occurrence of multiple melanomas.

Electrochemotherapy as a novel treatment for primary cutaneous marginal zone B-cell lymphomas.

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.

Giant melanoma of the abdomen: case report and revision of the published cases.

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.

Dermatomyositis: nailfold capillaroscopy patterns and a general survey.

INTRODUCTION: Dermatomyositis (DM) is a group of autoimmune idiopathic inflammatory myopathies characterized by typical cutaneous signs and symptoms of muscle involvement. The diseases can be associated with cancer in the paraneoplastic syndrome, calcinosis, interstitial lung disease, other autoimmune connective tissue diseases (in overlap syndrome), and Raynaud's phenomenon. METHODS: Clinical and capillaroscopic data were gathered from 43 patients with DM. The diagnosis was based on the Bohan‒Peter and European League against Rheumatism / American College of Rheumatology (EULAR/ACR) classification criteria. In addition, nailfold capillaroscopy was performed in all patients. RESULTS: In our cohort, eight patients had overlap syndrome, six had paraneoplastic syndrome, eight presented with interstitial lung disease, and nine had calcinosis, two of whom also had a cancerous pathology. Raynaud's phenomenon was reported in 74% of patients. Upon nailfold capillaroscopy, 84% of patients presented giant capillaries, 81% ramified capillaries, and 70% both. The latter, notably giant ramified capillaries, could be considered specific for DM. The detection of prominent subpapillary venous plexuses was associated with pulmonary involvement. In contrast, alterations of the pericapillary spaces were associated with the severity and prognosis of DM. CONCLUSIONS: Our results underline the usefulness of nailfold capillaroscopy in the diagnosis and prognosis of DM. Based on the results and literature data, specific nailfold capillaroscopy features should be included in DM diagnostic criteria.

Pediatric Lyme Disease in Northern Italy: An 18-Year Single-Center Case Series.

Tracing the profile of pediatric Lyme borreliosis (LB) in Europe is difficult due to the interregional variation in its incidence and lack in notifications. Moreover, the identification of LB can be challenging. This study is an 18-year case series of 130 children and adolescents aged under 18 years referred to the Pediatric Infectious Diseases Unit at L. Sacco Hospital, Milan, with suspicion of LB, between January 2005 and July 2023. The routine serological workup consisted of a two-step process: an initial screening test followed by Western blot (WB). Forty-four (34%) patients were diagnosed with LB. The median age was six years, and 45% were females. Of the children with erythema migrans (EM), 33 (57%) were confirmed as having true EM, and, of these, 4 (12%) were atypical. Ten (23%) patients had early disseminated/late diseases, including facial nerve palsy (n = 3), early neuroborreliosis (n = 1), arthritis (n = 3), relapsing fever (n = 2), and borrelial lymphocytoma (n = 1). No asymptomatic infections were documented. Over seventy percent of confirmed LB cases (n = 31/44) recalled a history of tick bites; in this latter group, 19 (61%) were from the area of the Po River valley in Lombardy. Almost half of the children evaluated for LB complained of non-specific symptoms (fatigue, musculoskeletal pain, skin lesions/rash, and persistent headache), but these symptoms were observed in only two patients with confirmed LB. Most LB cases in our study were associated with EM; two-tier testing specificity was high, but we found frequent non-adherence to international recommendations with regard to the timing of serology, application of the two-step algorithm, and antibiotic over-prescription. Most children were initially assessed for a tick bite or a skin lesion suggestive of EM by a family pediatrician, highlighting the importance of improving awareness and knowledge around LB management at the primary healthcare level. Finally, the strengthening of LB surveillance at the national and European levels is necessary.

Lyme Disease: A Review with Emphasis on Latin America.

The spirochete Borrelia burgdorferi sensu lato (Lyme Group) is the causative agent of Lyme disease, transmitted to humans through tick bites carrying the bacteria. Common symptoms include fever, headache, fatigue, and the characteristic erythema migrans skin rash. If left untreated, the infection can affect joints, the cardiac system, and the nervous system. Diagnosis relies on symptoms, clinical signs (such as the rash), and potential exposure to infected ticks, with laboratory tests proving valuable when appropriately employed with validated methods. Most cases of Lyme disease respond effectively to a few weeks of antibiotic treatment. In Latin America, knowledge of Lyme disease is limited and often confounded, underscoring the significance of this review in aiding medical professionals in recognizing the disease. This study delves explicitly into Lyme disease in Argentina, neighboring countries, and other Latin American nations.

Differences in tumor thickness between hematoxylin and eosin and Melan-A immunohistochemically stained primary cutaneous melanomas.

Tumor thickness (Breslow thickness) represents the main prognostic factor in primary melanoma. Potential differences in melanoma tumor thickness measurements between conventional hematoxylin and eosin (H&E) and Melan-A immunohistochemical staining were evaluated. Ninety-nine excisional biopsies were included in the study. From each sample, 2 consecutive histological sections were stained with H&E and Melan-A, respectively. Tumor thickness was measured from both sections by 2 independent observers. In 59 biopsy specimens (59.6%), higher tumor thickness measurements were recorded in Melan-A-stained than in H&E-stained sections. In 42.4% of such cases (25 biopsies), the observed differences were ≥0.2 mm. After Melan-A evaluation, 33% of in situ melanoma cases were reclassified as invasive melanoma, with thickness measurements ranging from 0.15 to 0.35 mm. In 23 biopsies, identical values were recorded with both techniques, whereas in 17 cases, measurements obtained with H&E staining were slightly higher (from 0.01 to 0.18 mm) than those obtained with Melan-A staining. A high rate of interobserver agreement was noted, and significant intertechnique measurement differences were detected. Significant discrepancies (≥0.2 mm) in thickness measurements between the 2 techniques were mainly attributed to the presence of individual or small clusters of melanocytic cells in the papillary dermis. These melanocytic cells could be easily overlooked in H&E-stained sections, especially in sections showing dense lymphohistiocytic inflammatory infiltrates, numerous melanin-containing histiocytic cells in the upper dermis, or extensive fibrotic changes or regression phenomena. This study confirms the practical interest of immunohistochemical staining with Melan-A in evaluating primary melanoma and, specifically, in situ melanoma cases.

Cutaneous B-cell chronic lymphocytic leukaemia resembling a granulomatous rosacea.

B-cell chronic lymphocytic leukemia (B-CLL) is a low-grade lymphoproliferative disease. Cutaneous involvement of B-CLL is limited and, in most cases, it represents non-specific manifestations related to an impaired immune system. Leukemic skin infiltrates (leukemia cutis) occur in 4-20% of patients. Herein we report the case of a 65-year-old woman with B-CLL presenting with papular, nodular, and plaque skin infiltrates affecting the nose, mimicking granulomatous rosacea. We discuss several aspects of rare cutaneous manifestations of B-CLL involving the face.

Red Palms' Syndrome and Red Fingers' Syndrome: A Mini Review.

Red palms syndrome consists of an intense redness on the palms of the hands and, occasionally, the soles of the feet. This infrequent condition may be primary or secondary. The primary forms are either familial or sporadic. They are always benign and do not require treatment. The secondary forms may have a poor prognosis related to the underlying disease, for which early identification and treatment are imperative. Red fingers syndrome is also rare. It manifests as a persistent redness on the fingers or toes pulp. It is typically secondary either to infectious diseases like human immunodeficiency virus, hepatitis C virus and chronic hepatitis B or to Myeloproliferative Disorders, such as Thrombocythemia and Polycythemia vera. Manifestations spontaneously regress over months or years without trophic alterations. Treatment is limited to that of the underlying condition. Aspirin has been shown effective in Myeloproliferative Disorders.

The history of Lyme disease in Italy and its spread in the Italian territory.

Lyme borreliosis (LB) is the most common vector-borne zoonotic inflammatory disease in the Northern Hemisphere. In Italy, the first case was diagnosed in 1985 in a woman in Liguria, while the second, in 1986 in Friuli-Venezia Giulia, documenting the infection in northern Italy. Both diagnoses were confirmed by serological assessment by an indirect immunofluorescence (IFI) technique. Borrelia cultivation from both Ixodes ricinus ticks and human lesions in Trieste (Friuli-Venezia Giulia) identified Borrelia afzelii as the prevalent genospecies; nevertheless, Borrelia garinii, Borrelia burgdorferi (sensu stricto), and Borrelia valaisiana (VS116 Group) were also detected, although less frequently. LB was also documented in other Italian regions: in Tuscany (1991), Trentino-Alto Adige (1995-1996), Emilia-Romagna (1998), Abruzzo (1998), and more recently, Lombardy. Nevertheless, data on LB in other Italian regions, especially in southern Italy and islands, are poor. The aim of this study is to document the spread of LB in Italy through the collection of data from LB patients in eight Italian hospitals located in different Italian regions. Diagnostic criteria for LB diagnosis are as follows: i) the presence of erythema migrans (EM) or ii) a clinical picture suggestive of LB, confirmed by serological tests and/or PCR positivity for Borrelia detection. In addition, data also included the place of residence (town and region) and the place where patients became infected. During the observation period, 1,260 cases were gathered from the participating centers. Although different in extent from northern Italy to central/southern Italy, this study shows that LB is widespread throughout Italy.

A Stewart-Treves syndrome of the lower limb.

We report a case of a Stewart-Treves syndrome of the lower limb. The tumor is best described in the upper limb following breast cancer treatment but a small number of cases have arisen in lymphedema of the lower limb. Electrochemotherapy could be useful in the palliative treatment of this lymphangiosarcoma.

The Impact of Telemedicine in the Diagnosis of Erythema Migrans during the COVID Pandemic: A Comparison with In-Person Diagnosis in the Pre-COVID Era.

BACKGROUND: Erythema migrans (EM) is the hallmark manifestation of the Lyme borreliosis (LB), and therefore its presence and recognition are sufficient to make a diagnosis and to start proper antibiotic treatment to attempt to eradicate the infection. METHODS: In this study we compared the clinical data of 439 patients who presented an EM either according to the diagnostic modality through physical assessment or through telemedicine. CONCLUSIONS: Our data clearly show that telemedicine for EM diagnosis is useful as it enables prompt administration of appropriate antibiotic therapy, which is critical to avoid complications, especially for neurologic and articular entities. Therefore, telemedicine is a tool that could be adopted for the diagnosis of Lyme disease both by specialized centers but also by general practitioners.