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SummaryBackground The nationwide prevalence of latent tuberculosis infection (LTBI) in Italian patients with psoriasis has never been investigated. Objectives To estimate the nationwide prevalence of LTBI in Italian patients with psoriasis who are candidates for systemic treatment. Methods Data were obtained from the Psocare Registry on those patients (n = 4946) with age > 18 years, systemic treatment at entry specified and tuberculin skin test (TST) performed according to the Mantoux method. LTBI diagnosis was based on a positive TST result in the absence of any clinical, radiological or microbiological evidence of active tuberculosis. Results Latent tuberculosis infection was diagnosed in 8·3% of patients with psoriasis (409 of 4946). The prevalence of LTBI was lower in patients on biologics than in those on conventional systemic treatments, ranging from 4·3% (19 of 444) of patients on adalimumab to 31% (eight of 26) of those on psoralen–ultraviolet A (P

The coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, is creating an unprecedented global public health emergency with the continuous growth of infected individuals worldwide. Italy was one of the first European country to face the first wave of infection outside mainland China. The first case of COVID-19 was confirmed in Lombardy on February 20th , 2020, and subsequently, a rapid increase in the number of detected cases was observed, spreading through Italy and the rest of Europe.3 As of April 22nd , confirmed COVID-19 cases in Italy were 183,957.

Background: Psoriasis is a chronic inflammatory skin disease affecting 2-3% of the population in the world. The scalp is the most common, and frequently the first site of disease involvement. Occasionally it may be the only localization of psoriasis. Objective: Treatment of scalp psoriasis is often unsatisfactory, due to limited available topical therapy and reduced efficacy of some systemic drugs. Biologic therapies are recommended for severe psoriasis, resistant to topical treatment, but evidence from randomized, controlled studies is lacking regarding effectiveness on scalp-localized lesions. The aim of this paper is to show our experience on the efficacy of secukinumab on scalp psoriasis involvement. Methods: Adult patients with moderate to severe plaque psoriasis located in the scalp, who were eligible for biological therapy were selected for treatment with secukinumab. Results and Conclusion: Several clinical studies have shown the efficacy of secukinumab on plaque psoriasis, and some encouraging experience suggest the use in difficult sites such as the scalp; this article reports effective treatment with secukinumab of a series of patients with plaque and scalp psoriasis.

Targetoid hemosiderotic hemangioma represents an uncommon, distinctive, benign lympho-vascular lesion, with a variable clinical appearance, typically occurs in the lower limbs and trunk. Most patients are in their 20's or 30's. We describe three dermoscopic cases followed by the review of the literature. Dermoscopic images have been collected and have been evaluated for the presence of dermoscopic features. Concerning the review, we researched on pubmed and records were reviewed noting patient age of onset, sex, location, overall size, dermoscopic appearance. The harmonic mean age of patients was 31 years; the female-to-male ratio was 1:1, and the most common locations were lower extremities (47%) and the trunk (29%). In the most recent years several pediatric cases were described, lowering the mean age of the patients in the literature. Most targetoid hemosiderotic hemangioma appear clinically as a small, solitary, reddish-violaceous vascular lesion with a targetoid appearance. The most typical dermoscopic findings are a homogeneous central area, red lacunae, dark lacunae, peripheric red-violaceous ring, whitish structures, peripheric vascular structures, yellowish intermediate areas, and a peripheral pigment network. Histologically, more superficially, in the papillary dermis there are dilated thin blood vessels lined by hobnail-appearing endothelial cells. In the deeper dermis there are typically slit-like capillaries that ramify in the dermis and dissect through collagen bundles. We wanted to evaluate the features of a large series of targetoid hemosiderotic hemangiomas for a better awareness of the entity, to improve its clinical and dermoscopic diagnostic accuracy, to focus attention on nontargetoid patterns, which often mimic other lesions, including melanoma.

We report a case of multiple cutaneous metastases from prostate cancer. A 78-year-old man with an 8-year history of prostate cancer had multiple nodular lesions in the chest. Histologically, the lesion showed an abortive glandular lumina and tall columnar cells with abundant cytoplasm. Immunohistochemical staining for AE1:AE3 cytokeratin cocktail, prostate-specific antigen, and prostate-specific acid phosphatase was positive in tumor cells, confirming the diagnosis of cutaneous metastases from prostate cancer. We report this case because of the rarity of cutaneous metastases from prostatic adenocarcinoma in the chest region.

Psoriasis and psoriatic arthritis are common diseases associated with considerable morbidity and disability. Their pathophysiology comprises similar processes leading to inflammation of skin, entheses, and joints. Although traditional systemic agents can be effective, their use may be limited by lack of efficacy and concerns regarding adverse effects. The objective of this study was to assess the efficacy and safety of adalimumab, a fully human antitumor necrosis factor (anti-TNF) monoclonal antibody, over 16 weeks. The present authors report their personal experience in 15 patients with severe plaque psoriasis and psoriatic arthritis, refractory to other treatments, in which a decisive regression of joint/skin involvement was obtained. Psoriasis and psoriatic arthritis are chronic inflammatory disorders resulting from a combination of genetic and environmental factors.

Pemphigus vulgaris (PV) is a severe chronic autoimmune blistering disease of skin and mucous membranes. The use of systemic corticosteroids in pemphigus has dramatically reduced its mortality rate, but the long-term use of steroids leads to severe side effects, many of which are serious. For this reason it is often necessary to add immunosuppressive agents to the regimen. However, there are occasional refractory cases in which therapy with conventionally accepted modalities is either not efficacious or not possible on account of side effects. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. We present an instance of refractory PV successfully treated with rituximab. The successful treatment of pemphigus described here demonstrates that rituximab is a viable therapeutic option for patients with refractory PV.

Objective: The aim of this study is to analyse alterations of the tongue and the correlation between these lesions and different types of tumor. Subjects and methods: A total of 258 cases (131 females, 127 males) of neurofibromatosis type 1 were screened between 1994 and 2004 in our Dermatology Department. All patients included in this study have NF1, as defined by the NIH Consensus Conference. Three cases of neurofibromas of the tongue in patients with neurofibromatosis type were reported. Results: Our patients showed nodular lesions on the tongue, related to neurofibromas in two patients and plexiform neurofibroma in one patient, respectively. Clinical and hystopatological findings were useful in distinguishing between neurofibromas and other soft tissue tumors. An increased prevalence of malignancy has been documented in patients affected by neurofibromatosis type 1. Changes in the size of a pre-existing mass, compression, or infiltration of the adjacent structures indicate malignant degeneration. Histological and clinical evaluation should be performed in order to choose the most appropriate treatment strategy for these patients. Conclusion: The oral manifestations of NF are well-documented but may not be at the forefront of the clinician's mind in the differential diagnosis of intra-oral swellings.

Summary Background Lipoid proteinosis (LP), also known as Urbach–Wiethe disease, is a rare autosomal recessive disorder characterized by a hoarse voice, warty skin infiltration and scarring. Mutations within the extracellular matrix protein 1 (ECM1) gene cause LP. Objectives We report the molecular analysis of the ECM1 gene in a Sicilian patient with LP in order to extend the mutation spectrum of this genodermatosis. Methods We studied a 32-year-old female born from consanguineous parents who was diagnosed at the age of 11 years as having LP. She has a clinical phenotype corresponding to Urbach–Wiethe disease characterized by papules/nodules, indurated plaques and sometimes ulcerated lesions primarily involving the skin and mucous membranes, and extracutaneous features such as epilepsy, hoarseness of the voice and neuropsychiatric abnormalities. Samples of clinically affected skin obtained by biopsies were analysed after staining with haematoxylin and eosin, periodic acid–Schiff (PAS), and PAS–diastase. The whole ECM1 gene was analysed by direct sequencing. Results We identified a homozygous nonsense mutation in exon 6 of the ECM1 gene, C589T (Q197Ter). Conclusions Over 60% of mutations occur in exons 6 and 7. Exon 7 is alternatively spliced and frameshift mutations in exon 7 lead to ablation of the ECM1a transcript, but not the shorter ECM1b transcript that normally lacks this exon. Homozygous nonsense or frameshift mutations in exon 6 are predicted to affect both full-length ECM1a and ECM1b transcripts, whereas ECM1b should be unaffected for similar types of mutation in exon 7. It has been suggested that individuals with mutations in exon 7 have a slightly milder phenotype than those with exon 6 mutations. This is the first report with respect to a novel mutation of the ECM1 gene responsible for recessive LP in Sicily.

Purpose: The purpose of this study was to show that gender also plays an important role in pharmacokinetics, pharmacodynamics, and drug toxicity. It is only fair to take into account the so-called “gender-based medicine.” Methods: We again selected admission for cutaneous adverse drug reaction (CADRs), from January 2012 to July 2012, in order to detect and verify, in an analytical way, the substantial gender differences in adverse drug reactions in term incidence, clinical manifestations, severity and drugs involved. Results:In the period January–July 2012, at the Department of Dermatology and Sexually Transmitted Disease of A.O.U.P. “Paolo Giaccone” Palermo, 384 patients were admitted, of which 192 were females (50%) and 192 males (50%). Out of a total of 384 patients, 34 cases of CADRs (8.9%) were identified including 24 females (71%) and 10 males (29%). Conclusions: The female gender is an important predisposing factor for the RAF: a large case series showed that in the RAF female/male ratio is 1.7:1.

Background: Some studies have shown that switching patients from one tumor necrosis factor (TNF)-alfa inhibitor to another may be beneficial when they have an inadequate response or an adverse event. Objective: We sought to assess the variables predicting the efficacy of the second TNF-alfa inhibitor in patients discontinuing the first TNF-alfa inhibitor. Methods: Data from all 5423 consecutive patients starting TNF-alfa inhibitor therapy for psoriasis between September 2005 and September 2010 who were included in the Italian Psocare registry were analyzed. Results: In 105 patients who switched to a second TNF-alfa inhibitor who had complete follow-up data, 75% improvement in the Psoriasis Area Severity Index score (PASI 75) was reached by 29% after 16 weeks and by 45.6% after 24 weeks. Patients who switched because of secondary loss of efficacy (loss of initial PASI 75 response) or adverse events/intolerance were more likely to reach PASI 75 than those who switched as a result of primary inefficacy (PASI 75 never achieved) (hazard ratio 2.7, 95% confidence interval 1.3-5.5 vs hazard ratio 2.0, 95% confidence interval 1.0-3.9 and 1, respectively). Limitations: There was a small number of patients with complete follow-up data. Conclusion: PASI 75 response in patients who switched from one antie-TNF-alfa agent to another was significantly reduced in patients who showed primary inefficacy of the first antie-TNF-alfa.

Adverse drug reactions to highly antiretroviral therapy (HAART) are major obstacles in its success. Although overall mortality from HIV has dramatically declined owing to HAART, these antiretroviral regimens have been associated with a wide spectrum of severe cutaneous reactions. The severity of cutaneous adverse reactions varies greatly, and some may be difficult to manage. To optimize adherence and efficacy of antiretroviral treatment, clinicians must focus on preventing adverse effects whenever possible, and distinguish those that are self-limited from those that are potentially serious. This paper presents the case of a serious cutaneous adverse reaction to Atripla in a HIV-positive 50-year-old Caucasian woman.

Background Melanoma incidence/mortality is increasing worldwide. “Euromelanoma Day” is a pan-European campaign for skin cancer prevention. Results of the 2010 Euromelanoma Day in Italy are reported herein. Materials and methods A questionnaire was used to collect data on participants' characteristics and suspected skin cancers. Result A total of 1085 participants was screened (64.1% females, median age 44 years). Suspicion rate, detection rate, and positive predictive values for melanoma were 1.3, 0.28 and 21.4%, respectively. Poorly educated, ≥35 years old, pale-skinned males were at higher risk for skin cancer than highly educated

Summary We report a case of a bot fly infestation of the scalp. A 45-year-old man after returning to Sicily noted a small white “worm” erupting from the upper lesion. Physical examination revealed a superficial furuncular lesion with central pores with sero-sanguineous discharge. The foreign body identified was diagnosed as the larva of the human bot fly, Dermatobia hominis.

Background. Psoriasis is a common inflammatory and immune-mediated skin disease. There is growing controversy as to whether cardiovascular risk is elevated in psoriasis. A number of studies suggest a high prevalence of cardiovascular risk factors as well as cardiovascular diseases in psoriasis patients. Objective. The objective of this study was to estimate cardiovascular risk score in psoriasis patients and the relation between cardiovascular risk and psoriasis features. Cardiovascular risk was assessed by CUORE project risk score built within the longitudinal study of the Italian CUORE project and suited to populations with a low rate of coronary heart disease. Results. A case-control study in 210 psoriasis outpatients and 111 controls with skin diseases other than psoriasis was performed. CUORE project risk score was higher in patients than controls (6.80 ± 6.34 versus 4.48 ± 4.38, P < 0.001). Compared to controls, psoriasis patients have higher risk of developing major cardiovascular events. Cardiovascular risk was not related to psoriasis characteristics. Conclusion. Increased focus on identifying cardiovascular risk factors and initiation of preventive lifestyle changes or therapeutic interventions in patients with psoriasis is warranted.

Although no study has definitively shown that unfocused screening of skin cancer is effective, many campaigns have been organized with the aim of increasing awareness on melanoma risk factors. The objective of this study was to analyse the results of the Skin Cancer Screening Day in Italy during the period 2005-2007, to determine the priorities for melanoma control plans in a Mediterranean country. A total of 5002 patients were screened by dermatologists in 31 cities. Individuals who considered themselves to have many naevi and those with a family history of melanoma showed a higher number of common and atypical naevi. Ten melanomas, 20 basal cell carcinomas and two squamous cell carcinomas were histopathologically confirmed. Our observations provide the following suggestions for melanoma prevention strategies: (a) an unfocused campaign is suitable to inform the public about the importance of self-examination of the skin, but is not useful to identify a larger number of melanomas; and (b) melanoma screening campaigns should focus on a selected population, which meets rigorous risk criteria to maintain higher cost-effectiveness. The financial support to effective melanoma screening programmes could be increased, especially in southern populations where lower levels of self-surveillance and socioeconomic conditions represent risk factors for late identification of melanoma.

Introduction: Pemphigus is an autoimmune blistering disease of the skin and mucous membranes characterized by the development of autoantibodies against the desmosomal proteins, desmoglein-1 and -3. Before the advent of corticosteroids, therapy was almost fatal. The introduction of high-dose corticosteroid therapy has reduced mortality rates to similar to 10%, but long-term use of steroids can lead to side effects, many of which are severe and associated with significant morbidity. Thus, the major goal of pemphigus therapy has been to reduce the patient's cumulative exposure to systemic corticosteroids. Over the last 2 decades, a range of corticosteroid-sparing immunosuppressive agents have been described, but these therapies are not without potentially serious complications. Despite the range of treatment options, a proportion of patients do not achieve remission, while others show an initial treatment response but remain poorly controlled. The recent availability of mycophenolate mofetil (MMF), originally developed for preventing allograft rejection, appears to be effective in autoimmune blistering diseases in combination with systemic corticosteroid or as a monotherapy. Areas covered: This review aims to provide an extensive overview of the literature on the clinical pharmacokinetics of MMF in pemphigus treatment and a brief summary of current pharmacodynamic information. After completing this learning activity, readers should be able to summarize the pharmacology of MMF as an immunosuppressant; recognize its potential role in the treatment of pemphigus, including general dosing guidelines and laboratory monitoring schedules, use in patient populations and potential adverse effects; and identify future considerations and developing areas of research regarding the use of mycophenolic acid in the treatment of autoimmune blistering diseases Expert opinion: Current morbidity of pemphigus is largely iatrogenic, caused by side effects of the long-term, high-dose corticosteroid therapy that is necessary to sustain disease control. MMF demonstrates complex pharmacokinetics and displays large between-subject pharmacokinetic variability. Experience with MMF has demonstrated long-term safety and tolerability in the treatment of pemphigus.

We present a rare case of a 34-year-old patient with persistent, progressive, acquired leukonychia totalis and partialis. Idiopathic acquired leukonychia is a rare chromatic disorder of the nail not associated with other abnormalities and discernible etiology. Our case report did not link the inheritance of leukonychia with diverse clinical syndromes. To our knowledge, only five cases of idiopathic, acquired, true total leukonychia were found in literature. This case was the sixth patient with asymptomatic idiopathic, white fingernails, and toenails without a hereditary cause.

We present the case of a woman with diabetes insipidus with subsequent genital and multiorgan Langerhans cell histiocytosis (LCH). A monolateral and slightly infiltrated erythematous plaque of the vulva was observed. Hematoxylin and eosin and immunophenotypic studies were performed. The primary antibodies used were monoclonal antibody to S100, CD1a, CD34, HLA-DR, PCNA, CD45Ro, CD40, and langerin. The histology of the infiltrates revealed a granulomatous reaction pattern, with extensive aggregates of histiocyte proliferation. The histiocytes, morphologically characterized by a pale staining of cytoplasm surrounding a grooved reniform nucleus, sometimes contained small distinct nucleoli. Lymphocytes, eosinophils, macrophages, and both plasma cells and giant cells typically infiltrated the lesions. Cells CD1a+ and S100+ infiltrated the epidermic and were dispersed over the infiltrates as well as in clusters, and around the vessels. A considerable number of CD40-expressing cells were restricted to CD1a+ LCH cells. The specimen contained a high percentage of langerin+ cells in both the dermis and the epidermis. The clinical manifestations of LCH affecting the genital area can be diverse, and in most patients take the form of ulcers or erythematous plaques. Histopathologic examination of the lesion evidences a mixture of Langerhans cell histiocytes (CD1a+, S100+, HLADr+, CD207+, CD 40+), lymphocytes (predominantly helper [CD4] CD 45 Ro+), eosinophils, and macrophages. Each of the cell types produces a “cytokine storm.” Many of the cytokines favor recruitment of Langerhans cell progenitors and rescue the Langerhans cell histiocytes from apoptosis.

Summary A patient is described with tuberculoid leprosy and Type 1 (lepra) reaction from Sicily a non-endemic region, who lived previously in Manila from 2000 to 2005. The skin lesions became acutely inflamed and edematous. The plaques were painless to touch or pinprick, and there was swelling of the nerves in the fibro-osseous tunnels under the surface of the skin, including both the ulnar nerve at the elbow, and the posterior tibial nerve (medial malleolus). During the course of electro-neurographic studies, conduction velocity in the motory nerves indicated a slowing-down. The diagnosis of leprosy was confirmed by residence in an endemic area for about 5 years, by simultaneous skin lesions and peripheral nerve abnormalities, and by skin biopsy. Outside of endemic areas, diagnosis remains a challenge for physicians for mainly two reasons. Firstly, the incubation period of leprosy is uniquely long among bacterial diseases and varies from a month to over 40 years. Secondly, outside leprosy-endemic areas, the diagnosis of leprosy is usually not considered, and patients are likely to be examined by a wide range of specialists. Physicians outside endemic areas should consider leprosy as a possible differential diagnosis if a patient from leprosy-endemic regions presents with painless skin lesions, nerve enlargement, or persistent skin lesions.

The potential role of stem cells in neoplasia has aroused considerable interest over the past few years. A number of known biologic characteristics of melanomas support the theory that they may originate in a mutated stem cell. Melanocytic stem cell markers have been described recently. Moreover, the CD133 cells that show surface markers for CD34 are stem cells primitive. These stem cells are capable of differentiating into neurons, glia, keratinocytes, smooth muscle cells, and melanocytes in vitro. The identification of cancer stem/initiating cells with a crucial role in tumor formation may open up new pharmacologic perspectives. The purpose of this study is to detect the expression of CD133 and CD34, two putative markers of cancer stem cells in the lentigo maligna melanoma. Thirty cases of lentigo maligna melanoma were analyzed using indirect immunohistochemical staining. The vast majority of the samples analyzed showed the presence of rare cells, which were clearly positive for CD133 and CD34. Strong CD133 and CD34 staining was found in the outer root sheath of the mid-lower hair follicles, intermixed with atypical melanocytes extending along layers of the hair follicles. A number of these staminal cells were adjacent and intermixed with melanoma cells. This study supports the stem cell origin of this tumor and suggests that the precursor of the melanoma in question is a stem-like cell rather than the primitive melanoblast committed to be exclusively involved in melanocytic differentiation.

CONTEXT: Lipase maturation factor 1 (LMF1) gene is a novel candidate gene in severe hypertriglyceridemia. Lmf1 is involved in the maturation of lipoprotein lipase (LPL) and hepatic lipase in endoplasmic reticulum. To date only one patient with severe hypertriglyceridemia and related disorders was found to be homozygous for a nonsense mutation in LMF1 gene (Y439X). OBJECTIVE: The objective of the study was to investigate LMF1 gene in hypertriglyceridemic patients in whom mutations in LPL, APOC2, and APOA5 genes had been excluded. RESULTS: The resequencing of LMF1 gene led to the discovery of a novel homozygous nonsense mutation in one patient with severe hypertriglyceridemia and recurrent episodes of pancreatitis. The mutation causes a G>A substitution in exon 9 (c.1395G>A), leading to a premature stop codon (W464X). LPL activity and mass were reduced by 76 and 50%, respectively, compared with normolipidemic controls. The proband over the years has shown a good response to treatment. The proband's son, heterozygous for the W464X, shows normal plasma triglyceride levels. CONCLUSIONS: We identified the second novel pathogenic mutation in LMF1 gene in a patient with severe hypertriglyceridemia. LPL deficiency in our patient was milder than in the carrier of the Y439X previously described.

Objective: A clinical trial was performed to evaluate the efficacy, speed of response, side effects and quality of life of patients treated with calcipotriol/betamethasone dipropionate (Dovobet (R)) for 4 weeks followed by maintenance with calcipotriol for 8 weeks (group A) versus calcipotriol (Daivonex (R)) alone for 12 weeks (group B) for the treatment of psoriasis. Materials and methods: A total of 150 patients were enrolled and randomized to groups A and B. PASI and Skindex-29 were considered the outcome measures. Results: Ninety-six patients completed the trial. At weeks 2 and 4, both groups showed a significant clinical improvement compared to baseline; group A demonstrated a higher clinical response compared with group B (p < 0.001). Treatment with calcipotriol was associated with a gradual improvement in group B and maintenance of the results in group A. Similarly, the quality of life assessment showed a marked improvement in terms of Skindex-29 in both groups at weeks 2 and 4 compared to baseline. Both treatments were safe and well tolerated. Conclusions: Our results demonstrate a higher efficacy and more rapid onset of action with the two-compound ointment compared with calcipotriol cream alone in short-term treatment. However, sequential application of calcipotriol allows maintenance of the results.

Exogenous ochronosis is a paradoxical hyper-pigmentation of the skin caused by the long-term use of hydroquinone-containing bleaching creams. Ochronosis is an uncommon condition characterized by yellow-brown pigmented deposits in the dermis. We report two cases of exogenous ochronosis in two female patients of the sub-Saharan African population. The lesions were characterized by an asymptomatic hyper-pigmentation of the face with gradually progressive blue-black macular patches, and in case no. 2, in addition to dyschromic lesions, striae atrophicae were present. This phenomenon is the outcome of the use of skin care products containing high concentrations of hydroquinone- and glucocorticoid-based products, and, in addition, certain modalities in the use of bleaching products are likely to facilitate complications.