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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

OBJECTIVE: To evaluate variations in laboratory parameters and diagnoses of selected clinical conditions up to 16 weeks after starting a new systemic psoriasis treatment for Psocare Registry enrollees. DESIGN: Prospective cohort study. SETTING: Italian public referral centres for psoriasis treatment. PATIENTS: First-time recipients (n = 10,539) of continuous systemic psoriasis treatment for at least 16 weeks. MAIN OUTCOME MEASURE: Mean variations in (weeks 8 and 16) and proportions of patients reaching a clinically meaningful increase in serum levels (week 16) of total and low-density lipoprotein cholesterol, triglycerides, aspartate amino transferase, alanine amino transferase and creatinine, as well as week-16 cumulative incidences of new diagnoses of diabetes mellitus and arterial hypertension. RESULTS: Mean cholesterol and triglyceride levels significantly increased in patients treated with acitretin or cyclosporine. Mean triglyceride levels also increased in efalizumab- and etanercept-treated patients. Mean transaminase values increased in methotrexate-treated patients, and mean aspartate amino transferase levels increased in infliximab-treated patients. The average serum creatinine value increased in cyclosporine-treated patients. Acitretin and cyclosporine were associated with risk of hypercholesterolaemia (odds ratios 1.51 and 1.34) and acitretin with risk of hypertriglyceridaemia (odds ratio 1.43). Methotrexate and infliximab were associated with risk of more than doubling the upper normal aspartate amino transferase (odds ratios 2.06 and 1.87) and alanine amino transferase (odds ratios 2.38 and 1.74) values. The relative risk of developing arterial hypertension and diabetes was increased for patients receiving cyclosporine (odds ratios 3.31 and 2.88). CONCLUSION: Systemic treatments for psoriasis resulted in heterogeneous effects on the parameters analysed.

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.

Matrix metalloproteinases (MMPs) are associated with Kaposi's sarcoma (KS) tumorigenesis and may contribute to the mechanism of KS invasive growth. To date, only a few MMPs have been studied in KS lesions, and exactly which MMPs are involved in KS development and progression remains unanswered. However, MMPs 2 and 9 have been associated with different phases of angiogenesis, but their role in the proteolytic modification of the extracellular matrix has not been investigated. The results of this study confirm that MMPs, specifically MMP-2 and MMP-9, can contribute to angiogenesis by disrupting the vessel basement membrane and other extracellular matrix barriers, and enabling endothelial cells migration through the surrounding tissues.

Background The term “leishmaniasis” defines a group of vector-borne diseases caused by species of the genus Leishmania and characterized by a spectrum of clinical manifestations. Parasite properties (infectivity, pathogenicity, virulence), host factors, and host responses regulate heterogeneous disease expression. Sicily is one of the major islands of the Mediterranean Basin and is considered to be a hypo-endemic area for cutaneous leishmaniasis. Leishmania infantum is the most common species on the island. Methods Fifty patients (both sexes and different ages) with lesions clinically suggestive of cutaneous leishmaniasis were recorded over a 1-year period. The diagnosis was based on positive slit-skin smear and histopathologic studies when needed. Polymerase chain reaction (PCR) was performed as test confirmation. Results Twenty-five patients had typical solitary lesions of cutaneous leishmaniasis. Multiple lesions were present in five patients. In 20 patients, the lesions were very unusual, including erysipeloid, zosteriform, and lupoid leishmaniasis. The results of Leishmania isoenzyme characterization identified Leishmania infantum as the species responsible for the 20 atypical cases. Conclusion The global number of cases of cutaneous leishmaniasis in Sicily has increased in recent years, and such increases can be explained, in part, by the fact that, in this region, sandflies are present during a large part of the year. This is a result of the climatic variation in recent years (increasing temperature and humidity). There has also been an increase in the number of new and rare variants of cutaneous leishmaniasis. A knowledge of the unusual clinical variants of cutaneous leishmaniasis, as well as classical forms, allows early detection.

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.

AGE6. Skin Ageing: Focus on the Role of Inflammatory Genetic Factors in Cutaneous Neoplasia C. M. Gambino1, F. Crapanzano1, G. Accardi1, A. Aiello1, C. Virruso1, G. Pistone1, M. R. Bongiorno1, D. Lio1, C. R. Balistreri1, G. Candore1 1University of Palermo, Palermo, Italy Background: Skin aging is a complex process that involves intrinsic and exogenous causes. Photo-oxidative damage caused by UV is the leading cause of extrinsic aging of the skin, known as photo-ageing. UV damages can be linked mostly to overproduction of ROS that induces a complex molecular cascade able to accelerate physiological aging, determining a typical dermal/epidermal inflammation with an increased risk of getting skin cancer. The skin response is strongly influenced by individual genetic background. Thus, polymorphisms in candidate inflammatory genes might play a role in photo-ageing and skin cancer. Methods: A total of 30 Sicilian subjects (12 females and 18 males; age range, 65–90 years) were enrolled. From histo-pathological data, 12 subjects showed skin cancer. Their blood samples have been used to obtain DNA samples and have been genotyped for TLR4 (+896 A/G; rs.4986790), TLR4 (+1196 C/T, rs.4986791), MMP2 (-1306 C/T, rs.243865) and MMP9 (-1562 C/T, rs.3918242) using a RFLP-PCR. Results: Allelic and genotypic frequencies of the SNPs analysed were evaluated by gene count. No significant differences in frequencies of these SNPs among cases and controls were observed. Conclusions: Our results are preliminary, so it is certainly necessary to increase the sample size of our study. In fact, the possible role of these SNPs in skin-ageing related neoplasia might open new perspectives for their analysis and prevention. However, it is imperative to underline the concept that functional effects of each SNP depend on the presence of one or different environmental causes (UV radiation, smoking, etc.).

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

Background Avian mite dermatitis is a skin disease caused in mammals by the incidental bites of blood-sucking mites which customarily parasitize wild and domestic birds. It manifests in the form of pruritic, erythematous, or urticarial papules, with a central sting mark, in skin regions normally covered by clothing. The species mainly implicated in human bite cases are Dermanyssus gallinae, Ornithonyssus sylviarum and, less frequently, Ornithonyssus bursa. The latter is mainly a tropical and subtropical mite and its – presumably transitory – presence has been recorded only once in Europe, in migratory birds. Case report We report a case of avian mite dermatitis in a 70-year-old man, an owner of chickens, who lived in Sicily, an island in southern Italy. He presented with an itching, erythematous, papular eruption. Numerous mites were seen racing across his skin. The precise identification of O. bursa was based on the morphology of its plates and chelicerae and on the arrangement of its setae. Conclusions Not only does this paper report the first European case of human infestation with O. bursa, it provides evidence that this alien species has settled and spread in the Old Continent. It may have been flown in from a small focus reported in Danish migratory birds in the 1980s or may have been accidentally introduced into Italy through the importation of infested poultry from South America. Such occurrences may have unpredictable epidemiological and ecological consequences. More comprehensive veterinary inspection of imported birds is desirable.

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.

Objective To evaluate variations in laboratory parameters and diagnoses of selected clinical conditions up to 16 weeks after starting a new systemic psoriasis treatment for Psocare Registry enrollees. Design Prospective cohort study. Setting Italian public referral centres for psoriasis treatment. Patients First-time recipients (n = 10,539) of continuous systemic psoriasis treatment for at least 16 weeks. Main outcome measure Mean variations in (weeks 8 and 16) and proportions of patients reaching a clinically meaningful increase in serum levels (week 16) of total and low-density lipoprotein cholesterol, triglycerides, aspartate amino transferase, alanine amino transferase and creatinine, as well as week-16 cumulative incidences of new diagnoses of diabetes mellitus and arterial hypertension. Results Mean cholesterol and triglyceride levels significantly increased in patients treated with acitretin or cyclosporine. Mean triglyceride levels also increased in efalizumab- and etanercept-treated patients. Mean transaminase values increased in methotrexate-treated patients, and mean aspartate amino transferase levels increased in infliximab-treated patients. The average serum creatinine value increased in cyclosporine-treated patients. Acitretin and cyclosporine were associated with risk of hypercholesterolaemia (odds ratios 1.51 and 1.34) and acitretin with risk of hypertriglyceridaemia (odds ratio 1.43). Methotrexate and infliximab were associated with risk of more than doubling the upper normal aspartate amino transferase (odds ratios 2.06 and 1.87) and alanine amino transferase (odds ratios 2.38 and 1.74) values. The relative risk of developing arterial hypertension and diabetes was increased for patients receiving cyclosporine (odds ratios 3.31 and 2.88). Conclusion Systemic treatments for psoriasis resulted in heterogeneous effects on the parameters analysed.

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.

BACKGROUND: One of the major goals of pemphigus therapy is to reduce the patient's cumulative exposure to systemic corticosteroids. To investigate the efficacy of enteric-coated mycophenolate sodium (EC-MPS), 10 patients with active, refractory pemphigus vulgaris (PV) or foliaceous (PF) were treated with EC-MPS (1440 mg daily) and prednisone (75 mg daily) over 18 months. OBSERVATIONS: Following EC-MPS/prednisone therapy, disease progression was inhibited between days 30 and 45 in 9/10 patients (8 PV; 1 PF). At 18 months, 8/9 PV patients had clinically quiescent disease; EC-MPS therapy was no longer required in two patients as a result of disease remission. The remaining PV patient showed no response to treatment. The PF patient also had clinically quiescent disease but with high levels of anti-desmoglein-1. ECMPS dose was reduced to 720 mg daily in 4/9 patients by month 6. Average daily prednisone requirement decreased to 25 mg at 6 months and to 15 mg at 18 months. Three adverse events were reported: headache (two cases; one mild and one moderate) and significant increase in blood glucose (one case; moderate). CONCLUSIONS: Enteric-coated mycophenolate sodium is effective and safe as an adjuvant therapy in patients with refractory pemphigus and may be effective even in patients whose disease is unresponsive to azathioprine.

Introduction: Neurofibromatosis type 1 is an autosomal dominant disorder that occurs across all ethnic groups and affects approximately one in 4000 individuals. One of the most noticeable characteristics of the disease is the development of neurofibromas. Case presentation: A total of 258 patients (131 women, 127 men) with neurofibromatosis type 1 were evaluated between 1994 and 2004 in our hospital's dermatology department. Nine patients (3.45%, 95% confidence limits 1.22 to 5.68) had neurofibromas of the breast. One of these nine patients presented with an extensive congenital plexiform neurofibroma in the outer quadrants of her right breast, extending to the nipple-areolar complex. Meanwhile, three patients had more than one neurofibroma on the nipple-areolar complexes. Three patients had a family history of neurofibroma. Over the years 1994 to 2004, the cutaneous lesions were not associated with any malignancies. Presenting symptoms were related to conditions such as increasing size of the mass, and associated loss of function and pain. Conclusions: This study suggests that the changes are limited to particular subgroups. That neurofibromatosis is more prevalent in women (7 women and 2 men) suggests that being female could be a susceptibility factor for the development of neurofibromas of the nipple-areolar complexes. There are few reports in the literature describing breast carcinomas in association with von Recklinghausen disease. It has been speculated that the presence of multiple neurofibromas of the breast may obscure a breast mass at palpation, leading to a delay in clinical detection. We suggest that patients with neurofibromas of the breast have more rigorous clinical and mammographic screening of the breast during adulthood to determine the presence or absence of malignancies. The finding that both the neurofibromatosis type 1 gene and a breast cancer predisposition gene are located in close proximity on chromosome 17q makes the association of these two conditions intriguing.

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.