Metabolic syndrome prevalence in psoriasis: a cross-sectional study in the Italian population.

BACKGROUND: The pathogenesis of psoriasis is complex, with a significant role suggested for pro-inflammatory mediators. There is strong evidence of an association between psoriasis and the metabolic syndrome (MetS), a cluster of cardiovascular risk factors, which impose a substantial disease burden. OBJECTIVE: This study aimed to evaluate the prevalence of MetS and to examine the implications of disease severity, type 2 diabetes mellitus, and cardiovascular disease in a large cohort of Italian psoriatic patients representative of the whole population. METHODS: This was a cross-sectional study involving 13 dermatological clinics in Italy. The primary study endpoint was a comparison of the prevalence of MetS between psoriatic patients and a non-psoriatic control group; secondary endpoints included the influence of psoriasis severity on the prevalence of MetS, and the relative prevalence and risk of type 2 diabetes mellitus and cardiovascular disorders. RESULTS: A total of 720 patients were enrolled (n = 360 per group). The prevalence of MetS was 26.84% in the psoriatic population and 15.16% in the control population (p = 0.0001; adjusted odds ratio 1.96). MetS was associated with a greater degree of psoriasis severity, and the prevalence and risk of diabetes tended to be higher in psoriatic patients than in the control group. CONCLUSION: In the Italian population, the prevalence of MetS and associated comorbidities is elevated in patients with psoriasis compared with non-psoriatic subjects, as has been demonstrated in other countries. Our findings reinforce the importance of considering the implications of metabolic comorbidities in treating patients with psoriasis.

Multiple cutaneous abscesses revealing disseminated nocardiosis in a patient with chronic rheumatoid arthritis.

Nocardiosis is a challenging infection that is difficult to diagnose and treat. Delayed recognition and prolonged illness are major concerns. We report the case of a 65-year-old man who underwent treatment with corticosteroids for rheumatoid arthritis and then presented with multiple abscesses on the right leg. A complete assessment performed by the dermatologist led to the diagnosis of disseminated nocardiosis with involvement of the lungs and central nervous system. The patient's history revealed development of pulmonary disease approximately 1 year prior to the appearance of the skin lesions, which was not recognized by several specialists. Long-term antibiotic therapy was effective in treating the skin and central nervous system lesions, while the chest radiologic profile improved more slowly.

Leg ulcer in Werner syndrome (adult progeria): a case report.

Werner syndrome (WS; MIM#277700) or adult progeria, is a rare disease, associated with mutations of a single gene (RECQL2 or WRN), located on chromosome 8 (8p12). It codes a DNA-helicase, whose defects cause genomic instability. The highest incidences are reported in Japan and Sardinia (Italy). On this major island of the Mediterranean Basin, the WS cases have been observed in the northern areas. The authors describe the apparently first case reported in southern Sardinia, a 51-year-old woman, who was born in and resides in the province of Cagliari. She presented with a 9-year history of an intractable leg ulcer and other characteristic symptoms, including "bird-like" face, high-pitched voice, premature greying, short stature, abdominal obesity in contrast with thin body type, scleroderma-like legs, decreased muscle mass, diabetes, atherosclerosis, and premature menopause. A specialized genetic Institute of Research (IRCCS-IDI, Rome) confirmed the clinical diagnosis. There is no cure or specific treatment and patients must be periodically screened for an increased risk of cardiovascular and cerebrovascular disease and malignancies. Among the many findings, leg ulcers significantly affect the patient's quality of life. This problem may send the patient to the dermatologist, who finally suspects the diagnosis. Poor response to medical treatment may require aggressive repeated surgery, with poor or temporary results.

Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association?

BACKGROUND: Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management may be difficult to plan. We describe a very uncommon case of functioning glucagonoma associated with primary hyperparathyroidism (pHPT) in which genetic testing failed to detect germline mutations of MEN-1 and other known genes responsible for MEN1. CASE PRESENTATION: The patient, a 65-year old woman, had been suffering for more than 1 year from weakness, progressive weight loss, angular cheilitis, glossitis and, more recently, skin rashes on the perineum, perioral skin and groin folds. After multidisciplinary investigations, functioning glucagonoma and asymptomatic pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distal splenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two hyperfunctioning parathyroid glands were detected respectively by ultrasound with fine needle aspiration cytology and 99mTc-sestamibi scan with SPECT acquisition. Total thyroidectomy was performed, whereas selective parathyroidectomy was preferred to a more extensive procedure because the diagnosis of MEN1 was not supported by genetic analysis and intraoperative intact parathyroid hormone had revealed "adenoma-like" kinetics after the second parathyroid resection. Thirty-nine and 25 months after respectively the first and the second operation, the patient is well and shows no signs or symptoms of recurrence. CONCLUSIONS: Despite well-defined diagnostic criteria and guidelines, diagnosis of MEN1 can still be challenging. When diagnosis is doubtful, appropriate management may be difficult to establish.

Dermatophyte infections mimicking other skin diseases: a 154-person case survey of tinea atypica in the district of Cagliari (Italy).

BACKGROUND: Although usually simple, the diagnosis of dermatophyte infection is sometimes neglected. An observational study has been realized to evaluate the role of corticosteroid exposure (tinea incognito) and of other primary characteristics of the dermatophytosis that from onset mimic other diseases and mislead an unexperienced physician. MATERIALS AND METHODS: Between 1990 and 2009, all cases of atypical dermatophytosis mimicking other skin diseases were collected from the more general number of dermatophyte infections diagnosed at the Dermatology Department of Cagliari University, Italy. RESULTS: One-hundred and fifty-four cases (71 male/83 female, 2-81 years old) were studied, with a median of 7 cases/year. The most observed clinical forms were those mimicking impetigo, eczematous dermatitis, lupus erythematosus, polymorphous light eruption, psoriasis, and rosacea. The identified dermatophytes were: Microsporum canis (70 cases), Trichophyton rubrum (43 cases), Trichophyton mentagrophytes var. mentagrophytes (29 cases), Trichophyton mentagrophytes var. interdigitale (six cases), Microsporum gypseum (three cases), Epidermophyton floccosum (two cases), and Trichophyton verrucosum (one case). Diagnostic difficulties are discussed, with special attention to the origin of the pathomorphosis. CONCLUSIONS: In our experience, clinical atypia is not a mere consequence of corticosteroid therapy but present at the very onset of the illness, due to the variable dermatophyte invasive capacity, the site of invasion, physiological individual, and/or acquired condition, such as excessive washing or sun exposure. Therefore, we suggest using the term "tinea atypica" rather than "tinea incognito" to include all forms of dermatophytosis that do not present the classic features for both primary and secondary pathomorphosis.

Tinea faciei due to microsporum canis in children: a survey of 46 cases in the District of Cagliari (Italy).

Dermatophytoses are frequent in children, but involvement of the facial skin has peculiar aspects that should be considered a separate entity: tinea faciei. Microsporum canis infection in tinea faciei has not been widely documented. To review cases of tinea faciei due to M. canis in children diagnosed at the Dermatology Clinic, University of Cagliari. Between 1990 and 2009, all children with dermatophyte infections of the facial skin were recruited for the study after parental consent. Diagnosis was made through direct microscopic and cultural examination. Age, sex, clinical form, illness duration, identified dermatophyte, source of infection, and treatment were recorded. Forty-six cases of tinea faciei due to M. canis in children aged 11 months to 15 years (29 male/17 female) were diagnosed. In 42 (91.3%) children, the illness was the result of contact with pets, and 4 (8.7%) cases resulted from contact with children affected by tinea capitis due to M. canis. Clinical manifestations were typical ringworm in 34 (74%) patients, whereas in 12 (26%) cases, atypical forms mimicking atopic dermatitis, impetigo, lupus erythematosus, and periorificial dermatitis were observed. In 18 (39%) cases, involvement of the vellus hair follicle was documented as ectothrix invasion. Topical or systemic antifungal therapy was effective in all patients. Tinea faciei shows a complex spectrum of differential diagnosis and age-related variations with respect to other superficial dermatophytosis. M. canis is the main organism responsible in children residing in Cagliari, capitol city of Sardinia, Italy. Close collaboration with veterinary and educational programs within infant communities are required for adequate prevention.

Italian Euromelanoma Day Screening Campaign (2005-2007) and the planning of melanoma screening strategies.

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.

D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature.

Long term D-penicillamine (DPA) therapy to treat Wilson disease can induce elastosis perforans serpiginosa (EPS), a very rare degenerative skin disease characterized by a transepidermal elimination of elastic fiber aggregates. The iatrogenous disease depends on DPA capacity to chelate copper and cause its depletion. Lysyl-oxidase is a copper dependent enzyme crucial to the dermal elastic fiber cross-linking, which is strongly affected by DPA copper depletion. Direct binding of the drug to collagen precursors also affects elastic fiber assemblage and maturation. The abnormal elastin accumulates into the middle dermis and produces a characteristic bramble brush or "lumpy-bumpy" appearance. In this way it acts as a foreign body and is progressively extruded through the epidermis. Clinically, the disease presents with multiple firm keratotic papules and nodules arranged in annular plaques over the neck, axillae, antecubital fossae, and forearms. The rarity of the disease frequently causes misdiagnoses and the process continues unabated causing concerns about systemic elastopathy.

Treating psoriasis with etanercept in italian clinical practice: prescribing practices and duration of remission following discontinuation.

BACKGROUND: conventional antipsoriatic therapies are often administered until remission, with treatment resumed in the case of relapse, in order to reduce the likelihood of cumulative, dose-dependent toxicities. Biological agents have been safely used in continuous therapy. OBJECTIVE: to assess the use of etanercept for psoriasis in clinical practice in Italy. METHODS: this was an observational study carried out in 13 dermatological centres across Italy in patients with plaque psoriasis (with a Psoriasis Area and Severity Index [PASI] score >or=10) treated with etanercept. The study comprised a treatment and subsequent discontinuation period. Patients were eligible if they had plaque psoriasis and had begun treatment with etanercept between 1 September 2007 and 1 April 2008. Patients were evaluable for the duration of discontinuation analysis if they achieved a PASI reduction >or=50% (PASI50) and a PASI score <10 at the end of treatment. Etanercept treatment was restarted if the PASI score reached >or=10 or the patient had a clinical relapse. Data were collected retrospectively up to June 2008 and prospectively between July 2008 and January 2009. Patients received etanercept during the treatment period, followed by no etanercept treatment (other psoriasis treatment permitted) during the discontinuation period, and etanercept again during re-treatment. The main outcome measures were: PASI scores (type A responders: PASI reduction >or=75% [PASI75]; type B responders: PASI50 and PASI final score <10), Dermatology Life Quality Index (DLQI) scores and body surface area (BSA) involvement. Time from discontinuation to re-treatment was evaluated. Use of other antipsoriatic medications was recorded throughout. RESULTS: eighty-five patients were evaluable for the treatment period. Overall, 55 (64.7%) of these patients were prescribed etanercept 50 mg twice weekly. The mean treatment duration was approximately 25 weeks. In total, 79 patients (92.9%) were considered type B responders and 77 of these patients were evaluable for the duration of discontinuation analysis. Overall, 68/85 (80%) were type A responders. During the treatment period, 7/85 (8.2%) patients received other antipsoriatic therapies. Improvements in mean DLQI score (-71.5%) and mean BSA involvement (-79.2%) were also observed. Etanercept was well tolerated. During the discontinuation period, 40/77 (51.9%) patients used other antipsoriatic medications (group 1) and 37/77 (48.1%) did not (group 2). The mean duration of discontinuation was significantly longer in group 1 (174 days) than in group 2 (117 days, log-rank test: p = 0.0013). CONCLUSION: in clinical practice, the duration of discontinuation from etanercept was in accordance with previously reported data, and was longer in patients who received other antipsoriatic drugs during discontinuation of etanercept than in those who did not. High rates of PASI50 and PASI75 response were obtained with etanercept, and these rates were higher than those observed in controlled clinical studies. Etanercept treatment was flexible, effective and well tolerated, and was associated with improved quality of life.

Primary cutaneous cryptococcosis in an immunocompetent host.

We report a case of primary cutaneous cryptococcosis in an immunocompetent host. Several nodules, isolated or sometimes joint to form plaques, affected the right arm. The arm was paralytic and hypoplastic, and a history of numerous abrasions picking firewood up preceded the onset of the eruption. Histology on skin biopsy documented a dermal infiltrate constituted of histiocytes, lymphocytes, fibroblasts and rare giant cells. Numerous rounded periodic acid-Schiff (PAS) bodies were also present. Cryptococcus neoformans var. neoformans grew upon culture. Complete blood, biochemical and instrumental examinations resulted in findings within normal range. Treatment with itraconazole 200 mg daily for 4 months led to complete recovery. During a 2-year follow-up, the patient did not present any relapse or dissemination to other organs.

Two cases of primary endonasal leishmaniasis in Sardinia (Italy).

Leishmaniasis is an endemic protozoan infection in Sardinia, one of the major islands of the Mediterranean Basin. We report two cases of endonasal primary Leishmaniasis, which is a very rare event in adult men who are immunocompetent, born in, and residents of Sardinia. The diagnosis was confirmed by the presence of intra and extracellular Leishmania amastigotes in the histological smear. Isoenzymatic characterization identified Leishmania infantum zymodeme MON-111 in both cases. Laboratory and instrumental investigations excluded visceral involvement. Treatment with meglumine antimoniate (Glucantim) intralesional administration, 1 ml weekly for 4-5 weeks, led to complete resolution. The unusual location is likely a reflection an uncommon site of inoculation of the protozoa, transmitted by flying vectors. The patients were a shepherd and a farmer, respectively, both professions at high risk of infection because of their habits of sleeping outdoors under trees or in country cottages during spring and summer and exposed to sand fly bites. Although mucosal involvement and infection by Leishmania infantum, a potential cause of visceral leishmaniasis, the Sardinian patients experienced a benign disease course considering muco-cutaneous forms described in the New World. Differential diagnosis and early detection are necessary in order to start effective treatment and prevent more serious complications.

Bullous skin eruption in an HIV patient during antiretroviral drugs therapy.

Dermo-epidermal blistering is an uncommon presentation of adverse drug reactions. Several drugs are associated to such eruptions, but review of current knowledge does not list antiretroviral drugs. A 37-year-old Caucasian HIV-positive woman presented with a 6-week history of diffuse annular blistering affecting the trunk and limbs. Lesions appeared both on erythematous and normal-appearing skin. The patient was in treatment with antiretroviral (lamivudine + didanosine + nelfinavir) for 2 years. A history of previous adverse reactions to betalactams, nonsteroidal anti-inflammatory drugs, and a nevirapine-induced hepatitis was also referred. Histopathology showed a dermo-epidermal blister; direct immunofluorescence was positive for IgG, C3c at the basement membrane zone; enzyme-linked immunosorbent assay was positive for BP180 antigen. Oral prednisone 1 mg/kg daily for 20 days led to poor improvement. Discontinuation of the antiretrovirals was followed by a rapid healing. Blisters reappeared at first re-introduction essay 1 month later. Awareness of iatrogenic dermo-epidermal blistering is necessary to suspect the diagnosis and avoid long-term immunosuppressant treatment. Complete spontaneous recovery after withdrawal of the responsible drug and relapse at rechallenge are the main criteria for the diagnosis. Factors related to the state of the HIV infection, and/or immunodeficiency may have contributed in precipitating the reaction in the present authors' case.

Pityriasis rosea-like adverse reaction: review of the literature and experience of an Italian drug-surveillance center.

Pityriasis rosea is a common, acute eruption of uncertain etiology. A rash very similar to this idiopathic disease is also attributed to several drugs, and recovery, which depends on withdrawal of the responsible drug, can be delayed by its late identification. A prospective study to record all cases of adverse cutaneous reactions presenting with pityriasis rosea like manifestations was conducted at the center for drug-surveillance of the dermatology department of Cagliari University. We developed an intensive surveillance program from June 2002 to May 2005, adopting the WHO Collaborating center for Drug Monitoring causality assessment criteria and algorithm. Eight cases, six male and two female, were studied in a 3-year period. None had previously suffered from drug intolerance or allergy. Clinical manifestations were very similar to pityriasis rosea. Responsible drugs were mainly angiotensin-converting enzyme inhibitors, alone or in combination with hydrochlorothiazide, followed by one case each for hydrochlorothiazide plus sartan, allopurinol, nimesulide, acetyl salicylic acid. Recovery was obtained in all cases with drug withdrawal. Final causality assessment was probable for all eruptions. Frequency of drug pityriasis rosea-like eruptions is probably underreported. The mildness of the eruption, mimicking a very common and self-limiting disease does not prompt physicians to verify the use of medications until persistence, severity of lesions and itching require re-evaluation of the original diagnosis.

Cutaneous Crohn disease in a child.

Cutaneous Crohn disease refers to granulomatous skin manifestations not contiguous with gastrointestinal affected areas. It is a very rare condition, especially in children, and is easily misdiagnosed when characteristic gastrointestinal symptoms are absent. We report a 10-year-old Caucasian girl with a 6-month history of erythematous, firm tumescence of the left labium majus pudendi and moist vegetations circumscribing the anal ostium. Histologic analysis of skin biopsy specimens from both types of lesions showed a characteristic granulomatous noncaseating infiltrate throughout the dermis. Endoscopic examination and a colon biopsy specimen showed chronic granulomatous inflammation consistent with Crohn disease. Treatment with prednisolone 20 mg daily, metronidazole 250 mg three times daily, topical corticosteroids, and mupirocin ointment produced marked improvement of the vulvar edema, whereas the perianal lesion had a relapsing course. Early recognition of extra-intestinal manifestations of Crohn disease, which are extremely rare in children, may be difficult. Coexistence of contiguous and noncontiguous lesions, representing the very first signs of the disease, are further peculiarities in our patient.

Facial cellulitis associated with Pseudomonas aeruginosa complicating ophthalmic herpes zoster.

Cellulitis is a rare and severe soft-tissue infection characterized by acute, diffuse, spreading inflammation, often associated with systemic symptoms such as malaise and fever. Surgery of the head and neck, dental infections, sinusitis, upper respiratory tract infections, and trauma are the most common portal of entry for pathogens in facial cellulitis. A very unusual case complicating an ophthalmic herpes zoster in a 74-year-old woman was observed at the department of dermatology, Cagliari University (Italy). Culture of skin swabs showed growth of numerous Gram-negative bacilli, further identified as Pseudomonas aeruginosa. Therapy with intravenous ciprofloxacin was promptly instituted on the basis of the culture and sensitivity report. She was initially treated with daily drainage and twice-daily topical fusidic acid. The lesion completely resolved in 4 weeks, and no general complications or recurrence have been observed for 6 months. Early recognition and management of facial cellulitis is mandatory to avoid serious and generalized complications. Pseudomonas aeruginosa is rarely reported in facial cellulitis; there are apparently no reports of this infection occurring as a complication of ophthalmic herpes zoster. Herpetic damage of the anatomic barrier as well as impairment of defense mechanisms because of decompensated diabetes mellitus may have facilitated the colonization and proliferation of this opportunistic pathogen in our patient.

Tinea capitis caused by Microsporum canis treated with terbinafine.

Tinea capitis is a disease found throughout the world. It frequently affects children and only rarely adults, usually post-menopausal women. Numerous dermatophytes of the genus Microsporum and Tricophyton can cause tinea capitis and griseofulvin is still today the treatment of choice. To study the effectiveness and tolerability of terbinafine treatment in tinea capitis caused by Microsporum canis we treated 26 patients - 22 children and four women - for a period of 12 weeks. Dosage adopted was 62.5 mg day(-1) in patients weighing less than 20 kg, 125 mg day(-1) in those weighing between 20 and 40 kg, and 250 mg day(-1) in patients weighing more than 40 kg. Clinical and mycological healing was achieved in 22 patients (84.6%), tolerability was excellent and in no cases were side effects or abnormal results in blood chemistry tests observed.

Kerion Celsi in a newborn due to Microsporum canis.

The present study deals with a case of a 40-day-old girl with kerion Celsi caused by Microsporum canis. The source of the infection were the parents who presented tinea corporis caused by M. canis. Systemic treatment was carried out with terbinafine and complete recovery was achieved. Tinea capitis is unusual in children during their first year of life and its evolution towards kerion is very rare in newborns.

Nail pigmentation caused by hydroxyurea: report of 9 cases.

We report a series of 9 patients, 6 men and 3 women, who presented nail hyperpigmentation arising between 6 and 24 months from the start of hydroxyurea therapy. The most commonly observed clinical pattern was that of longitudinal melanonychia. In only 1 patient, who was affected in all 20 nails, we observed longitudinal melanonychia, diffuse melanonychia, and hyperpigmentation of the skin.

Case report. Pityriasis versicolor mimicking Pityriasis rotunda.

Pityriasis versicolor is a common dermatomycosis, occurring throughout the world, characterized by irregular, slightly scaly patches, varying in color from red/light brown to white. Pityriasis rotunda, on the other hand, is an uncommon disease, reported in specific ethnic groups, and characterized by perfectly round or oval patches of varying color, with a scaly surface. The histologic pattern is that of ichthyosis vulgaris. We report here the case of a male patient, aged 31, from Sardinia (Italy), affected by Pityriasis versicolor mimicking Pityriasis rotunda. Mycological examination allowed us to formulate the correct diagnosis, and ensuing treatment with antifungal drugs was entirely successful. The authors, while pointing out the rarity of this case, stress the possibility that Pityriasis versicolor mimics Pityriasis rotunda and vice-versa, especially in those countries in which the two diseases are endemic. More widespread recourse to microscopic examination can help avoid the risk of mistaken diagnosis and consequent incorrect treatment.