Ulcerative necrobiosis lipoidica: case report of an atypical presentation and literature review.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous degenerative skin disease by unknown causes, which is mostly associated with diabetes mellitus, usually presenting with typical plaques of the shins. Although less common, some ulcerative forms may be seen in clinical practice. The occurrence of an atypical presentation in one of our patients was the occasion to review the pertinent literature.

Real-life experience on effectiveness and safety of dupilumab in adult patients with moderate-to-severe atopic dermatitis.

BACKGROUND: Dupilumab, a fully human monoclonal antibody targeting the alpha subunit of IL-4 was recently approved for the treatment of moderate-to-severe atopic dermatitis (AD) in adult patients. OBJECTIVE: To assess dupilumab effectiveness and safety in adults with moderate-to-severe AD in a real-life Italian multicentre retrospective cohort. METHODS: Adult moderate-to-severe AD patients, referring to 39 Italian centers, received dupilumab in the context of a national patient access program. Disease assessment was performed at baseline, after 4 and 16 weeks of treatment using Eczema-Area-and-Severity-Index (EASI) score, itch and sleep numerical-rating-score (itch-NRS, sleep-NRS) and Dermatology-Life-Quality-Index (DLQI). RESULTS: A total of 109 (71 M/38F) patients was studied. There was a significant reduction in EASI score, itch-NRS, sleep-NRS and DLQI from baseline to week 4 and a further significant decline to week 16. EASI 50, EASI75 and EASI90 were achieved by 59.6%, 28.4% and 9.3% of patients at 4 weeks and by 87.2%, 60.6% and 32.4% of them at 16 weeks, respectively. Adverse events were experienced by 19.2% (21/109) of the patients and they were all mild in intensity, being conjunctivitis the most common side effect. CONCLUSIONS: Dupilumab significantly improved disease severity, pruritus, sleep loss and quality of life with an acceptable safety profile.

Effectiveness of platelet-rich plasma in healing necrobiosis lipoidica diabeticorum ulcers.

Necrobiosis lipoidica diabeticorum (NLD) is a chronic condition, which is characterized by single or multiple lesions on the legs, and occurs in 0.3% of patients with diabetes. Recently, platelet-rich plasma (PRP) has been used in several clinical settings to promote sustained healing and better outcome. We propose the use of PRP for the treatment of NLD-associated recalcitrant wounds. In this study, 15 patients were treated with homologous PRP; all showed marked enhancement in wound healing without any adverse effects. After PRP application, advanced dressings were used until closure of the lesion was obtained, which was complete for all our patients. We therefore propose the use of PRP for recalcitrant wounds in patients with NLD.

[Looking for "lost occupational cancers": a systematic evaluation of occupational exposure in a case series of cutaneous squamous cell carcinomas in Italy]. / Alla ricerca dei "tumori professionali perduti": valutazione sistematica dell'esposizione professionale in una serie consecutiva di carcinomi cutanei spinocellulari.

BACKGROUND: Cutaneous carcinomas are tumors with a potential occupational etiology due to exposure to established carcinogens such as polycyclic aromatic hydrocarbons (PAH), ionizing radiation, ultraviolet (UV) radiation and arsenic. The occupational origin of such neoplasms is hugely underestimated in Italy. OBJECTIVES: To asses the proportion of Squamous Cell Carcinomas (SCC) cases with a previous occupational exposure to carcinogens. METHODS: We systematically evaluated occupational exposure in a series of consecutive cases, discharged in the period 2010-11 from the Dermatology Unit of Varese Hospital, Italy, with a histological diagnosis of SCC. Through a structured telephone interview we identified patients with a potential exposure to skin carcinogens. As a second-level step, an extensive evaluation by an occupational physician was performed to assess the occupational etiology in those selected cases. RESULTS: 105 patients were identified (65 men). 15 male cases out of a total of 85 patients who did the telephone interview, revealed a potential occupational exposure; 7 cases were confirmed as occupational cancers after second-level evaluation (proportion of male occupational cases = 13.2%). UV radiation and PAH were recognized as major causal agents. Applying those results to the national incidence data, we estimated a number of 700 annual occupational cases, 100-fold more than the cases currently evaluated by the Italian National Workers Compensation Authority. CONCLUSIONS: Our results revealed that occupational SCC is still at present a substantially "lost disease" in Italy. Greater attention and enhanced collaboration between specialists is thus needed to overcome this tendency.

Lyell syndrome due to ticlopidine.

The antiplatelets drug ticlopidine is widely used in patients suffering from cardiomyopathies, coronary cardiopathies and thromboembolic pathologies. The potential and frequently seen side effects are diarrhoea, hematological dyscrasias and rashes. We describe a case in which the use of ticlopidine in the treatment and prophylaxis of transit ischemic attacks triggered the unusual and rare complication of toxic epidermal necrolysis (TEN). We will then go on to analyse the clinical-pathological aspects and therapies through the use of i.v. immunoglobulins at high dosages that led to a full recovery of the patient.

Calciphylaxis: a case report.

Calciphylaxis or calcific uremic arteriolopathy is characterized by the involvement of the tunica elastica of the reticular or deep subcutaneous dermis, with extravascular and vascular thrombosis calcifications that lead to tissular ischemia. The torso muscles, the lumbar region, and lower limbs are more frequently affected. The pathogenesis is unknown. Calciphylaxis is associated to hyperparathyroidism, chronic renal failure, and diabetes mellitus. The best therapy to date is prevention: early treatment of renal failure, performing a partial parathyroidectomy where necessary, surgical debridement of the necrotic tissue, as well as avoiding the trigger factors such as systemic corticosteroids.

Allergy and the skin.

Allergic skin disorders include urticaria, angioedema, contact dermatitis and atopic dermatitis, but the model fitting most closely the systemic concept of allergy is atopic dermatitis (AD), the pathogenesis of which is linked to a complex interaction between skin barrier dysfunction and environmental factors such as allergens and microbes. In particular, an important advance was the demonstration that the mutation of the skin barrier protein filaggrin is related strictly to allergen sensitization and to the development of asthma in subjects with AD. The altered skin barrier function, caused by several factors, results in the passage of allergens through the skin and to systemic responses. A pivotal role in such a response is exerted by Langerhans cells which, via their immunoglobulin E (IgE) receptor, capture the allergens and present them to T cells. When T helper type 2 (Th2) cells are activated, the production of a proinflammatory cytokines and chemokines pattern sustains the persistence of inflammation. Known AD-related cytokines are interleukin (IL)-5, IL-13 and tumour necrosis factor (TNF)-alpha, with emerging importance for IL-17, which seems to drive airway inflammation following cutaneous exposure to antigens, and IL-31, which is expressed primarily in skin-homing Th2 cells. Skin-homing is another crucial event in AD, mediated by the cutaneous lymphocyte-associated antigens (CLA) receptor, which characterizes T cell subpopulations with different roles in AD and asthma.

Ekbom syndrome: a disease between dermatology and psychiatry.

The Ekbom syndrome or parasitosis delirium represents a rare mono-symptomatic psychosis characterized by the delirious firm belief of the patient, against all evidence, of being infested by cutaneous parasites. The syndrome affects in particular middle-aged women, and can be the single manifestation of psychological uneasiness or represent one of the aspects of a more complex psychiatric case, compromising almost totally any normal daily work and/or social activity. The authors present a recently observed clinical case and discuss the clinical-diagnostic and therapeutic aspects.

Treatment of allergic diseases during pregnancy.

Pregnancy may variously modify the natural history of allergic disorders through occurring endocrinologic, functional and immunological changes. A pharmacologic treatment of allergic diseases (mainly asthma) is often necessary during pregnancy. On the other hand, a drug should be not potentially teratogenic and should not have serious side effects, both for the mother and the fetus. This paper reviews current knowledge about allergic diseases during pregnancy, considering the points of view of the different specialists involved in their management. Topical mucosal agents seem to be the safest, due to their minimal or absent absorption which should reflect reduced side effects. Preferred agents should be topical antihistamines (for rhinitis and conjunctivitis), and cromones and topical steroids (for asthma), as they are both safe and effective.

Statistical evaluation of the persistence of acquired hypersensitivity by standardized patch tests.

Numerous studies have focused attention on the influence of various biological and environmental factors on contact hypersensitivity. In order to evaluate the persistence and/or modification of allergic contact sensitivity to a number of common contact sensitizers, the same standardized patch tests were repeated on 174 subjects with contact sensitivity after a time lapse of 5 years (1987-1992). In 18.4% of the cases, 1 or more sensitivities were lost; 28.7% of the patients had a higher number of positive patch tests after 5 years, while the remaining 52.9% of the patients showed no change in the number of positive patch tests. In 88%, the positive allergens were unchanged, whereas in the remaining 12% of the subjects, they showed 1 or more variations. The association between the allergens most often positive was calculated for both the 1st and the 2nd patch test results. Moreover, to evaluate the frequency of an allergen's positivity, we studied the disappearance of old sensitivities and the appearance of new sensitivities by the McNemar test. Cobalt chloride was the only allergen with a significant frequency of new positivities over the period of observation (p < 0.01). Logistic regression analysis was performed to evaluate the possible influence of positive tests to other allergens, and of some clinical findings associated with contact dermatitis, on the sensitivity to cobalt chloride in 1987 and in 1992.

Sensitization to reactive textile dyes in patients with contact dermatitis.

Reactive dyes are used especially for colouring natural fibres (cotton, silk and wool) that are widely used in Western countries, particularly Italy, in the production of clothes. The aim of our study was to investigate sensitization to the most commonly used reactive textile dyes in patients undergoing patch tests, and to assess the clinical relevance of contact sensitization to these dyes. 1813 consecutive patients underwent patch tests with the GIRDCA standard series and an additional textile series of 12 reactive dyes. 18 of these patients were sensitized to reactive dyes (0.99%) (4 only to reactive dyes). The dyes most frequently responsible for positive patch tests were Red Cibacron CR and Violet Remazol 5R (respectively, 8 and 5 positivities). In 5 cases only was a history of intolerance to particular garments given; of 4 patch tests performed with pieces of garment, 2 were positive. In 1 occupationally-exposed patient, airborne contact dermatitis was suspected. Owing to the lack of up-to-date patch test series, some cases of allergic contact dermatitis from textile dyes are probably misdiagnosed: new colouring agents are continuously introduced to the market, so that a close relationship with textile industry is necessary to improve our diagnostic tools.

Pre-treatment of nickel test areas with sodium lauryl sulfate detects nickel sensitivity in subjects reacting negatively to routinely performed patch tests.

A fair % of patients with a clinical history of nickel allergy show negative patch test results. To improve the response rate to NiSO4 5% pet. patch tests, a testing procedure utilizing pre-treatment of the test area by a 24-h application of sodium lauryl sulfate (SLS) was introduced. 46 women with a clinical history of nickel sensitivity, who exhibited negative reactions to nickel sulfate 5% pet. patch tests, were studied. Patients underwent 6 patch tests on adjacent sites on the volar surface of the forearms. 4 patch tests were performed with a 72-h application of 40 mg nickel sulfate 5% pet. While 1 of these patch tests served as control, 3 test areas underwent 24-h pre-treatment with 40 microliters SLS, 1 with 0.1% and 2 with 0.5% solution. To evaluate differences in the reactivity to SLS plus nickel sulfate related to the site on the forearm, 0.5% SLS pre-treatment was performed both on a proximal and on a distal test site. At the 72-h evaluation, 19 subjects out of 46 showed positive reactions to nickel sulfate 5% pet. at skin sites pre-treated with 0.1% SLS, whereas 23 patients reacted positively at 0.5% SLS pre-treated areas. Echographic values of skin thickness and of hypo-echogenic dermal areas at positive pre-treated nickel test areas were higher than at control test areas, confirming the clinical evidence of an increased response to NiSO4 after SLS pre-treatment. The inflammatory reaction, as evaluated clinically and echographically, was much higher at distal skin areas (0.1% SLS and distal 0.5% SLS) than at proximal 0.5% SLS ones.

Contact allergy to Dermatophagoides in atopic dermatitis patients and healthy subjects.

To compare different house-dust-mite-derived allergenic materials and to correlate the presence of IgE to Dermatophagoides with patch test results, 313 atopic dermatitis (AD) patients and 100 healthy volunteers (HV) underwent patch tests with: Dermatophagoides pteronyssinus (DPT) lyophilized purified alpha fraction in buffered saline/glycerol 50% and/or in petrolatum (Bayropharm); 50% DPT and 50% Dermatophagoides farinae (DF) whole bodies in petrolatum and petrolatum oil (Allergopharma-Bracco); DPT and DF whole bodies in petrolatum and petrolatum oil (Lofarma). We found 39% positive reactions among AD subjects and 13% in HV. The presence of serum-specific IgE did not influence the patch test results. 38% of AD patch-test-positive patients and 4 of 13 HV, respectively, showed a positive prick test and/or RAST to Dermatophagoides. Similar sensitization rates were observed with the allergenic material from Bayropharm (54% positives) and Allergopharma-Bracco (51% positives), whereas the preparations from Lofarma gave a 20% response rate.