Scalp dysaesthesia and lichen simplex chronicus: diagnostic and therapeutic update with literature review.

Scalp dysaesthesia, considered a variant of the cutaneous dysaesthesia syndrome, is characterized by chronic sensory symptoms, including pruritus, pain, burning and stinging in a well-defined location, without objective findings. Its aetiology is not well elucidated and treatment options are limited, thus it can be challenging and frustrating for both patient and physician. It can be associated with lichen simplex chronicus. In this paper, we review the literature on the pathogenetic factors, diagnostic methods and therapeutic options in the management of scalp dysaesthesia. Dissociation, cervical spine disease and muscle tension seem to be the most important pathogenetic factors. Trichoscopy, reflectance confocal microscopy and biopsy are all helpful for the diagnosis of the disease. Therapies include high-potency topical or intralesional corticosteroids, capsaicin and topical anaesthetics, sedative antihistamines, tricyclic antidepressants, transcutaneous electric nerve stimulation, botulinum toxin and vitamin B12.

Hidradenitis suppurativa in a prepubertal case series: a call for specific guidelines.

Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease associated with a high physical and psychological burden. It is a disorder of the infundibular segment of the pilosebaceous unit, characterized by subcutaneous nodules, abscesses, sinus tracts and scar formation on the intertriginous and apocrine-bearing areas. HS is quite rare in young and prepubertal children. It usually begins after puberty, but several reports of prepubertal HS onset have been described. These cases are strongly linked to hormonal disorders and genetic susceptibility. Specific guidelines for prepubertal patients are still lacking, so further studies are warranted to better delineate a tailored approach. This paper aims to summarize the most significant aspects, as well as the most recent information about the epidemiology, pathogenesis, clinical features, diagnosis, comorbidities and treatment of paediatric HS. In addition, we report our clinical experience in managing HS in a group of eight prepubertal patients based on systemic antibiotics (azithromycin) and zinc oral supplementation.

Detection of D-penicillamine in skin lesions in a case of dermal elastosis after a previous long-term treatment for Wilson's disease.

BACKGROUND: Skin adverse events associated with D-Penicillamine (DPA) are common and multi-faceted, although the presence of DPA or its metabolites has never been documented in the skin, because of inherent difficulties in determining its tissue levels. Thus, the association between DPA and DPA-related dermatoses has been only hypothesized on the basis of careful history, clinical observation and typical histopathological findings. OBJECTIVE: To detect DPA in biopsy specimens in a unique case of 25-year-late-onset elastosis perforans serpiginosa and pseudo-pseudoxanthoma elasticum associated with a history of long-term high dose DPA, by applying a recently described analytical method to assess the presence of DPA in skin. METHODS: We used a reliable analytical method based on high-performance liquid chromatography coupled with amperometric detection to look for the presence of DPA in skin biopsy specimens. RESULTS: A chromatographic peak corresponding to DPA was evidenced in some affected skin samples collected from the patient. CONCLUSION: We documented the effective presence and the persistence after 25 years of DPA in the skin of a woman affected by elastotic cutaneous change due to a long-term therapy with DPA. This report provides further evidence of the relationship between DPA deposit in affected skin and clinical manifestation.

GH responsiveness before and after a 3-week multidisciplinary body weight reduction program associated with an incremental respiratory muscle endurance training in obese adolescents.

Several studies have demonstrated that the obesity-related hyposomatropism is usually reversible after a consistent weight loss induced by diet and/or bariatric surgery. Recently, a single bout of respiratory muscle endurance training (RMET) by means of a specific commercially available device (Spiro Tiger®) has been reported to induce a marked GH response in obese adults, its GH-releasing effect being significantly lower in obese adolescents. The GH response disappeared in both obese adults and adolescents when RMET was repeated at 2-h intervals in-between. The aim of the present study was to evaluate GH responses to repeated bouts of RMET administered before and after a 3-week in-hospital multidisciplinary body weight reduction program (entailing energy-restricted diet, 90 min/daily aerobic physical activity, psychological counseling, and nutritional education) combined with a progressively increasing RMET (15 daily sessions, 5 sessions per week) in 7 obese male adolescents [age: 12-17 years; body mass index (BMI): 38.5±3.1 kg/m2; percent fat mass (FM): 37.0±2.0%]. Blood samplings for GH determinations were collected during the 1st and 15th sessions, which were composed of 2 consecutive bouts of RMET (of identical intensity and duration) at 2-h interval in-between. At the beginning of the study, baseline GH levels significantly increased after the first bout of RMET in all subjects (p<0.05). The administration of the second bout of RMET resulted in a significantly lower (p<0.05) GH increase in comparison with the first one. Three weeks of the integrated intervention significantly reduced both body weight (from 115.3±9.2 kg to 111.5±8.7 kg, p<0.05) and FM (from 43.1±5.7 kg to 41.9±5.3 kg, p<0.05), these combined effects being, however, not sufficient to influence GH responsiveness to the 2 repeated bouts of RMET (GH peaks to the first bout: 4.8±1.6 ng/ml vs. 4.8±1.6 ng/ml; GH peaks to the second bout: 0.9±0.2 ng/ml vs. 1.1±0.1 ng/ml, before and after 3 weeks of the treatment, respectively, p=NS). In conclusion, a 3-week incremental RMET combined with a body weight reduction intervention does not seem useful to positively influence the reduced GH responsiveness to 2 repeated RMET bouts in obese adolescents. More intensive and/or long-term RMET protocols, associated with energy-restricted diets, determining more consistent changes in body composition, are likely needed to restore the impaired GH-IGF-1 function of obese adolescents.

Calciphylaxis in dialysis patients, a severe disease poorly responding to therapies: report of 4 cases.

Calciphylaxis or calciphic uremic arteriolopathy (CUA) is a rare syndrome characterized by the deposition of calcium within the walls of small and medium size vessels in the dermis and in the subcutaneous tissue. The disease mainly affects patients with end-stage renal disease. We report here our experience with 4 cases of calciphylaxis in dialysis patients. The main predisposing factor observed in our 4 patients was warfarin use (2 patients, 50%), while local traumas and diabetes were respectively present in only one patient. None of our patients was obese. Lower legs were the most frequently involved site of CUA (3/4 patients, 75%). In our experience biopsy was crucial to achieve a correct diagnosis and did not cause aggravation of the ulcers. Therapeutic approach was multimodal: mainly hyperbaric oxygen therapy, cinacalcet and sodium thiosulphate. Although many recent case reports have shown exceptional results and healing with the use of sodium thiosulphate, we did not experience any change in the poor prognosis of our patients with the use of this drug, at a dosage of 5 g thrice weekly endovenously.

GH responses to 2 consecutive bouts of respiratory muscle endurance training in obese adolescents and adults.

Repeated bouts of GH-releasing stimuli (both pharmacological and physiological, such as aerobic exercise) at 2-h intervals are associated with a blunting of somatotropic responsiveness in normal adults, while a persistent GH responsiveness to consecutive stimuli is reported to occur in children and adolescents. Recently, a single bout of respiratory muscle endurance training (RMET) by means of a specific commercially available device (Spiro Tiger®) has been shown to induce relevant GH responses in both normal-weighted and obese adult subjects. The aim of the present study was to evaluate GH responses to repeated bouts of RMET in obese adolescents and adults. Seven obese male adolescents (age: 15.7±0.4 years; body mass index, BMI: 38.0±3.3 kg/m2) and 10 obese adults (age: 22.2±1.4 years; BMI: 39.9±1.0 kg/m2) underwent an incremental progressive RMET protocol of 11 daily sessions. Blood samplings for GH determinations were collected during the 12th session, which was composed of 2 consecutive bouts of RMET (of identical intensity and duration: 1 min at a respiration rate of 28 acts/min, 5 min at 32 acts/min, 5 min at 34 acts/min, 4 min at 36 acts/min) at a 2-h interval in-between. Baseline GH levels significantly increased after the first bout of RMET in all subjects, higher GH peaks being found in obese adults than in obese adolescents (peaks: 14.3±2.1 ng/ml vs. 4.8±1.6 ng/ml, respectively, p<0.05). The administration of the second bout of RMET resulted in significantly lower (p<0.05) GH increases in both obese adolescents and obese adults (peaks: 0.9±0.2 ng/ml and 1.6±0.2 ng/ml, respectively) in comparison with the first one. In conclusion, exercise protocols based on repeated bouts of RMET do not seem a valid strategy to persistently stimulate GH-IGF-1 release in obese adolescents, since GH responses to a single bout are actually modest in comparison with those of obese adults and completely abolished after repeated bouts at 2 h interval in-between.

GH and cortisol responses following an acute session of respiratory muscle endurance training in severely obese patients.

It is well established that obese patients are hypo-responsive to classical GH-releasing stimuli, including aerobic exercise. Recently, we have demonstrated that whole body vibration was able to markedly stimulate GH secretion in obese patients, thus suggesting that this refractoriness is not absolute but dependent on the GH-releasing stimulus. Furthermore, we have shown the ability of a respiratory muscle endurance training (RMET) to stimulate GH and cortisol secretion in healthy subjects. The objective of this study was to evaluate the effects of RMET on GH and cortisol responses in severely obese patients. Eight severely obese patients (4 M/4 F, mean age±SEM: 22.8±1.6 years, body mass index, BMI: 39.9±1.1 kg/m2) underwent an incremental progressive RMET protocol of 11 daily sessions, obtained through the use of a specifically designed respiratory device (Spiro Tiger®). The 12th session of RMET (15 min duration: 1 min at a respiration rate of 28 acts/min, 5 min at 32 acts/min, 5 min at 34 acts/min, 4 min at 36 acts/min) was associated with blood samplings for determination of GH, cortisol, and lactate (LA) levels. An age- and sex-matched normal-weighted control group (n=7, 4 M/3 F, age: 26.1±3.1 years, BMI: 22.4±0.6 kg/m2) was also recruited. In both normal-weighted subjects and obese patients, GH secretion significantly increased after a 15-min RMET session. Although serum GH levels at 30 min were higher in normal-weighted subjects than in obese patients, there was no statistically significant difference in either GH peaks or net GH areas under the curve between the 2 groups. RMET significantly increased serum cortisol levels in normal-weighted subjects, but was associated to a progressive cortisol decline in obese patients. RMET stimulated LA production, with no significant differences in normal-weighted subjects and in obese patients. A 15-min RMET session was capable to induce a GH response in severely obese patients, which was comparable to that recorded in normal-weighted subjects. A progressive decline in serum cortisol levels occurred in obese patients after RMET, while an opposite pattern (i. e., a significant cortisol increase) was found in normal-weighted subjects. Optimization of long-term RMET protocols could represent a valid strategy to (physiologically) stimulate GH/IGF-I system in those GH hyposecretory states such as obesity.

GH responses to two consecutive bouts of respiratory muscle endurance training in healthy adults.

Repetition of voluntary exercise bouts and of different pharmacological GH-releasing stimuli at 2-h intervals is associated with a complete abolishment of GH responsiveness. By contrast, a different pattern is observed after repeated neuromuscular electrostimulation, which is characterized by preservation of GH responsiveness. Aim of the study was to evaluate GH responses to repeated bouts of respiratory muscle endurance training (RMET) by mean of a specific commercially available device (Spiro Tiger®). Eight healthy men underwent an incremental progressive RMET protocol of 11 daily sessions. Blood samplings for GH, cortisol and lactate (LA) determinations were collected during the 12th session, which was composed of two consecutive bouts of RMET (of identical intensity and duration: 1 min at a respiration rate of 28 acts/min, 5 min at 32 acts/min, 5 min at 34 acts/min, 4 min at 36 acts/min) at a 2 h interval. Baseline GH levels (mean: 0.9±0.4 ng/ml) significantly (p<0.01) increased after the first bout of RMET (peak: 15.7±4.0 ng/ml). The administration of the second bout of RMET resulted in a significantly lower (p<0.05) GH increase (peak: 3.9±0.8 ng/ml) in comparison with the first one. Baseline LA levels (mean: 1.2±0.1 mmol/l) significantly increased (p<0.001) after the first bout of RMET (peak: 2.3±0.2 mmol/l). The administration of the second RMET bout resulted in a comparable LA increase (from a basal value of 1.2±0.1 mmol/l up to a peak of 2.0±0.1 mmol/l, p<0.001). The first bout of RMET caused a significant increase of cortisol levels (p<0.01), starting from a basal mean value of 142.9±9.4 ng/ml up to a peak of 188.8±10.3 ng/ml. By contrast, the second bout of RMET did not induce any significant change of cortisol levels (basal: 149.1±9.0 ng/ml, peak: 168.5±5.1 ng/ml). In conclusion, a single bout of RMET is capable of stimulating GH and cortisol secretions and LA production. When a second bout is repeated after 2 h, there is a blunting of GH and cortisol responses with a preservation of LA release. Further studies are needed to schedule long-term RMET protocols capable of persistently stimulating GH-IGF-I release and to maximally enhance the ergogenic and metabolic benefits of this intervention either in normal subjects (e.g. athletes) or patients with an impairment of motor capabilities requested to perform normal daily activities (i.e. severely obese and elderly people).

Efficacy and tolerability of a cream containing AR-GG27® (sorbityl furfural palmitate) in the treatment of mild/moderate childhood atopic dermatitis associated with pityriasis alba. A double-blind, placebo-controlled clinical trial.

AIM: Pityriasis alba (PA) is a skin disorder characterized by finely scaly, hypopigmented patches, typical of childhood, that also represents an atopic dermatitis (AD) minor sign according to Hanifin and Rajka criteria. It may be isolated or associated with AD representing, sometimes an atypical manifestation of AD during the long-term follow-up of the disease. Aim of the study was to evaluate of the efficacy and tolerability of AR-GG27® (sorbityl furfural palmitate) cream in the treatment of childhood mild or moderate AD associated with PA. METHODS: The trial is a single center, double-blind, randomized, placebo-controlled study. The study included patients of both sexes, aged between two months and 15 years, suffering from mild and moderate AD always associated with PA. Xerosis was present in all patients. The treatment with topical steroids or topical calcineurin inhibitors (TIMs) had to be suspended for at least 15 days. Any systemic therapy and phototherapy or sun exposure were withdrawn at least 30 days before. Emollients were stopped at least seven days before. During the trial, no other local or systemic treatments were allowed, as well as sun exposure. Patients affected by AD with viral, bacterial or fungal overinfection or patients with diabetes mellitus, severe systemic diseases or intolerance to one or more components of the product were excluded. The primary endpoint was the evaluation of the average change in the Investigator Global Assessment (IGA) after 15 and 30 days of treatment. The second endpoint was the evaluation of severity of three different clinical signs: erythema, excoriation desquamation, using a subjective five-point scale. Changes in pruritus severity was also considered during the entire period of treatment, through the use of a Visual Analogue Scale (VAS). A P<0.05, two tailed was considered as statistically significant. RESULTS: After 15 and 30 days there was a statistically significant difference in the group treated with AR-GG27®, compared to the placebo (respectively, P=0.0007 and P=0.005). After 15 days of treatment, itching was clearly reduced in AR-GG27® treated group compared with the placebo, both in the study population (P=0.01) and in patients where the symptom was present from the beginning (P=0.05). CONCLUSION: AR-GG27® showed a beneficial action associated with high compliance and tolerability in dermatological skin conditions characterized by inflammation and tissue oxidative stress in children, as PA with mild and moderate AD.

Growth hormone response induced by a respiratory muscle endurance training in healthy subjects.

To date, the large majority of studies evaluating growth hormone (GH) response to acute physical exercise has been performed involving gross muscle groups. To the best of our knowledge, none has evaluated the effects of a respiratory muscle endurance training (RMET) on hormonal secretions, particularly on GH release, though some respiratory devices have been widely used in athletes to train respiratory muscles and to improve cardiopulmonary function and physical performance. 8 healthy men underwent an incremental progressive RMET protocol of 11 daily sessions, obtained through the use of a specifically designed respiratory device (Spiro Tiger®). The 12th session of RMET (15 min duration: 1 min at a respiration rate of 28 acts/min, 5 min at 32 acts/min, 5 min at 34 acts/min, 4 min at 36 acts/min) was associated with blood samplings for determination of GH, cortisol, ghrelin, glucose, and lactate (LA) levels. GH and cortisol responses significantly increased after a 15-minute RMET session, which, in contrast, inhibited ghrelin secretion. There was a minimal, though significant, increase in LA levels with a significant elevation in glycemia. A 15-minute RMET session, administered after a 11-days incremental progressive RMET protocol, was capable of stimulating GH and cortisol release and suppressing ghrelin secretion. Optimization of incremental progressive RMET protocols would be important to maximize the positive chronic effects of this intervention on somatotropic function and muscle performance.

Correlation between BMI and PASI in patients affected by moderate to severe psoriasis undergoing biological therapy.

Obesity is common in psoriatic patients, and it has been shown to be important for many aspects of the condition. In particular, low-calorie diets can improve the symptoms and response to treatment in pustular psoriasis. The present study investigates the influence of body-weight alteration on the disease's clinical manifestations in moderate to severe psoriasis patients treated with biological drugs. Finally, the influence of a caloric restriction was assessed. This observational transversal study enrolled 33 patients attending our Severe Psoriasis Outpatient Clinic, who were treated with biological drugs. Body Mass Index (BMI) was used as a diagnostic indicator of being overweight and of obesity. Waist circumference was also measured. Body weight and Psoriasis Area Severity Index (PASI) index were measured at follow-up visits at 4 and 8 months. Nonparametric test of Mann-Whitney was used to detect the differences between patient groups. Fisher's exact test was performed to evaluate the different results depending on the therapeutic changes of BMI. There was a strong prevalence of overweight-obese individuals in the group with a mean BMI of 30.59 +/- 6.94. Waist circumference was also above normal in the majority of the patients. Obese patients had a PASI index higher than the average of the whole group (25.03 +/- 12.43), with grade III obese patients having an average PASI of 44 +/- 3.37. At the first and second follow-ups, patients who put on weight did not achieve PASI 50; patients who had a stable weight presented variable response to treatment, while patients who decreased their weight achieved PASI 90 or PASI 75 even when not responding at the first. Further studies are needed to understand if the poor response observed in heavier patients is due to biological drugs pharmacokinetics or because therapy should be BMI based rather than administered in fixed doses, posing then an ethical consideration.

Atopiclair.

BACKGROUND: MAS063DP cream has received marketing authorization in the USA and the EU as a medical device for the relief of symptoms of atopic dermatitis (AD) and contact dermatitis. OBJECTIVE: Review of technical and clinical data in the literature on MAS063DP to evaluate the role that MAS063DP (Sinclair Pharmaceuticals Ltd) holds in the management of patients affected by AD. METHODS: We explored the characteristics and efficacy of the product by a wide-ranging search of the medical and scientific studies in the literature and drew our conclusions also on the basis of our medical practice and knowledge concerning the treatment guidelines and therapy options of AD. CONCLUSION: MAS063DP is a product dermatologists could enroll in the treatment of signs and symptoms of mild-to-moderate AD in adult and pediatric patients.

The effect of gamma-linolenic acid on clinical status, red cell fatty acid composition and membrane microviscosity in infants with atopic dermatitis.

A double blind placebo-controlled study of two doses of gamma-linolenic acid, provided by evening primrose oil (EPO, Epogam, Searle, U.K.), in children with atopic dermatitis was performed: 1) to examine the effect of gamma-linolenic acid administration on the clinical status of children with atopic dermatitis and abnormalities of IgE-mediated immune responses compared to those without such IgE abnormalities; 2) to investigate the effect of gamma-linolenic acid on red cell fatty acid composition and 3) to assess whether treatment with gamma-linolenic acid induced changes in red cell membrane microviscosity. A significant improvement in the overall severity of the clinical condition was seen in children treated with gamma-linolenic acid, independent of whether the children had manifestations of IgE-mediated allergy. Furthermore, gamma-linolenic acid treatment increased the percentage content of n-6 fatty acids in erythrocyte cell membrane; this increase was more marked in the membranes of children treated with high doses of EPO. In the high dose group a significant increase in dihomogamma-linolenic acid (DGLA) occurred. This may be of particular relevance because of the potential importance of DGLA as a precursor of antiinflammatory prostanoids. Red cell membrane microviscosity did not change in any group after treatment with EPO, even in high doses, despite a significant increase in the proportion of long chain polyunsaturated fatty acids.

Evening primrose oil (Efamol) in the treatment of children with atopic eczema.

It has been reported that essential fatty acid levels may be low and that there may be reduced levels of delta-6-desaturase metabolites of linoleic acid in patients with atopic eczema. Good therapeutic results have been reported on the use of evening primrose oil (Efamol) in adults but not in children. Efamol contains gamma-linolenic acid, the delta-6-desaturase metabolite of linoleic acid. The authors have studied 24 children with atopic eczema: 12 of them were treated with a higher dose of evening primrose oil than in previous studies and 12 with placebo olive oil. The clinical status and plasma, neutrophil and lymphocyte fatty acid composition in these children have been evaluated. After 4 weeks the eczema of essential fatty acid-treated children significantly improved in comparison with that of placebo-treated children (p less than 0.01). There were significant changes in plasma fatty acid composition between the basal values and the end of active treatment, and between the placebo and actively treated children. Neutrophil and lymphocyte fatty acid composition did not seem to be related to disease activity.

A long-term study on the use of evening primrose oil (Efamol) in atopic children.

The effect of essential fatty acids on atopic eczema is controversial. Some workers have reported that patients with atopic eczema improved following oral treatment with evening primrose oil (an oil with a high concentration of gamma-linolenic acid), but others have disputed this. This study was designed to look at the effect of evening primrose oil as a long-term oral supplementation for children with atopic eczema. Treated children dramatically improved their clinical condition after 4 weeks of therapy, and this improvement was maintained during the whole period of treatment (20 weeks). At the same time, modifications in plasma, neutrophil and lymphocyte fatty acid composition were detected.