Successful treatment of severe recalcitrant vernal keratoconjunctivitis and atopic dermatitis associated with elevated IgE levels with omalizumab.

We report the clinical case of an atopic patient with important manifestations impacting the quality of life at the cutaneous and ocular site. The condition resisted conventional treatments, and omalizumab had to be used to treat the disease successfully.

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.

Comparison of two different neonatal skin care practices and their influence on transepidermal water loss in healthy newborns within first 10 days of life.

AIM: Physiologic post-partum skin adaptation to the relative dry extra-uterine environment is a dynamic process which begins immediately after birth. Considering the differences from adult skin, the neonatal skin is more prone to damage by environmental factors; therefore, skin care regimens should be age adapted to ensure a good epidermal maturation. The effects of two different skin care practices were evaluated by transepidermal water loss (TEWL) measurement in 94 newborns aged ≤ 10 days: group 1 (G1), newborns washed only with a cotton washcloth moistened with water; group 2 (G2), newborns washed with liquid baby cleansers and hydrated with moisturizers. These recordings were compared to TEWL baseline values of the same neonates and to adults' values. METHODS: A prospective study was conducted in healthy full-term newborns, measuring TEWL with TEWAMETER® TM300. The areas tested were the volar forearm and the popliteal fossa. RESULTS: In G1 (52 subjects), TEWL mean values were 6.65 ± 2.81 SD (g/m2/h) at volar forearm and 7.49 ± 2.47 SD (g/m2/h) at popliteal fossa. In G2 (42 subjects), TEWL mean values were 8.83 ± 3.05 SD (g/m2/h) at volar forearm and 10.18 ± 3.64 SD (g/m2/h) at popliteal fossa. There were statistically significant differences of TEWL mean values between G1 and G2, newborns and adults, baseline and post-skin care procedures. CONCLUSION: Tested skin care regimens could influence the process of functional adaptation of skin, in the early postnatal period. We could hypothesize that daily washing with liquid baby cleansers and moisturizing may delay the natural maturation of skin barrier function.

A PHACES syndrome unmasked by propranolol interruption in a tetralogy of Fallot patient: case report and extensive review on new indications of beta blockers.

Infantile hemangiomas (IHs) are the most common benign tumors of infancy and usually they don't require specific therapy. In 10-20% of cases IHs are able to generate complication and medical/surgical intervention is needed. For many decades standard treatment consisted in oral or intralesional corticosteroids until Leaute-Labreze and colleagues published the first report on the efficacy of propranolol for cutaneous infantile hemangiomas in 2008. IHs can be sometimes part of complex syndrome. Here we report the case of a patient with tetralogy of Fallot operated at 5 month of age who stopped propranolol treatment for hypoxic spells and unusually developed facial and subglottic IHs configuring the diagnosis of PHACES syndrome (posterior fossa brain malformations, hemangioma, arterial anomalies, cardiac defects and/or aortic coarctation, ocular anomalies and sternal defects). To our knowledge this is the first report in the international literature of a delayed appearance of an infantile hemangioma involving the skin and the airways (PHACES syndrome). The pathophysiological explanation relies on the mechanism of action of propranolol which seems to act initially with vasoconstriction, down-regulating proangiogenetic factors and inducing endothelial cell apoptosis. Many decades since their introduction ß-blockers are useful in a growing group of diseases. The pleiotropic effect of ß-adrenoceptors antagonists is not yet deeply understood, residing in neurohormonal regulation systems and angiogenesis and proving to be an effective treatment from cardiovascular to oncological illnesses.

Generalized morphea successfully treated with extracorporeal photochemotherapy (ECP).

A patient is presented with generalized morphea whose disease completely resolved after combination therapy with extracorporeal photopheresis and broad band UVA treatments.

Unilesional folliculotropic mycosis fungoides: a true rarity in the clinico-pathologic spectrum?

AIM: Unilesional mycosis fungoides (UMF) was firstly described in 1981 as solitary lesion with clinical and histological features of MF. Although over 100 cases have been reported in the literature, there is a lack of clear-cut criteria characterising UMF. Only 10 cases featured by follicolotropism of the neoplastic T-cells have been reported: the so-called unilesional folliculotropic MF (UFMF). This paper questions whether or not UFMF should be considered as a true rarity in MF clinico-pathological spectrum. METHODS: We retrieved 28 folliculotropic MF cases in the database of the Dermatological Divisions of Bologna (12 patients) and Florence University (16 patients). Four of them were UFMF patients (2 males and 2 females, mean age 45 years; median age: 39 years). RESULTS: All patients achieved after therapy disease complete remission. Notably, only one patient was treated with radiotherapy, that seems the most recommended strategy in UMF. For the remaining patients, we choose different managements in order to achieve both clinical efficacy and the best aesthetical outcome. CONCLUSION: No definitive conclusions can be drawn whether or not UFMF has the same indolent clinical course of UMF. Recently, Kempf et al. reported 2 UFMF patients with progression to tumour stage and large-cell transformation, respectively. UFMF in our database is 14.3% of the 28 FMF cases. Our data suggest that UFMF can be regarded as a true rarity in MF clinico-pathological spectrum.

Association of halo nevus/i and vitiligo in childhood: a retrospective observational study.

BACKGROUND: Although halo nevus (HN) is frequently observed, the relationship between vitiligo and HN in children has rarely been investigated. OBJECTIVES: To investigate the association between HN and vitiligo in children and understand if HN/HNi might be a risk factor for vitiligo. METHODS: Ninety-eight patients with only HN/HNi and 27 with HN/HNi and vitiligo were investigated for number and localization of HN/HNi, family history for HN/HNi and vitiligo and personal and family history for autoimmune or other diseases. A follow-up telephone interview was performed to investigate the evolution of HN/HNi and the possible onset of vitiligo and/or other diseases. RESULTS: In the HN/HNi and vitiligo group, HN/HNi and vitiligo had started almost simultaneously in 11 children; in nine, the onset of HN/HNi was followed by vitiligo after 6 months to 5 years; seven patients presented vitiligo first and HN/HNi after 3-9 years. Patients with associated vitiligo had, significantly more often, multiple HNi and a positive personal and/or family history of autoimmune thyroiditis compared with those with only HN/HNi. Follow-up longer than 5 years was available in 54/98 patients with only HN/HNi; two of them, both with multiple HNi, developed vitiligo. After follow-up, multiple HNi were noticed in 18/52 patients without vitiligo and in 9/11 of those in whom HN/HNi heralded vitiligo (s.s.). CONCLUSIONS: In patients with multiple HNi, the risk of vitiligo and other autoimmune diseases seems to be higher than in pediatric patients with a single HN; clinicians should pay particular attention to children with multiple HNi and personal or family history of autoimmune diseases.

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.

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.

Usefulness of dermatoscopy for the early diagnosis of sebaceous naevus and differentiation from aplasia cutis congenita.

In early infancy, a congenital area of alopecia of the scalp may be the clinical presentation of various diseases, with the two most common being sebaceous naevus and aplasia cutis congenita. Typical lesions are easily diagnosed, but soon after birth, the differential diagnosis may be difficult if the clinical features of the lesion are not distinctive. In this paper, we report the usefulness of dermatoscopy in such cases.

Primary Cutaneous Large B-Cell Lymphoma, Leg Type, Localized on the Dorsum.

Primary cutaneous large B-cell lymphoma, leg-type (PCLBCL-LT), is a large B-cell lymphoma primarily involving the skin. It is distinguished from the other 3 subsets of this lymphoproliferative disorder by its immunohistopathological features, configuring confluent sheets of medium-sized to large B lymphocytes with round nuclei provided with evident nucleoli, resembling centroblasts or immunoblasts, which express Bcl-6, Bcl-2. Prevalently appearing on the lower limbs, as a single or multicentric and frequently ulcerated skin nodule or plaque, PCLBCL-LT has a worse prognosis than the other large B-cell lymphomas. Moreover, the age of onset is delayed (7th decade) compared to those of the other 3 subtypes (6th decade); it presents a slight female predominance (2:1), and a higher percentage of positivity to Bcl-2. We present a 52-year-old man who showed a 2-year standing, non-ulcerated, round, 4 cm in diameter, red plaque, medially located on the dorsum. After biopsy the diagnosis of PCLBCL-LT was made on histopathological and immunohistochemical studies, the latter showing positivity to CD20, Bcl-2, and Bcl-6. After treatment with radiotherapy the patient has shown a 4.4-year follow-up free of disease.

[Clinical evaluation of the efficacy and tolerability of the ''NoAll Bimbi Pasta Trattante'' barrier cream in napkin dermatitis]. / Valutazione clinica dell'efficacia e della tollerabilità di NoAll Bimbi Pasta Trattante nelle dermatiti dell'area del pannolino.

AIM: Napkin dermatitis or diaper dermatitis is one of the most frequent cutaneous disease in the pediatric age. The most common type of napkin dermatitis is the irritant contact form, which is frequently complicated by an overinfection with bacteria or Candida albicans. METHODS: In this paper, 25 children (7 males and 18 females), aged between 1 month and 4 years and affected by napkin dermatitis, were treated with a barrier cream containing zinc oxide, vitamin E, panthenol (NoAll Bimbi Pasta Trattante, PSN Rottapharm, Milan, Italy). In 20 patients, a diagnosis of irritant contact diaper dermatitis was made, 3 patients suffered from atopic dermatitis, 1 patient was affected by psoriasis and 1 patient presented to a perianal streptococcal dermatitis. A candidal overinfection was diagnosed in 12 patients and was treated with a topical antifungal agent, applied twice daily for 2 weeks. NoAll Bimbi Pasta Trattante was applied at every diaper change. In all cases, we considered 2 subjective symptoms (burning and itching), and 3 objective parameters (erythema, oedema, exudation/vesiculation), at baseline and after 4 weeks of treatment. RESULTS: One patient withdrew the study. In 13 patients clinical healing was observed, 9 patients showed marked improvement, in 2 patients we did not observe improvement. Tolerability was considered excellent in 13 patients, good in 9 and sufficient in 2. CONCLUSIONS: The results obtained demonstrated that NoAll Bimbi Pasta Trattante, applied at every change of the napkin, is very effective and well tolerated in the treatment of irritant diaper dermatitis. Moreover, NoAll Bimbi Pasta Trattante, applied in association with other pharmacological treatments, may accelerate healing in case of overinfection due to Candida albicans and in case of inflammatory dermatoses localized in the gluteo-perineal area.

[Pachydermodactyly: seven new cases]. / Pachydermodactylie: 7 nouveaux cas.

INTRODUCTION: Pachydermodactyly is a superficial benign digital fibromatosis usually involving the proximal portions of the fingers. It is clinically characterized by an asymptomatic, bulbous, soft-tissue swelling around proximal phalanges and interphalangeal joints. We report here seven new cases of pachydermodactyly. CASE REPORTS: Seven patients (4 F, 3 M) ranging in age from 14 to 63 years were studied. Two of them were affected by tuberous sclerosis; two other patients were sisters, one of whom was affected by the transgrediens form of pachydermodactyly. The personal history of two male patients revealed the compulsive habit of interlacing the fingers. Finally a 23-year-old patient was affected by the localized pachydermodactyly. In all the patients roentgenogram and echography of the affected fingers as well as histological and ultrastructural of a cutaneous biopsy examination were carried out. The results of the tests confirmed the diagnosis of pachydermodactyly. DISCUSSION: Our data suggest that pachydermodactyly is underestimated rather than rare and more frequent in females than in males as until now reported in the literature. We suggest classifying pachydermodactyly into five types: classic pachydermodactyly frequently associated with mechanical trauma, monopachydermodactyly or localized pachydermodactyly, transgrediens pachydermodactyly in which the cutaneous thickness extends to the metacarpophalangeal areas, familial pachydermodactyly which may by transgrediens and pachydermodactyly associated with tuberous sclerosis.