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Psoriasis is a chronic, inflammatory, immune-mediated, potentially disfiguring and disabling skin disorder, affecting over 100 million individuals worldwide. Calcipotriol plus betamethasone fixed-combination (0.005% Cal/0.064% BD) is the recommended topical treatment for psoriasis. However, topical treatments are associated to lack of efficacy and low adhesion rates due to poor cosmetic characteristics' formulations, and vehicle issues. To answer the need for improving patients' acceptability and adherence, an innovative Cal/BD aerosol foam formulation was developed for the topical treatment of adults with plaque psoriasis. Phase II and III clinical trials have consistently shown that the two-compound formulation is more effective and safer than its individual ingredients in the same vehicle. Cal/BD aerosol foam formulation has proved higher efficacy in clinical studies, and a pooled analysis of the main clinical studies has demonstrated that this does not occur at the safety profile expense. Furthermore, Cal/BD aerosol foam has shown a significantly improved efficacy compared with more traditional formulations, such as ointments, gels, and lotions. The improved efficacy and safety profile of this new formulation, together with the once-daily treatment, a more acceptable tolerability profile, and an early and rapid response, offers improved convenience and better acceptance over the twice- -daily applications required for the respective monotherapies, which may improve adherence to treatment, leading to faster and greater improvements in HR-QoL, representing a useful therapeutic option to the management of patients with plaque psoriasis. Further clinical investigations to explore the possibility of Cal/BD aerosol foam treatment ability to provide long-term psoriasis' management are required. [ABSTRACT FROM AUTHOR]

Psoriasis is a chronic inflammatory disease frequently seen in dermatologic practice. Although it is possible to control the majority of patients using only topical therapies, a significant percentage is refractory to these treatments, requiring systemic therapies. In these cases, one of the possible options is phototherapy. This paper details the various available therapeutic modalities of phototherapy in the treatment of psoriasis and the most frequently used protocols. [ABSTRACT FROM AUTHOR]

Chronic urticaria is a skin disorder with a very high impact on patients' quality of life. It has a prevalence between 7,8 and 22,3% with a higher incidence in women between 20 and 40 years old. The intensity of the itch, the anxiety caused by its recurrent nature and also the incapacity caused by the unpredictable exacerbations are among the factors that mostly impact patient's quality of life. Being a mast cell-driven disease, the most recent recommendations of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA2LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) suggest a symptomatic treatment for chronic urticaria, with very limited routine diagnostic measures being recommended. Non-sedating anti-histamines H1 (AHH1ns), in approved dose, are recommended as first line of treatment followed by the utilization of omalizumab, cyclosporine A or montelukast as add-on treatment in patients that do not respond to high doses AHH1ns. The treatment must continue until the patient is symptoms free, in the most possible safe manner. [ABSTRACT FROM AUTHOR]

Junctional epidermolysis bullosa is a group of inherited blistering diseases characterized by increased skin fragility, blisters and erosions after minor trauma, due to tissue cleavage at the dermal-epidermal junction. We report the case of a male Caucasian infant, born with erosions and tense blisters on the hands, abdomen and scalp. Immunoflurescence antigen mapping revealed paucity of collagen XVII immunolabelling, compatible with the diagnosis of non-Herlitz junctional epidermolysis bullosa. Molecular analysis of the COL17A1 gene revealed compound heterozygosity for two frameshift mutations. Family history was positive for a brother with a bullous dermatosis, who died from sepsis on the 3rd week of life without a definitive diagnosis that could enable precise risk estimation and prenatal diagnosis. We emphasize the importance of rapid referral to specialized centers, to provide early accurate diagnosis, adequate clinical management and prenatal diagnosis in subsequent pregnancies. [ABSTRACT FROM AUTHOR]

Melanocytic lesions and other skin tumors represent one of the most frequent reasons for pediatric dermatology consultation. Although often benign, some of them may simulate melanoma, which can be a source of anxiety for parents and clinicians. Dermoscopy is a non-invasive and painless technique that increases the diagnostic accuracy for skin tumours, while avoiding unnecessary skin biopsies. In part I of this article, we highlight the importance of dermoscopy for the diagnosis of skin tumors in pediatric patients. [ABSTRACT FROM AUTHOR]

Cutaneous leishmaniasis may mimic other infections in overlapping endemic areas and timely treatment prevents dissemination of the parasite. The required histopathological and microbiological examinations are not always available, and can only give a deferred confirmation of the diagnosis. In contrast, reflectance confocal microscopy (RCM) allows real-time visualization till the level of papillary dermis. A 59-year-old Brazilian male presented with ulcerated plaques and tumors on the extremities. The clinical differential diagnosis included leishmaniasis and other infections with lymphocutaneous pattern of dissemination. RCM showed the characteristic picture of «eggs in a bird’s nest» which has been described in cutaneous leishmaniasis. The diagnosis of leishmaniasis was later confirmed by skin biopsy, in which Leishmania guyanensis was identified by parasitological examination. After treatment with liposomal amphotericin B, reassessment with RCM corroborated the clinical cure, showing an «empty nest» picture. In conclusion, RCM noninvasively provides useful information for diagnosis and follow-up of cutaneous leishmaniasis. [ABSTRACT FROM AUTHOR]

Cancer patients may experience several dermatological symptoms during treatment. We describe a case of synchronous presentation of hand, foot, and mouth disease (HFMD) and palmoplantar erythrodysesthesia (PPE) in a breast cancer patient. A 36-year-old female breast cancer patient under chemotherapy presented to our consult with a 1-week history of painful palmoplantar lesions associated with the recent onset of fever and dry cough. Her daughter had been diagnosed with HFMD. There was a positive immunoglobulin M (IgM) and IgG serology for coxsackievirus. We performed two skin biopsies; one specimen was consistent with HFMD, and the other one was with PPE. To the best of our knowledge, the synchronous presentation of HFMD and PPE has not yet been reported in the literature to date. PPE and immunosuppression might have been responsible for the atypical presentation and clinical severity of HFMD. [ABSTRACT FROM AUTHOR]

Fumaric acid esters are licensed for the treatment of psoriasis in Germany, since 1994. They are effective, safe and economicalwhen compared with other systemic therapies used in moderate to severe plaque-type psoriasis. Fumarate esters have promisingpotential not only for the treatment of psoriasis but also for other less frequent dermatological conditions. The aim of this article is toreview the mechanisms of action, indications and contraindications for this therapy. [ABSTRACT FROM AUTHOR]

Spiny keratoderma is a rare dermatosis of unknown etiology that has been described with both hereditary and acquired variants. The acquired form has been associated with underlying malignancy and systemic diseases. We report a case of a 65-year- -old caucasian male presenting with multiple filiform hyperkeratotic papules in the palmar aspect of his hands which were present for the past year. Skin biopsy revealed aspects compatible with spiny keratoderma. After 10 months of follow-up, the patient developed mycosis fungoides, staged as IB according to the European Organization for Research and Treatment of Cancer and treated with PUVAtherapy with good control of both dermatosis. The authors raise the hypothesis of a new paraneoplastic association of spiny keratoderma. [ABSTRACT FROM AUTHOR]

Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are inflammatory diseases of unknown aetiology, which association is rare but well established. We describe the case of a 27-year-old female patient, with axillary and inguinal hidradenitis supurativa lesions since four years ago who developed a pyoderma gangrenosum lesion in her right leg. Treatment was instituted with systemic corticosteroids in inpatient setting. Afterwards, clindamycin and rifampicin were chosen followed by low dose isotretinoin with good response. The association of HS and PG can possibly have a common aetiology related to neutrophilic activity dysfunction. The description autossomic dominant syndrome PAPA (PG, acne and pyogenic arthritis) as well as PASH (PG, acne and HS), dermatos included in the spectrum of autoinflammatory diseases, promise a better comprehension of aetiopathogenesis of this association with possible diagnostic and therapeutic improvements. [ABSTRACT FROM AUTHOR]

Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis. It is characterized by a recurrent eruption of vesicles, erosions, and scaly erythematous plaques involving intertriginous areas and first occurring after puberty, mostly in the third or fourth decade. In 2000, mutations in the ATP2C1 gene on band 3q22.1, encoding the secretory pathway Ca2+/Mn2+-ATPase protein 1(hSPCA1), have been identified as the cause of HHD. We report the identification of two novel mutations of ATP2C1 gene in two Portuguese patients, which expands the spectrum of ATP2C1 mutations underlying HHD and provides useful information for genetic counseling. [ABSTRACT FROM AUTHOR]

Nowadays, with the recent development of biological agents and other new drugs, some of which already approved in pediatric age, it is important to contextualize the role of phototherapy in dermatology. They have an excellent and well-recognized safety profile for decades, in addition to not requiring laboratory monitoring, which is an advantage, especially in the pediatric population. They have been successfully used in this age group in the treatment of several dermatological diseases. In this article, a review is carried out about the main particularities of the use of phototherapy in pediatric age, focusing on adverse effects, the main challenges, their indications and the individualized selection of different modalities, in order to improve the clinical practice of this therapy. [ABSTRACT FROM AUTHOR]

Dermoscopy is a noninvasive technique that increases diagnostic accuracy of several skin lesions. Being painless, dermoscopy is particularly useful in children, sparing them from unnecessary biopsies and treatments. In part II of this article, we highlight the importance of dermoscopy for the diagnosis and follow-up of infectious and inflammatory skin disorders in pediatric patients. [ABSTRACT FROM AUTHOR]

Schwannoma is a benign encapsulated nerve sheath tumor composed of Schwann cells, that may arise anywhere along the course of a nerve. Cutaneous schwannomas, associated with peripheral nerves, usually present as a slow-growing well-circumscribed nodule, localized in the dermis or subcutaneous tissue. They are generally asymptomatic; however, they may become painful by the nervous compression. Since the clinical signs are non-specific, histology is required for a definitive diagnosis. Herein, we present a case of a solitary cutaneous ancient schwannoma of the ear, a histopathological variant of schwannoma with distinctive morphological characteristics. [ABSTRACT FROM AUTHOR]

Around 20% of the population suffers at least one urticaria episode, with 0.5 to 1% suffering from chronic urticaria. This disease has a mean duration of 1 to 5 years, and it has a higher incidence in women between 20 and 40 years old. Due to the publication in 2014 of the European guidelines for the management of urticaria, the authors performed a revision covering different aspects of chronic urticaria. Chronic urticaria is manly driven by the release of pro-inflammatory mediators from mast cells. It is characterized by the daily or almost daily appearance of transient itchy wheals and/or angioedema for more than 6 consecutive weeks. It is classified as either chronic spontaneous or inducible urticaria. Complementary diagnostic tests based on clinical history are recommended. An evidence-based symptomatic treatment includes as 1st line non-sedating 2nd generation anti- -histamines H1, which can be up dosed up to 4 times. Omalizumab and cyclosporine are 3rd line therapeutic options. Due to the highly negative impact on patients' quality of life together with the costs associated with this disease, it is highly important to raise awareness within the medical community. A correct and early diagnosis, along with the appropriate and timely referral to specialized centers has the main purpose of total symptom control in the safest possible way. [ABSTRACT FROM AUTHOR]

During the last decades, the incidence of opportunistic fungal infections has been increasing, namely in the context of immunosuppression. The Scedosporium apiospermum is a ubiquitous filamentous fungus in soil, decaying vegetation, sewers and polluted waters. Can cause infections in immunocompetent patients after trauma and severe and potentially fatal infections in immunocompromised patients. The authors describe a case of an infection by S. apiospermum on the dorsum of the left foot of a patient under prolonged systemic corticosteroid therapy combined with methotrexate due to the diagnosis of giant cell arteritis. Treatment of these infections can be a challenge due to the inherent resistance to many of the available systemic antifungal agents, including amphotericin B. In the case described, therapy with voriconazole was performed, with complete resolution of the lesions. [ABSTRACT FROM AUTHOR]

Introduction: Actinic keratosis is the most common premalignant lesion. The therapeutic approach to patients with multiple lesions involves field-directed treatment. Objective: To evaluate the efficacy and safety of ingenol mebutato, a new drug treatment for topical field-directed treatment of actinic keratosis. Design: Systematic review and meta-analysis of randomized controlled trials (RCTs) trials. Data sources: Medline and Cochrane Library (June 2014). Study selection: Two reviewers independently searched for studies and retrieved their characteristics and data estimates. Data synthesis: Random-effects meta-analysis. Heterogeneity was assessed with the I2 test. Results: Six trials (n = 1,492) versus placebo were included. The odds of a patient experiencing complete removal of the lesions was 17 (95%CI: 9 to 31, I2 = 0%) and 8.5 (95% CI: 5 to 15, I2 = 0%) times higher, compared to placebo, in the face/scalp and trunk/extremities, respectively. The incidence of adverse events related to treatment was higher in the group of mebutato ingenol (+23%, 95%CI: 11 to 35%), with no differences between groups in the discontinuation rate due to adverse events. Conclusions: Mebutato of ingenol is efficacious in the treatment of actinic keratosis. Compared to other field-directed treatments available in Portugal, its therapeutic value comes from the favourable safety profile and tolerability, simplicity and short duration of the treatment regimen and the possibility of different treatment to injuries according to anatomical location (individualization of therapy). Future studies should directly compare the different therapeutic options and evaluate the effectiveness of the same in the real world. [ABSTRACT FROM AUTHOR]