Repositório RCAAP

The autoimmune photodermatoses are a group of heterogeneous idiopathic dermatoses including five different clinical entities some potentially severe with great impact in patients’ quality of life. General photoprotection measures are sufficient for the treatment of patients with mild forms of disease. In severe disease the therapeutic options are still limited, reflecting the ill-defined physiopathological mechanisms. However recent advances in photoprotection field are contributing to a higher therapeutic success. In an era where photodermatology has been losing its importance, we propose to revise the clinical manifestations and therapeutic options of autoimmune photodermatoses. These are rare and challenging disorders concerning diagnostic and treatment that need a specialized approach by dermatologists.

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.

Introduction: In chronic spontaneous urticaria (CSU) unresponsive to anti H1-histamines, national recommendations propose omalizumab, which is not always promptly available. Thus, other treatments should be discussed. In this context, we have analysed efficacy and safety of cyclosporine and possible relations between characteristics of urticaria and response to cyclosporine.Material and Methods: Retrospective study of CSU patients from a department of dermatology treated with cyclosporine (2010-2016). We evaluated the demographic and clinic-laboratorial parameters, doses, treatment duration, follow-up, side effects and efficacy, using the weekly urticarial activity score 7 (UAS7). Statistical significance was considered for p < 0.05.Results: We evaluated 23 patients (19 female/4 male; mean age 46.0y ± 14.0) with CSU which evolved for 129.7 ± 128.1 months and who were treated with cyclosporine (mean dose 3.2 ± 0.3 mg/kg). There was a good response in 10 patients (44%) at the first month, with a significant mean UAS7 reduction. Side effects from cyclosporine occurred in eight patients, leading to treatment suspension in six patients (26%), mostly due to arterial hypertension and infections. None of the parameters evaluated was associated with response to cyclosporine.Conclusion: Cyclosporine was effective in 44% of patients with moderate to severe CSU. When omalizumab is not immediately available, it may be worth trying cyclosporine, with a narrow monitoring for adverse effects.

Morphea or localized scleroderma is a connective tissue disorder in which excess collagen deposition leads to the development of sclerotic lesions. Initially distinguished from systemic sclerosis by the absence of systemic manifestations, involvement limited to the skin and a relatively benign course, it is now known that localized sclerosis may be associated with multiple extracutaneous manifestations. This article reviews the musculoskeletal, neurological, ophthalmological, gastrointestinal, respiratory, cardiac and renal manifestations of this disease.

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Endometriosis is characterized by the presence of ectopic implantation of endometrial tissue, normally functioning and hormone-sensitive. Extrapelvic involvement can occur in about 12% of women with the condition. Umbilical endometriosis has been reported to account for about 0.4% to 4.0% of all endometriosis and 30% to 40% of cases of cutaneous endometriosis. It is a rare case of a disease that presents important differential diagnosis with other umbilical papular disorders, which may be primary or secondary, neoplasic or inflammatory. This paper reports the case of a patient with no comorbidities, with a diagnosis of umbilical cutaneous endometriosis, based on anamnesis, clinical and histopathological examination, showing a good evolution after complete excision of the lesion. The definitive diagnosis is based on histopathology, but immunohistochemistry may be helpful as well as abdomen ultrasound, computed tomography and magnetic resonance imaging. Surgical management is the preferred treatment.

Parinaud's oculoglandular syndrome consists of a special form of granulomatous conjunctivitis, often related to cat scratch disease, whose classic presentation is characterized by a fever, conjunctival granuloma and ipsilateral lymph node enlargement. Diseases associated with this syndrome are cat-scratch disease, herpes simplex infection, sporotrichosis, blastomycosis and coccidioidomycosis, among others. We report the case of a patient with a unilateral conjunctival enanthema on the right eye and nodules on the ipsilateral eyelid. Complementary examinations including culture confirmed the diagnosis of sporotrichosis. The patient was treated with itraconazole and got cured.

Introduction: Partner notification plays a key role in the epidemiological control of the increasingly frequent sexually transmitted infections (STI).Material and Methods: One hundred consecutive patients from the Venereology consultation of the Santa Maria Hospital in Lisbon answered a questionnaire on notification of partners.Results: Forty three of the individuals had a previous history of STI, 25 of which had a previous diagnosis of HIV infection. Sixty-four patients said that they informed their partners about the diagnosis. Of those who did not inform, 50% no longer have contact with the partners and 36% consider that was not important. Regarding partner notification methods: 98% found appropriate to bring a leaflet about the disease to the partners; 72% would agree to give a consultation authorization to the partner, 58% considered that taking the medication directly to the partner would be an option, 58% thought appropriate that the partner's family doctor call them for a consultation; and 42% considered appropriate to send an email or mobile message.Discussion: The high percentage a previous STI diagnosis suggests this is a group of individuals with the highest risk of STI transmission. A high percentage (36%) of those who did not inform the partners felt that it would not be important, which may reveal a failure of communication from the health professional. The method considered the most suitable was passive reporting.Conclusion: It is necessary to improve effective partner notification at it is highly needed to reduce the incidence of STI.

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Introduction: Phototherapy has long been recognized as beneficial for psoriasis treatment, with a favorable risk- -benefit relation. Photochemotherapy comprises the use of psoralen, either orally (oral PUVA) or topically (bath PUVA), prior to UVA irradiation. Oral administration of psoralen may lead to short or long-term side effects. Bath PUVA is particularly useful to minimize systemic toxicity and psoralen side effects. The aim of this study was to compare the effectiveness of these two PUVA modalities.Material and Methods: A retrospective review of patients with chronic plaque psoriasis treated with PUVA therapy (oral and bath) in our dermatology department, between January 2001 and December 2016.Results: We performed 81 treatments with oral PUVA and 38 treatments with bath PUVA, in 68 and 26 patients, respectively. The mean age of the patients was 50,6 years. Oral PUVA group achieved PASI 75 in 68 cases (89.5%), and bath PUVA group in 26 (74.3%), with p-value=0 .05. The mean total dose needed to achieve PASI 75 in the oral PUVA group was 113.1 J/cm2 and in the bath PUVA group was 69.8 J/cm2. The mean number of sessions performed to achieve remission in the oral PUVA group was 23.31, and in the bath PUVA group was 17.58.Conclusion: Despite requiring specialized equipment and being more time consuming, bath PUVA represents one of the most effective therapies available for psoriasis and it should be considered as a treatment option for patients who are not candidates for systemic treatment.

Disseminate and recurrent infundibulo-folliculitis is a rare entity of unknown etiology, characterized clinically by a papular and follicular eruption and histologically by spongiotic changes in the infundibular portion of the hair follicle. It usually occurs in young adults males with phototype V-VI of Fitzpatrick. The dermatosis habitually reaches the trunk and limbs, has a recurrent course and is characterized by poor response to therapy. We present a case of infundibulo-folliculitis in a 10-year-old child.

Erythema annulare centrifugum is a dermatosis of unknown aetiology which usually follows a self-limiting course. Nevertheless, some cases tend to chronicity, especially when a culprit stimulus is not found, and can be challenging to treat. A 24-year-old male presented with a 3-year history of persistent plaques with polycyclic outlines and an infiltrated rim on the forearms, buttocks and thighs. After histopathological correlation, a diagnosis of erythema annulare centrifugum was therein made. Secondary causes were ruled out. Treatment with topical and systemic steroids provided no benefit, and the patient was reluctant to further systemic oral immunosuppression. Photochemotherapy was then attempted. A marked improved was observed since the first session, and the patient was clear by the 7th treatment. No adverse events were noted. The patient is still in remission at the 3rd month of follow-up. To our knowledge, we report the first case of an erythema annulare centrifugum successfully treated with photochemotherapy. Our case highlights the potential of this time-honoured therapeutic modality to address chronic and debilitating cases of this figurate dermatosis.

Introduction: Hand eczema is a multifactorial disease that can include manifestations of atopic dermatitis, irritant contact dermatitis, allergic contact dermatitis (mediated mainly by T cells) and/or immediate reactions such as contact urticaria or protein contact dermatitis (mediated or not by IgE). The study aim was the characterization of patients with hand eczema who reported immediate symptoms with the handling of suspected triggers.Material and Methods: Retrospective analysis of medical records of patients from the Dermatology Department with hand eczema who performed patch tests (baseline and complementary series according to the clinical history) and skin prick test whenever patients presented immediate symptoms with suspected triggers. Skin prick test results were correlated with specific and total IgE.Results: A total of 52 patients were evaluated (49F/3M), mean age: 33.4 ± 9.9 years, 25 (48.1%) with a personal history of atopy (nine with atopic dermatitis). Besides hand eczema, considered occupational in 33 patients (63.5%), dermatitis also involved the face in six (11.5%). We found positive patch tests in 31 patients (59.6%), mainly to nickel (45.2%). Skin prick test were positive in 28 patients (53.8%), mainly for latex (10, 35.7%) and kiwi (8, 28.6%). There was no statistically significant correlation between positive skin prick test and the presence of atopy or high total serum IgE.Conclusion: Although the study included a limited number of patients with hand eczema and we only performed skin prick test in those with complaints, we observed immediate reactions in a very significant proportion of patients (53.8%), mainly in the atopic group. Specific IgE was not useful for the diagnosis, compared to the skin prick test. Immediate mechanisms, not directly dependent on T cells, may be significantly involved in the pathogenesis of hand eczema, so immediate tests should be added more often to patch tests in the study of hand eczema.

Extramammary Paget's disease (EMPD) is a distinct form of a rare malignant skin neoplasm, primarily seen in postmenopausal Caucasian women. Surgery is the most common treatment. However, concerning vulvar EMPD, radical surgery can be mutilating and leads to loss of femininity. Recent therapeutic development has included medical and medical-surgical approaches, allowing better preservation of genital anatomy with subsequent reduced morbidity. Accordingly, off-label use of imiquimod has been reported as a monotherapy or adjuvant therapy in EMPD.We report the case of a woman with extensive EMPD in the anogenital area, submitted to surgical resection complemented with plastic reconstruction and preceded by imiquimod medical treatment. Neoadjuvant imiquimod induced a significant cytoreduction of the tumor area, minimizing surgical radicality and maintaining local function and morphology

Multiple eruptive dermatofibromas is a rare event characterized by the appearance multiple dermatofibromas in few months. It is usually associated with immunodysregulation/immunosuppression. An appropriate work-up of underlying diseases must be done after this diagnosis, including search for HIV infection and lupus erythematosus. We present a case of multiple eruptive dermatofibromas in a female patient with HIV infection, possibly associated with a decrease in the CD4+ T cell count and viral load increase.

Photodistributed hyperpigmentation has been associated with several drugs. We describe a 69-year-old woman who developed facial skin hyperpigmentation starting after treatment with a combination of olmesartan medoxomil and hydrochlorothiazide and improving following its withdrawal, suggesting drug-induced dyspigmentation. Olmesartanmedoxomil-hydrochlorothiazide should be added to the list of drugs that can induce photodistributed cutaneous hyperpigmentation.

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Lyme disease is the tick-borne disease with highest incidence in Europe and North America. Climate change seems to be modifying its geographic distribution, with spreading of endemic areas. Simultaneously, an increasingly number of cases is being diagnosed as the knowledge of its clinical presentations is growing.Most individuals come to the Dermatology visit after a tick bite or with lesions diagnosed as erythema migrans. Other cutaneous clinical presentations, like borrelial lymphocytoma and acrodermatitis chronica atrophicans, are rare. In the presence of a tick bite, doctors should remove it from the body surface and perform clinical vigilance for a period of at least 30 days. Prophylactic antibiotic therapy is not recommended in Europe. Preventive measures as the use of protective clothing in endemic areas should be promoted.The diagnosis of erythema migrans is clinical and first line antibiotic therapy is doxyxycline, which has a high success rate. Serologic tests are essential for the diagnosis of less common cutaneous presentations. Histopathologic examination of skin biopsies also supports the diagnosis. Prompt treatment allows faster and greater improvement, with less irreversible lesions.

Introduction: Superficial fungal infections are the most frequent infectious dermatoses and their incidence continues to increase. Dermatophytes are the principal agents presenting, however, a variable geographic distribution.Material and Methods: This study aimed to characterize the epidemiology of superficial fungal infections diagnosed in Dermatology departments/ units of the Portuguese National Health System between January 2014 and December 2016, through a retrospective analysis of the results of positive cultures performed during this period.Results: A total of 2375 isolates from 2319 patients were studied. The most frequently isolated dermatophyte was Trichophyton rubrum (53.6%), which was also the main cause of glabrous skin tinea (52.4%) and of onychomycosis (51.1%). In relation to tinea capitis, Microsporum audouinii was the most prevalent agent globally (42.6%), followed by Trichophyton soudanense (22.1%). While in the Lisbon metropolitan area these dermatophytes were the main causative agents, in the North and Center regions of Portugal, Microsporum canis was the most frequent agent (58.5%). Yeasts were the main agents isolated from onychomycosis of the hands (76.7%).Conclusion: The results of this study are globally in agreement with the scientific literature. Trichophyton rubrum is the most frequent dermatophyte overall. As for tinea capitis, in the Lisbon metropolitan area, the imported anthropophilic species assume particular importance.

Introduction: Premalignant cutaneous lesions, skin cancer, and cutaneous xerosis are diseases whose incidence continues to increase in our older population. Concerning the contest “Dermatologist from the Heart” awarded by Fondation La Roche Posay in 2015, we put into practice the winning project “Life goes on, Live your skin!”. The main purpose of the project was to provide specialized health assistance and prevention of skin cancer and cutaneous xerosis in a population over 65 years old living in social institutions in the district of Santarém.Material and Methods: Between February 2016 and April 2017, a medical team consisting of a specialist and two Dermatovenereology residents visited eight carefully selected institutions of the area of Santarém.Results: A total of 301 patients were observed, with a high prevalence of actinic keratoses (42.8%) and cutaneous xerosis (34.2%), followed by basal cell carcinoma (9.3%), suspected pigmented lesions (3.7%) and squamous cell carcinoma (1.7%).Conclusion: This project allowed us to provide health care in distant places and to a population with limited access to specialized medical care, thereby increasing their quality of life, to obtain real numbers about prevalence of frequent pathologies in elderly, screen a high number of patients, treat premalignant lesions and avoid their evolution to skin cancer, and establish primary and secondary prevention programs in the institutions.