Repositório RCAAP

A dermite atópica é uma doença inflamatória crónica caraterizada por intenso prurido e lesões eczematosas recorrentes. Observa-se com maior frequência em crianças com história pessoal e/ou familiar de atopia, porém também ocorre na idade adulta. Embora anteriormente pensada como uma doença desencadeada por uma resposta imunitária Th2 inapropriada, estudos recentes têm demonstrado que a disfunção da barreira cutânea desempenha um papel crucial para a fisiopatologia da doença. Neste artigo será feita uma revisão dos mecanismos moleculares mais recentemente implicados na fisiopatologia da dermite atópica.

Atopic dermatitis is a chronic inflammatory skin disease that usually affects children, but can also start in adults. This disease has a strong impact on the quality of life, however treatment options for severe atopic dermatitis are scarce. New insights into the physiopathology, emphasizing the crucial role of epidermal barrier dysfunction and its relationship with immune dysregulation, contributed to the development of new treatments.

Introduction: Scabies is a parasitic cutaneous infection, which is common in primary care, pediatrics and dermatology. Although it isn’t life threatening it causes considerable morbidity and has a substantial impact in the quality of life. In developing countries it is associated with high rates of cutaneous bacterial superinfection, namely impetigo.Objectives: To review the general aspects of scabies, with a particular emphasis on the diagnosis and treatment. To analyze the national and international epidemiological situation and the available treatment options, particularly in Portugal.Material and Methods: Narrative review of the scientific literature, including textbooks and scientific databases (PubMed/Medline, Cochrane, UpToDate, IndexRMP).Discussion: Scabies is a dermatosis with typical clinical characteristics, namely pruritus, type of cutaneous lesions and their distribution, and presence of epidemiological context. Efficacy of treatment of scabies patients and close contacts depends on a timely diagnosis, and diagnostic delay, low-adherence to treatment and incorrect treatment application should fundamentally be avoided.Conclusions: Given the efficacy and toxicity of the various treatment options, 5% permethrin and oral ivermectin are considered first-line. However, in Portugal, since neither are available, benzyl benzoate is most frequently used. A careful explanation of treatment steps and its correct application is essential for therapeutic success. In Portugal it would be useful and advisable that scabies becomes a notifiable disease.

The incidence of new HIV infections remains high despite efforts in early diagnosis and treatment in the last decade. In Western Europe and in countries where access to health care is not an obstacle, there have been changes in epidemiology. The subgroup of men who have sex with men has been particularly targeted and Portugal is no exception. In 2015, for the first time since 1984, more than 50% of the diagnoses of HIV infection in men were made in this group and represent 69.7% of the diagnoses in individuals, men or women, under the age of 30 years. This reality has forced to think about new ways of acting including the concept of pre-exposure prophylaxis to HIV, which is already a reality in some countries. In this article the authors review the current knowledge on the subject. Dermatologists and Venereologists should be alert to a possible increase in the incidence of other sexually transmitted infections as, in the context of this prophylaxis the adoption of risk behaviours may potentially facilitate transmission of new infections.

Introduction: Allergic contact dermatitis in children, although usually considered infrequent, has been the object of numerous recent publications and an apparent increase in prevalence. This may be due to early exposure and sensitization to contact allergens, increasing the likelihood of skin reactions in a new exposure, or the more widespread use of patch tests, which are crucial to the diagnosis of this pathology.Methods: In order to know the characteristics and causes of allergic contact dermatitis in the pediatric age, we evaluated the medical records of patients with < 18 years who were patch tested between January 2005 and December 2014 at the Contact Allergology Unit of the Department of Dermatology University Hospital of Coimbra. The following parameters were analyzed: sex, age, personal and familiar history of atopy, main location of the dermatitis, reactive allergens within the baseline or complementary test series, suspected and final diagnosis.Results: A total of 106 pediatric patients were tested (3.6% of all patients), 68 females (64.2%) and 38 males (35.8%), four aged between 3-5 years (3.8%), 28 between 6-11 years (26.4%) and 74 between 12-17 years (69.8%), 52,8% with a personal history of atopy, 36.8% with facial and/or neck lesions. At least one positive test was observed in 51 patients (48.1%), mostly in girls especially because of nickel (18.9%). Nevertheless there were no positive tests to nickel in the last two years of the study. After nickel, cobalt, p-phenylenediamine, caine mix and palladium were reactive in five patients. Twelve patients reacted to allergens that are not included in the baseline series, mostly to drugs.Discussion: Contact allergy and allergic contact dermatitis were a relatively common diagnosis before the age of 18. An early etiologic diagnosis is important to prevent chronicity of lesions and a possible future negative personal and professional impact. Knowing the main allergens in this age group is important to establish avoidance measures and prevent further sensitization. 

Introduction: Like in allergic contact dermatitis, in non-immediate (NI) cutaneous adverse drug reactions (CADR) delayed hypersensitivity to antibiotics and anticonvulsants is considered to be lifelong. Although in allergic contact dermatitis patch tests remain positive for several years, this has seldom been assessed in the setting of NI-CADR to systemic drugs.Objective: To evaluate the long term behaviour of positive patch test reactions in NI-CADR to beta-lactams, clindamycin and carbamazepine. Methods: The drugs associated with the largest number of positive patch test reactions (beta-lactams, clindamycin and carbamazepin) were selected and 64 patients with history of NI-CADR to these drugs and relevant positive reactions were invited to repeat patch tests, at least 2 years thereafter. New patch test reactions were compared with the original ones.Results: In the 23 patients included in the study (10 males/ 13 females, median age 51 years) there were 44 positive reactions at the first patch tests and 40 (91.1%) of these remained positive after a median interval of 6.5 years (min. 2.0 – max. 30.7 years). Concerning beta-lactams, 17/19 reactions persisted positive for aminopenicillins (amoxicillin or ampicillin), 8/8 for isoxazolyl penicillins (flucloxacillin or dicloxacillin), 3/3 for benzylpenicillin, 1/1 for cefoxitin and 1/1 with piperacillin. Reactions with clindamycin remained positive in 5/7 cases. All five patch tests repeated with carbamazepine were positive. In 62.5% of the tests the same intensity of reaction was observed and was not affected by the time interval between tests, gender or age at performing patch tests.Conclusion: In NI-CADR to several antibiotics and carbamazepine, most of the patch test reactions remained positive after several years.

Sexually transmitted infections are an important area of public health, requiring clinicians to be alert to their diagnosis. We present three issues that we consider pertinent: the Zika virus as a new sexually transmitted infection, hepatitis, including the epidemic hepatitis C infection in men who have sex with men that are HIV positive and the sexually transmitted hepatitis A outbreak verified in Europe, and the antimicrobial resistance of Neisseria gonorrhoeae.

The primary adrenal insufficiency or Addison's disease is a rare disorder of the adrenal glands and is a risk factor, since its diagnosis is often not recognized in the early stages of the disease. Currently, its main cause is idiopathic atrophy, but it can occur due to infectious, traumatic and neoplastic causes. Symptoms of the condition include: asthenia, weakness, nausea, weight loss, hypotension, and hyperpigmentation. The skin and mucosa hyperpigmentation is the most characteristic symptom of the disease, but may be present in other differential diagnoses, such as hemochromatosis, chronic kidney disease, hyperthyroidism, ocronosis, among others. We report a case of a patient who was referred for investigation of diffuse cutaneous and mucosa hyperpigmentation. The patient also indicated complaints of weight loss, fatigue, anorexia, hypotension and constipation. Several tests were conducted to research and accomplished the diagnosis of Addison's disease.

Pyoderma gangrenosum is a chronic neutrophilic dermatosis with no established etiology. It is often associated with systemic diseases such as inflammatory rheumatic diseases, inflammatory bowel diseases, malignant neoplasms and haematological diseases including nocturnal paroxysmal hemoglobinuria. It presents as ulcerated and painful skin lesions with rapid and progressive evolution, mainly in the lower limbs. Ulcerations may arise spontaneously or induced by trauma (pathergy phenomenon). The period between the onset of the lesions and the diagnosis is usually prolonged. The diagnosis is based on clinical data and confirmed after the exclusion of other causes of cutaneous ulcerations. We report a case of Pyoderma gangrenosum in the left upper limb with onset after a traumatic event in a patient with nocturnal paroxysmal hemoglobinuria.

Excessive sweating, either isolated or associated with other symptoms, characterizes hyperhidrosis. It commonly occurs on the forehead, hands, feet and armpits. It affects about 1% of the general population, both men and women of all ages. Although sweating represents an indispensable thermoregulatory skin function, excessive sweating is a benign disease that causes significant impact on quality of life and career of patients. Diagnosis is, essentially clinical and treatment can be either conservative or surgical. We describe the case of a 27-years-old female patient with palmar hyperhidrosis from adolescence who, after several therapeutic failures and difficulties, achieved excellent clinical response to iontophoresis with tap water. The patient has been at least one year without clinical treatment.

Sporotrichosis is a chronic infection of the skin and subcutaneous tissue, caused by the fungus Sporothrix schenkii. In childhood this entity is rare and currently the most frequent in this age group is the zoonotic transmission. The delay in diagnosis and inadvertent use of multiple antimicrobial regimens favor disease progression, therefore causing pain and subsequent scars. The main differential diagnosis for the skin and lymphocutaneous forms is leishmaniasis. Although there are international recommendations for using itraconazole as the first line treatment, it has a level of evidence equal to the saturated solution of potassium iodide and this liquid presentation is more confortable for children.

Overall, skin metastases from internal malignancies are a rare event, ranging from 0.9% to 4.4% in large autopsy studies and reaching 9% in large series of patients. Skin metastases from thyroid carcinoma are even rarer and, among them, follicular carcinomas have seldom been reported in this context, far less so than papillary and medullary carcinomas. A 57-year-old Caucasian male presented with an angioma-like papule on the parietal area of the scalp with a 4-month history. The patient had endured 2 years previously total thyroidectomy, radiotherapy and several chemotherapeutical regimens for a metastatic Hürthle cell carcinoma of the thyroid gland. Excisional biopsy of the nodules established the diagnosis of metastatic (lung and liver) follicular cell carcinoma on plain histological and immunohistochemical grounds. Lymph nodes, lungs and bones are the most common sites of distant metastases of thyroid carcinomas. Although accounting for about 12% of all thyroid carcinomas, skin metastases from follicular carcinomas have however only rarely been reported. These tumours are usually well-differentiated carcinomas, bearing a fair long-term prognosis and characterized pathologically by the presence of follicular structures and colloid material in the midst of diverse amounts of anaplasia and architectural disorganization. In our case, it is noteworthy the angioma-like morphology of the metastatic nodule, classically associated with renal cell carcinoma scalp metastases, as well as its aggressive nature which led eventually to a fatal outcome. This case is ichnographically depicted and a review of the relevant literature is done.

Multiple adult xanthogranuloma is a non-Langerhans cell histiocytosis, a rare variant of the juvenile xanthogranuloma. The etiology is unknown, affecting individuals predominantly below 30 years of age, without predilection for gender. Clinically presents itself as multiple, disseminated, yellow-brownish papules or nodules. Diagnostic confirmation is given by histopathology and by immunohistochemistry, which further allow to distinguish it from Langerhans cell histiocytosis. The spontaneous regression is uncommon. We report a classic, rare and clinically exuberant case of multiple adult xanthogranuloma and took the opportunity to perform a brief literature review on the topic.

The coexistence of two or more different neoplasms, benign or malignant, in the same cutaneous lesion is called a "collision tumor”. The clinical diagnosis is difficult and dermatoscopy is an often useful tool, however it may not be enlightening. We present the case of a rare association between a blue nevus and a histiocytofibroma that both clinically and dermatoscopically could be confused with malignant melanoma.

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Pruritus persisting for six weeks or longer is considered chronic. It may arise from dermatological, systemic, neurological, psychosomatic or psychiatric conditions or result from a combination of several factors. Due to chronicity processes, such as peripheral and central sensitization, pruritus may persist even after treatment of the underlying cause. Additionally chronic pruritus constitutes often a high burden for the affected patients, who frequently develop associated conditions, such as anxiety, depression or sleep disorders. Owing to the multiple dimensions of chronic pruritus, it presents a diagnostic and therapeutic challenge to the attending physician. The categorization of the condition according to the clinical presentation helps directing the diagnostic and treatment efforts. Therapeutically a step-wise approach should be undertaken. First basic measures, such as the use of emollients for dry skin, topical steroids for inflamed or excoriated skin and antihistamines should be initiated. If the origin underlying the chronic pruritus is found, a causal therapy should be attempted. If no cause is found or a causal treatment is not possible, a symptomatic multimodal therapy with topical and systemic agents is often necessary. With increasing knowledge of the pathophysiological mechanisms underlying chronic pruritus, novel drugs with promising effects are being developed.

Docetaxel is a second-generation taxane commonly used in the treatment of advanced malignant tumours such as breast adenocarcinoma. We describe the case of a 67-year-old female undergoing adjuvant chemotherapy with docetaxel for invasive ductal carcinoma of the left breast presenting with typical psoriatic lesions on the dorsum and limbs - the most representative of them arising close to the venous access for docetaxel infusion. No personal or family history of psoriasis was reported. The patient started and maintained treatment with oral acitretin and topical betamethasone diproprionate/calcipotriol for nearly eight months, with almost complete regression of cutaneous lesions. Drug-induced psoriasis is a rare condition. The most commonly reported drugs include beta-blockers, interferon and antimalarials. In this case we demonstrate that docetaxel should be included in the group of drugs that can potentially induce psoriasis.

This report describes a case of disseminated cutaneous histoplasmosis in a 30 years old black woman, born and living in Mozambique, with disseminated eruptive multiple papules, some of them with erosions, localized also in palms, plants and oropharyngeal mucosa. The HIV 2 test was positive and the CD4 count was 1 cel/mm3. Histopathology was compatible with infection by Histoplasma capsulatum. She was treated with amphotericin B during 21 days, with clinical improvement, followed by oral fluconazole 400 mg/day and antiretroviral, but she died 13 months later from gastroenteritis.

Leprosy is an infectious disease of chronic evolution caused by Mycobacterium leprae. Its chronic course can be interrupted by acute or subacute reactional episodes. Lucius phenomenon is a vasculitic immune reaction that occurs in patients with lepromatous leprosy. It appears with purpuric macules that progress to ascending necrosis and ulceration and heal with an atrophic scar. The authors describe the case of a 73-year-old male patient with ulceronecrotic lesions in the lower limbs and tendinous calcaneal exposure. Clinical-laboratory data and dermatological examination confirmed the diagnosis of lepromatous leprosy with vasculitis of the Lucius-like type. It progressed with a good response to the alternative treatment with multibacillary multidrug therapy.