RCAAP Repository

Introduction: Stevens-Johnson syndrome and toxic epidermal necrolysis are severe skin reactions associated with mucosal lesions and systemic involvement. Glucocorticoids were used for many years in the treatment of Stevens-Johnson syndrome / Toxic Epidermal Necrolysis, but there is no evidence to support this approach.Objective: Assessment of mortality in Stevens-Johnson syndrome/Toxic Epidermal Necrolysis cases in a group of patients that used systemic glucocorticoids and another group who did not.Secondary objective: Description of the study population; review of secondary infection and extra-cutaneous complications in both groups of patients.Material and Methods: Retrospective cohort of patients selected by consulting requested to the Dermatology Service of the Hospital Nossa Senhora da Conceição, as well as the diagnosis at admission or death, during the period March 2007 to August 2014.Results: Mortality rate was 63.2% in the group that used corticosteroids and 9.1% in the group that did not use steroids, with p value 0.005 (95% CI 1.79-163.8). Multivariate analysis adjusted for SCORTEN showed only a trend for harm, with an odds ratio for mortality in patients who used glucocorticoids being of 7:38 (95% CI 0.87-161.43) and p value 0.06.Conclusion: This study suggests a trend for harm associated with glucocorticoids use for treatment of these diseases, especially in regard to mortality.

Introduction: Photodynamic therapy is noninvasive and is based on the topical application of a photosensitizing agent, which after exposure to light source generates free radicals that selectively injure the pre-neoplastic and neoplastic cells. It is approved for the treatment of actinic keratosis, Bowen's disease and basal cell carcinomas.Methods: This retrospective study evaluated the results obtained in the treatment of basal cell carcinoma with photodynamic therapy in the Department of Dermatology, Hospital de Braga, during a 10-year period (2003-2013), in particular cosmetic outcome, patient satisfaction, the relapse and side effects.Results: A total of 161 patients were included. In females, the median age of patients was 69 years and for males the median age was 68 years. Regarding sex, 85 (52.8%) of the patients were female and 76 (47.2%) were male. In females there was a predominance of basal cell carcinomas on the face (n = 97, 50.8%), while in males there was a predominance in the trunk (n = 67, 41.1%), and this difference was statistically significant. The median area of treated basal cell carcinomas was 20 mm2. All patients reported slight pain at the time of irradiation. The recurrence of treated lesions was 0.5%.Discussion: Photodynamic therapy had excellent cosmetic results, low percentage of recurrence, few secondary effects and high patient satisfaction. However, the classical surgery continues to play a key role, particularly in nodular basal cell carcinoma and morpheaform basal cell carcinoma, which are contraindications to performing the photodynamic therapy.

Pigmented skin lesions are sometimes misdiagnosed due to clinical similarities between melanocytic and non-melanocytic lesions. We report the case of a patient with a pigmented lesion that clinically resembles melanoma. With dermoscopy, observed features, namely leaf-like areas, allowed us to make the diagnosis of pigmented basal cell carcinoma. This case represents a clinical setting in which clinical examination alone could lead to a misdiagnosis, but with the use of dermoscopy an accurate diagnosis was possible.

The authors describe the combination of simple techniques to correct a complex surgical defect of malar area, nasolabial fold and ala of nose. The subcutaneous hinge flap restores the anatomic contour and enables the use of a simple flap or graft for reconstruction of the skin surface. Hinge flaps are an excellent alternative to more complex interpolated flaps for reconstruction of central facial surgical wounds.

Introduction: There has been in the last decades an increasing number of reported gonorrhea cases in Europe.Methods: We retrospectively reviewed the clinical records of patients attending Hospital de Curry Cabral’s STD Clinic between January 2006 and December 2013 (8 years). The screening for Neisseria gonorrhoeae infection is performed systematically in all patients (molecular diagnosis technique – BD Probetec-SDA system). The authors assessed epidemiological and socio-demographic data and determined the frequency of N. gonorrhoeae infection.Results: During the reviewed period, out of 1735 patients, gonorrhea was diagnosed in 114 cases (6.6%), 104 men and 10 women, with a median age of 29 years. The diagnosis of gonorrhea was found in 63 men who have sex with men (MSM) and 41 men who have sex with women. Of the totality of patients diagnosed with gonorrhea, 26 were HIV positive (24%). The urogenital site was the most frequent with 103 cases, followed by anorectal in 12 patients and oropharyngeal in 9 patients. Of all these diagnoses, 17% occurred in anatomic sites where patients did not report symptoms (13 men and 5 women).Conclusions: The results of this study were similar to data recently published in the literature. The high number of gonorrhea cases in MSM must be highlighted. In this epidemiological context, the identification of this agent in asymptomatic anatomic sites is not negligible, supporting the importance of extragenital screening.

Delayed-type hypersensitivity to metals in patients with orthopedic implants may cause cutaneous lesions and/or pain, effusion and functional impairment of the prosthesis. As these signs are common to periprosthetic joint infection, the differential diagnosis is difficult, but metal hypersensitivity has to be considered as a possible cause of this growing problem. Seven months after total knee arthroplasty, a female patient developed pain and functional impairment of the knee, cutaneous erythema, swelling and a nodule with serous-hematic drainage in the inferior limit of the surgical scar. Microbiologic study was repeatedly negative. Patch testing showed reactivity to chromium (potassium dichromate 0.5% pet), a constituent of the metallic prosthesis, and also to titanium oxide 0.5% pet, neomycin 20% pet and gentamicin 20% pet, which might explain the inflammatory reaction surrounding the prosthesis. Although rare, there is an association between metal hypersensitivity and implant failure. Therefore, in the presence of skin lesions around the prosthesis implantation site, metal hypersensitivity should be considered and studied.

Pityriasis rubra pilaris is a chronic scaly papular disease, of unknown etiology. Although there are familial cases, most of them are acquired. It Affects men and women equally, presenting bimodal incidence peaks in the first and fifth decades of life. We report a case of Pityriasis rubra pilaris in the classic adult form, on a male patient aged 40, who had chronic erythematous scaly follicular papules, 4 years of evolution, on the dorsum of the fingers. This study addresses the clinical course of the disease, differential diagnoses, histopathological findings and treatment with acitretin.

Dermatomyositis is a systemic autoimmune disease with cutaneous, muscular and pulmonary involvement. MDA-5 has recently been described as a specific dermatomyositis target antigen associated with a higher risk for interstitial lung disease. A 54-year-old female patient presented with proximal muscular weakness, myalgia and cutaneous signs of dermatomyositis. She presented with high aldolase and CK levels, and the muscular biopsy and electromyography were consistent with dermatomyositis. Pulmonary CT scan showed ground glass appearance. Autoimmunity revealed strong anti-MDA-5 positivity. No neoplasm was detected. She began treatment with prednisolone 20mg, hydroxicloroquine 400mg and methotrexate 20mg/week with very little improvement. She then began IVIG, with better clinical response. The authors report a case of dermatomyositis with interstitial lung disease associated with anti-MDA-5, an association unknown until recently.

Propylthiouracil (PTU) is widely used in the treatment of Hyperthyroidism. Usually well tolerated, it is though associated with antineutrophil cytoplasmic antibodies (ANCA) and, less frequently, with late-onset ANCA positive vasculitis. The authors describe the clinical case of a 45-year-old female patient with Graves’ disease for which she was being treated with PTU since 3 years before, who developed an ANCA-positive cutaneous leukocytoclastic vasculitis without systemic involvement. Initially seen as idiopathic the vasculitis was posteriorly associated with PTU. The drug was discontinued with a progressive and maintained recovery. The patient remains asymptomatic with 3 years of follow-up.

Introduction: Allergy to nonsteroidal anti-inflammatory drugs (NSAIDs) is often associated with severe hypersensitivity reactions and with difficult diagnosis. Basophil activation test by flow cytometry with Flow2cast (BAT) is a novel in vitro test for identification of hypersensitivity reactions to these drugs but with some limitations.Clinical case: Young male manifesting chronic urticaria since childhood presented with worsening of hives with angioedema immediately after NSAID intake. Last reaction happened over a year before after lysine acetylsalicylate. Initial BAT for diagnosis with acetylsalicylic acid (ASA) was inconclusive. Drug provocation test (DPT) negative to celecoxib. Another DPT with 700mg of ASA triggered anaphylaxis three hours later with hypoxia and serum tryptase 27.1μg / L. A second BAT had higher basal activation and marked increase with ASA incubation.Discussion: This case shows current BAT recommendation in NSAID hypersensitivity: a negative BAT does not avoid NSAID DPT. However, time between last reaction and BAT seems to influence results.

Vasculitis is characterized by blood vessel inflammation, resulting in their damage or even complete destruction, and may lead to either ischemic or hemorrhagic presentations. Infection is a common cause of vasculitis, usually by deposition of immune complexes, complement activation and endotoxins. Less often, endocarditis or sepsis, due to embolization and direct invasion, can cause vasculitis. We present a 35 year-old woman, with purpuric and necrotic lesions, showing a relapsing course during several months. Complementary workup revealed cardiac vegetations, which were considered the most likely etiology for cutaneous manifestations. However, based on ethical/ religious concerns, the patient refused surgical treatment and died. This case represents a rare and exuberant clinical presentation of a thrombotic vasculopathy, probably caused by septic embolism from staphylococcal endocarditis.

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This paper presents a case of Hebra’s prurigo, with the typical picture in a child under similar socioeconomic conditions and predisposing factors described by Hebra in Vienna, in the nineteenth century. This disease is an eruption of multiple extremely itchy small papules. Because of scratching, there are abrasions or lichenification, frequent secondary infection and progression to scars with dyschromia. Affected children are usually malnourished and have increased inguinal, axillary or cervical lymph nodes. They are generally atopic children with hypersensitivity to insect bite, in a context of inadequate hygiene and poor nutrition.

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Líquen estriado é uma erupção papulosa inflamatória que acomete principalmente crianças. Apresenta etiologia desconhecida e uma baixa prevalência. Surge menos frequentemente na idade adulta, e é caracterizado pelo aparecimento abrupto e assintomático de pápulas descamativas em disposição linear, por vezes seguindo as linhas de Blaschko, usualmente unilaterais e em extremidades. É importante considerar no diagnóstico diferencial outras dermatoses que acompanham as linhas de Blaschko, como por exemplo o líquen plano linear. Relata-se um caso atípico de líquen estriado com surgimento na idade adulta e localizado em flanco e região lombar à esquerda. Discutimos sobre a importância da dermatoscopia como ferramenta auxiliar no diagnóstico de líquen estriado.

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The porphyrias are a group of rare metabolic disorders that result mostly from a genetically determined dysfunction of specific enzymes involved in the heme biosynthetic pathway. Clinical presentation is highly variable, as well as its severity and prognosis. The photosensitivity defines the cutaneous porphyrias. Differential diagnosis can be difficult due to uncharacteristic clinical symptoms and overlapping laboratory findings. The treatment is still controversial, although sun avoidance is essential. In this article, we intend to conduct a theoretical review of the porphyrias with cutaneous manifestations in children, emphasizing the diagnosis and treatment of each subtype.

The use of the latest advances in nanotechnology has revolutionized the way towards prevention and treatment of some skin affections. The use of nanoparticles and microparticles in dermatology with a cosmetic, preventive and therapeutic aim is becoming increasingly common. Less known but also thoroughly researched and used is the application of several active nano and microparticles in materials that are in contact with the skin, such as clothing, paper, various types of equipment used in everyday life, furniture, concrete, varnishes paints and so on.

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

Introduction: Zoon balanitis or balanitis circumscrita plasmocellularis is a chronic disease of unknown etiology, described mainly in middle-aged or elderly uncircumcised men.Objectives: Characterization of the clinical variables and the response to treatment in patients with histological diagnosis of Zoon balanitis in Hospital de Santa Maria between January 1th 2009 and December 31th 2014.Results: The sample included a total of 23 patients, aged 65 (±11) years old. The clinical differential diagnoses more frequently set were erythroplasia of Queyrat and lichen sclerosus. Concerning therapy most of the patients applied corticosteroids or were submitted to circumcision. From the five patients and the only patient in which CO2 laser therapy was used there is no evidence of recurrence. Of the remaining 17 patients, 4 are under disease progression over corticosteroids topical treatment.Conclusion: Although Zoon balanitis is generally considered a benign condition, the differential diagnoses with squamous cell carcinoma makes the histological confirmation, follow-up and surveillance of this entity mandatory. The persistence of this disease with topical treatments makes chirurgical intervention the gold standard approach in the treatment of these patients.