Resumo em Inglês:

Abstract Oculocutaneous albinism is an autosomal recessive disease caused by the complete absence or decrease of melanin biosynthesis in melanocytes. Due to the reduction or absence of melanin, albinos are highly susceptible to the harmful effects of ultraviolet radiation and are at increased risk of actinic damage and skin cancer. In Brazil, as in other parts of the world, albinism remains a little known disorder, both in relation to epidemiological data and to phenotypic and genotypic variation. In several regions of the country, individuals with albinism have no access to resources or specialized medical care, and are often neglected and deprived of social inclusion. Brazil is a tropical country, with a high incidence of solar radiation during the year nationwide. Consequently, actinic damage and skin cancer occur early and have a high incidence in this population, often leading to premature death. Skin monitoring of these patients and immediate therapeutic interventions have a positive impact in reducing the morbidity and mortality associated with this condition. Health education is important to inform albinos and their families, the general population, educators, medical professionals, and public agencies about the particularities of this genetic condition. The aim of this article is to present a review of the epidemiological, clinical, genetic, and psychosocial characteristics of albinism, with a focus in skin changes caused by this rare pigmentation disorder.

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Abstract Background The use of monoethanolamine oleate 5% is effective for the treatment of vascular malformations with low blood flow. Objectives To report a case series of vascular malformations in the mouth and oral cavity treated with monoethanolamine oleate 5%. Methods A retrospective descriptive study was performed in electronic patient charts covering seven years. Patient demographics, diagnostic resources, lesion site, size, and number of applications of monoethanolamine oleate 5% were collected. Results A total of 21 vascular malformations were recorded, located mostly on the lower lip (52.3%) and resolved in a single application in 14 patients. The authors found 19 patients treated with sclerotherapy. Thirteen were women and six were men, with a mean age of 61 years. Study limitation Small sample size. Conclusions Sclerotherapy is an effective treatment for vascular malformations of the lips and oral cavity, with resolution after only one or two applications (n = 16).

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Abstract Background Malassezia, a skin saprophyte, is frequently isolated from patients with seborrheic dermatitis, which is one of the most common dermatoses in HIV-infected patients. Its role in pathophysiology has not been defined. Objective To determine whether patients living with HIV and seborrheic dermatitis have more Malassezia than those without seborrheic dermatitis. Method This is an descriptive, observational, prospective cross-sectional study to which all adult patients living with HIV that attend the infectious disease outpatient clinic at the Dr. Manuel Gea González General Hospital were invited. Patients presenting with scale and erythema were included in Group 1, while patients without erythema were included in Group 2. Samples were taken from all patients for smear and culture. Results Thirty patients were included in each group. All patients with seborrheic dermatitis had a positive smear, with varying amounts of yeasts. In the control group, 36.7% of patients had a negative smear. The results are statistically significant, as well as the number of colonies in the cultures.Study limitations The study used a small sample size and the subspecies were not identified. Conclusions Patients with clinical manifestations of seborrheic dermatitis have larger amounts of Malassezia. Further studies need to be performed to analyze if the greater amount is related to imbalances in the microbiota of the skin.

Resumo em Inglês:

Abstract Background The knowledge of general practitioners about photoprotection is unknown. Objectives To develop and validate an instrument to evaluate the knowledge of general practitioners and pediatricians about photoprotection, gauging the knowledge of these professionals. Methods The study followed the steps: (1) Literature identification and item elaboration related to the theme; (2) Content validation; (3) Apparent validation; (4) Construct validation: internal consistency analysis and discriminatory analysis; (5) Reliability analysis. In Step 4, the instrument was applied to 217 general practitioners and pediatricians who worked in the host city of the study; the scores were compared with dermatologists scores. Results The final instrument had 41 items and showed satisfactory internal consistency (Cronbach's alpha = 0.780), satisfactory reproducibility and good test-retest reliability (good-to-excellent kappa statistic in more than 60% of items). The discriminatory analysis registered a mean score of 54.1 points for dermatologists and 31.1 points for generalists and pediatricians, from a total of 82 possible points, representing a statistically significant difference (p < 0.001). Generalists and pediatricians demonstrated an understanding of the relationship between excessive sun exposure and skin cancer, but they revealed lack of technical information necessary for their professional practice. Study limitations The instrument evaluates only knowledge, without evaluating the conduct of the participants. Conclusion The results show that the instrument has good internal consistency and good reproducibility. It could be useful in the identification of general practitioners and pediatricians knowledge gaps on the subject, for the subsequent development of training and educational strategies.

Resumo em Inglês:

Abstract Background There is an obvious need for more prompt and specific biomarkers of bacterial infections in generalized pustular psoriasis patients. Objective The aim of this study was to evaluate the diagnostic properties and define appropriate cut-off values of procalcitonin and C-reactive protein in predicting bacterial infection in generalized pustular psoriasis patients. Methods Sixty-four generalized pustular psoriasis patients hospitalized from June 2014 to May 2017 were included in this retrospective study. The values of procalcitonin, C-reactive protein, details of infection, and other clinical parameters were analyzed. Results Receiver operating characteristic curve analysis generated similar areas (p = 0.051) under the curve for procalcitonin 0.896 (95% CI 0.782-1.000) and C-reactive protein 0.748 (95% CI 0.613-0.883). A cut-off value of 1.50 ng/mL for procalcitonin and 46.75 mg/dL for C-reactive protein gave the best combination of sensitivity (75.0% for procalcitonin, 91.7% for C-reactive protein) and specificity (100% for procalcitonin, 53.8% for C-reactive protein). Procalcitonin was significantly positively correlated with C-reactive protein levels both in the infected (r = 0.843, p = 0.040) and non-infected group (r = 0.799, p = 0.000). Study limitations The sample size and the retrospective design are limitations. Conclusions The serum levels of procalcitonin and C-reactive protein performed equally well to differentiate bacterial infection from non-infection in generalized pustular psoriasis patients. The reference value of procalcitonin and C-reactive protein applied to predicting bacterial infection in most clinical cases may not be suitable for generalized pustular psoriasis patients. C-reactive protein had better diagnostic sensitivity than procalcitonin; however, the specificity of procalcitonin was superior to that of C-reactive protein.

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Abstract Background Nipple eczema is a less common presentation of atopic dermatitis. No studies in the literature have correlated nipple eczema in pregnancy as a manifestation of atopic dermatitis. Objective To evaluate whether nipple eczema presenting in pregnancy is a manifestation of atopic dermatitis. Methods This was a prospective observational study including 100 women who presented with nipple eczema for the first time during pregnancy. The exclusion criteria were any patient with previous history of nipple eczema, those already on oral or topical treatment for atopic dermatitis or nipple eczema, and other disorders mimicking eczema. Patients were divided into two groups ‒ nipple eczema with atopic dermatitis and without atopic dermatitis. Demographic data, clinical features, total leukocyte count, differential leukocyte count, absolute eosinophil counts, and serum IgE levels were compared between the two groups to detect association between nipple eczema in pregnancy and atopic dermatitis. Results Out of 100 patients, 39 were diagnosed with atopic dermatitis, whereas 61 were ruled out to have any features suggestive of atopic dermatitis. There were no statistically significant differences in mean age, mean duration of symptoms, and serum IgE levels. In patients with atopic dermatitis, bilateral symptoms were noted more commonly than in patients without the disease, but this was statistically insignificant. Study limitations Lack of long term follow-up and no large studies in literature to compare results. Conclusion Nipple eczema in pregnancy follows a similar pattern as in other age groups. The clinical profile of patients is similar in cases with and without atopic dermatitis.

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Abstract Background Skin picking disorder is a prevalent disorder frequently comorbid with depression and anxiety, which is underdiagnosed mainly by dermatologists. Assessment of skin picking disorder is based on instruments influenced by the awareness about skin picking disorder and comorbid symptoms. To date, there is no validated instrument for Brazilian individuals nor an instrument to evaluate the severity of skin lesions in an objective way. Objectives Validate the Skin Picking Impact Scale for Brazilian Portuguese and create a photographic measurement to assess skin lesions. Methods The sample was assessed through the Skin Picking Impact Scale translated into Brazilian Portuguese, the Hamilton Anxiety Scale, the Beck Depression Inventory, and the Clinical Global Impression Scale. The patients’ skin lesions were photographed. Photos were evaluated regarding active excoriation, crust/bleeding, exulceration, and linear lesions. Results There were 63 patients included. The Skin Picking Impact Scale translated into Brazilian Portuguese had good internal consistency (Cronbach's alpha = 0.88), which tests of goodness-of-fit, showing a suitable model. The reliability of photographic measurement was 0.66, with a high internal consistency (Cronbach's alpha = 0.87). Photographic measurement was not correlated with the Skin Picking Impact Scale, the Clinical Global Impression Scale, or comorbid symptoms. Study limitations Lack of a previously validated instrument to evaluate dermatillomania in the Brazilian population for comparison. Conclusion The Skin Picking Impact Scale validated in Brazilian Portuguese is a good instrument to evaluate skin picking disorder. Photographic measurement is a consistent way of assessing skin lesions, but it does not reflect the impact of skin picking disorder on the individual's life.

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Abstract Background Rubeosis faciei diabeticorum is a persistent facial erythema in patients with diabetes mellitus. The actual pathogenesis has not been studied. However, it is speculated to be a cutaneous diabetic microangiopathy. Objective Examine the correlation between the severity of facial erythema and the possible causes of microvascular diabetic complications, namely oxidative stress, hyperglycemia, and cutaneous accumulation of advanced glycation end-products . Methods Patients diagnosed with Type 2 diabetes mellitus (n = 32) were enrolled in the study. The facial erythema index was measured using the Mexameter MX18; cutaneous accumulation of advanced glycation end-products was estimated by measuring skin auto fluorescence with the AGE Reader (DiagnOptics Technologies B.V. - Groningen, Netherlands). Glycated haemoglobin, total antioxidant status, and malondialdehyde were measured in blood by TBARS assay. The correlation between the selected variables was assessed by Spearman's rank test; p ≤ 0.05 was considered statistically significant. Results There was a statistically significant correlation between total antioxidant status and the facial erythema index (ρ = 0.398, p = 0.024). Malondialdehyde, skin autofluorescence, glycated haemoglobin, body mass index, duration of diabetes, and age did not demonstrate statistically significant correlation with the facial erythema index. Study limitations This is an observational study. Elevation of total antioxidant status could have been caused by several factors that might have also influenced the development of rubeosis faciei, including hyperbilirubinemia and hyperuricemia. Conclusions The results contradicted expectations. Total antioxidant status correlated positively with facial erythema index; however, there was no correlation with oxidative stress and skin autofluorescence. Further investigations should be conducted to reveal the cause of total antioxidant status elevation in patients with rubeosis faciei.

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Abstract Background One of the main prevention methods against skin cancer is the use of sunscreen; however, incidence of this disease has not declined despite prevention campaigns. Objective Investigate the prevalence of non-use of sunscreen and its associated factors. Method A population-based cross-sectional study with individuals aged 18 years or over living in the urban area. Conducted between April and July of 2016. Participants were interviewed about socioeconomic, demographic, and behavioral questions. Non-use of sunscreen was considered as the outcome. For multivariate analysis, Poisson regression with robust adjustment for variance was used. Results Among the 1300 participants, prevalence of non-use of sunscreen was 38.2% (95% CI: 34.6-41.8). The variables independently associated with the outcome were male sex, older age, brown or black skin color, lower income, fewer years of education, no physical activity in leisure time, no medical consultations in the last year, and self-perception of health as regular or poor. Study limitations The prevalence may be underestimated by reports of more use of sunscreen than actually used, which could increase the figure in the outcome. Conclusion It was estimated that about four out of ten adults and elderly do not use sunscreen in this sample. Prevention strategies are needed to advance health policy and ensure that sun protection options are easily accessible.

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Abstract The authors report a case of 40-year-old male patient with a five-year history of chromoblastomycosis on his right leg. Diagnosis was performed by direct 40% KOH exam of skin scales, culture with micro- and macromorphologic analysis, and genotypic characterization (sequencing of a fragment of the ITS region and phylogenetic analysis) of the isolated fungus. Rhinocladiella aquaspersa was identified as the etiological agent. Initially, the treatment was with oral itraconazole 200 mg/day for one year. However, the presence of “sclerotic cells” with filaments (“Borelli spiders”) resulted in a change of medical treatment: a higher dose of itraconazole (400 mg/day) and surgery, achieving clinical and mycological cure in one year. This is the first report of chromoblastomycosis caused by R. aquaspersa in Guatemala.

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Abstract The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Positron emission tomography and computed tomography scans found a mass in her retroperitoneal region. Pathology and immunohistochemistry investigation further corroborated the diagnosis of follicular dendritic cell sarcoma originated from hyaline-vascular Castleman's disease. Complete remission was achieved and the patient has been monitored for four years.

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Abstract Granulomatous pigmented purpuric dermatosis clinically manifests as hyperpigmented maculae and petechiae, predominantly on the lower extremities. Histopathologically, it is characterized by a lymphocytic infiltrate in the upper dermis, extravasated erythrocytes, and hemosiderin deposits. There is an infrequent variant called granulomatous pigmented purpuric dermatosis, which histologically is characterized by the presence of non-necrotizing granulomas associated with the classic findings of other pigmented purpuric dermatoses. It more frequently affects middle-aged women of Asian origin, and predominantly on the lower extremities. The authors present the case of a female patient with granulomatous pigmented purpuric dermatosis on the lower extremities with blaschkoid distribution.

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Abstract Cutaneous neoplasms frequently occur in leprosy, but there are few reports of the coexistence of leprosy and basal cell carcinoma in the same lesion. This case reports a 49-year-old male with an ulcerated plaque on the right lateral nasal wall, bright papules on the sternal region, and a blackened plaque on the right temporal region. The nasal and temporal lesions were diagnosed by histopathology as basal cell carcinoma and melanoma, respectively. The sternal lesions were excised with the repair of the “dog ear” which histopathological examination showed macrophages in the dermis parasitized with acid-fast bacilli, confirming the diagnosis of lepromatous leprosy with Fite-Faraco staining. This case report highlights the importance of referring the dog-ear specimen for histopathologic analysis.

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Abstract The correct identification of pigmented nodular lesions of the scalp is often challenging. Despite the importance of clinical patterns and dermoscopy, important adjuvant tools that are usually helpful, their interpretation sometimes is not clear-cut. Here, the authors discuss a case of sebaceoma mimicking a malignant pigmented neoplasia, with conclusive histopathology.

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Abstract Bartonellosis are diseases caused by any kind of Bartonella species. The infection manifests as asymptomatic bacteremia to potentially fatal disorders. Many species are pathogenic to humans, but three are responsible for most clinical symptoms: Bartonella bacilliformis, Bartonella quintana, and Bartonella henselae. Peruvian wart, caused by B. bacilliformis, may be indistinguishable from bacillary angiomatosis caused by the other two species. Other cutaneous manifestations include maculo-papular rash in trench fever, papules or nodules in cat scratch disease, and vasculitis (often associated with endocarditis). In addition, febrile morbilliform rash, purpura, urticaria, erythema nodosum, erythema multiforme, erythema marginatus, granuloma annularis, leukocytoclastic vasculitis, granulomatous reactions, and angioproliferative reactions may occur. Considering the broad spectrum of infection and the potential complications associated with Bartonella spp., the infection should be considered by physicians more frequently among the differential diagnoses of idiopathic conditions. Health professionals and researchers often neglected this diseases.

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Abstract The objective of this study was to describe the trend of detection of the disease in Brazil from 1990 to 2016. The joinpoint regression model was used. There was a significant trend of decreased detection in the country (average annual percent change −1.8%) and in the South (average annual percent change = −3.5%) and Southeast regions (average annual percent change = −4.5%). The Northeast (average annual percent change = 0.2%), the Central-West (average annual percent change = −1.5%), and the North (average annual percent change = −2.6%) showed a stationary trend (p > 0.05). Eleven states showed a decreasing trend. Alagoas (average annual percent change = 2.1%) and Rio Grande do Norte (average annual percent change = 1.4%) presented significant increase (p < 0.001). The heterogeneous pattern of trend between regions and states shows that efforts are needed to eliminate the disease.

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Abstract Dissecting cellulitis is an inflammatory, chronic, and recurrent disease of the hair follicles that mainly affects young Afro-descendent men. Trichoscopy is a method of great diagnostic value for disorders of the scalp. Clinical and trichoscopic findings of dissecting cellulitis are heterogeneous and may present features common to non-cicatricial and scarring alopecia. This article presents the trichoscopic findings of dissecting cellulitis that help in the diagnosis and consequent institution of the appropriate therapy and better prognosis of the disease.

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Abstract Tinea incognito resulting from corticosteroid abuse is becoming very common in the tropics. Its diagnosis is tricky owing to its confusing morphology, as well as practical and technical issues associated with mycological tests. Dermoscopy has now evolved as a novel diagnostic tool for diagnosing tinea incognito in such challenging situations, since the typical hair changes such as Morse-code hairs, deformable hairs, translucent hairs, comma and cork screw hairs, and perifollicular scaling may be seen despite steroid use, irrespective of mycological results.

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Abstract Langerhans cell histiocytosis is a rare clonal proliferative disease, characterized by the infiltration of one or multiple organs by histiocytes. Due to the diversity of signs and symptoms, the diagnosis of this disease is often late. The estimated incidence in adults is one to two cases per million, but the disease is probably underdiagnosed in this population. This report presents a case of disseminated Langerhans cell histiocytosis. The authors highlight the most characteristic aspects of this rare and heterogeneous disease, which usually presents as a challenging clinical diagnosis.