Tinea faciei in a central Portuguese hospital: A 9-year survey.

Tinea faciei is a relatively uncommon dermatophytosis that affects the glabrous skin of the face. The aim of this study was to analyse the epidemiologic, clinical and mycological features of tinea faciei cases diagnosed at the Dermatology and Venereology Department of Hospital Santo António dos Capuchos (Lisbon, Portugal). Consecutive cases diagnosed between 2008 and 2016 were studied retrospectively. A total of 72 tinea faciei cases have been diagnosed, involving 37 male and 35 female, aged between 8 months and 86 years. The majority were observed in patients younger than 12 years of age (59.72%). Anthropophilic isolates (mainly Microsporum audouinii, Trichophyton soudanense and Trichophyton rubrum) accounted for 75.7% of the identified dermatophytes. One quarter of the patients were also affected by dermatophytosis in other areas, such as the scalp. Only 10 cases were previously treated with topical steroids due to misdiagnosis. Most patients were treated with topical and systemic antifungal therapy with total resolution of skin lesions, without relapse or side effects. In contrast to other European studies, anthropophilic dermatophytes were the main causative agents of tinea faciei. As previously described to tinea capitis, this result is probably due to changes in the epidemiology of dermatophytes worldwide.

Dyskeratosis congenita--two siblings with a new missense mutation in the DKC1 gene.

Dyskeratosis congenital is reported in two siblings. They presented with the classic triad of mucocutaneous features: leukoplakia of the tongue, dystrophic nails, and a widespread reticulate pigmentation on the neck and upper chest. A genetic analysis was performed and a new missense mutation S356P, hemizygous, was identified in the DKC1 gene in both patients. Acitretin was started at a low-dose in both patients, resulting in clinical improvement and important, positive psychosocial effects.

Nodular vasculitis associated with lung adenocarcinoma.

Nodular vasculitis is an uncommon type of panniculitis. It was firstly associated to the infection by Mycobacterium tuberculosis and named erythema induratum of Bazin, however nowadays it has been associated with several other underlying disorders and it is better described as nodular vasculitis. Concerning its relationship with malignant conditions, there is only one reported case, in association with metastatic colon adenocarcinoma. We report a case of nodular vasculitis as the first manifestation of lung adenocarcinoma. To the best of our knowledge, this is the first reported association of both diseases.

Toenail Changes in Patients with Diabetes Mellitus with and Without Onychomycosis.

BACKGROUND: Diabetes mellitus is a predisposing factor for onychomycosis (OM). A high frequency of nonfungal onychodystrophy (OD) is also alleged, although information on the prevalence of specific nail changes is scant. We evaluated the prevalence and types of nail changes in a cohort of diabetic patients with fungal and nonfungal OD. METHODS: During a 6-month period, inpatients with diabetes mellitus were screened for foot and toenail changes. Demographic, social, and clinical data were recorded, as was information concerning foot and toenail care. Fungal infection was confirmed by mycologic examination and by histologic analysis of nail clippings. RESULTS: Of the 82 patients included, 65 (79.3%) had nail changes, and 34 of these 65 patients (52.3%) were diagnosed as having OM. The most frequently observed nail signs were subungual hyperkeratosis, onycholysis, yellow discoloration, and splinter hemorrhages, each seen in more than 25% of the patients. Tinea pedis and superficial pseudoleukonychia were observed more frequently in the OM group (P < .05). Conversely, prominent metatarsal heads and history of nail trauma were more frequent in patients with nonfungal OD (P < .05). CONCLUSIONS: Physicians who care for diabetic patients should not ignore nail changes. Fungal and nonfungal OD are common and should be addressed in the global evaluation of the feet to help prevent breaks in the skin barrier and subsequent bacterial infections and ulcers.

The dark side of the light: Phototherapy adverse effects.

Phototherapy is a valuable therapeutic tool in Dermatology, but there may be drawbacks. Acute and long-term adverse effects, of variable severity, include skin erythema, xerosis, pruritus, blistering, altered pigmentation, photoaging, and photocarcinogenesis. Despite concerns over the carcinogenic potential of ultraviolet radiation, most studies have not found an increased risk of non-melanoma or melanoma skin cancer in patients treated with ultraviolet B (broadband and narrowband) and ultraviolet A1 phototherapy. These are therefore considered reasonably safe treatment modalities concerning the development of skin neoplasms, although caution and further investigation are warranted. Photoprotective measures, such as avoidance of concurrent sunlight exposure and covering skin areas not afflicted with disease, or more modern strategies, including phytochemical antioxidants and exogenous DNA repair enzymes, can minimize the hazards of phototherapy. Patients submitted to phototherapeutic regimens should undergo complete, careful dermatologic examination regularly and lifelong.

The dark side of the light: mechanisms of photocarcinogenesis.

Ultraviolet radiation (UVR) can have a beneficial biologic impact on skin, but it is also the most significant environmental risk factor for skin cancer development. Photocarcinogenesis comprises a complex interplay between the carcinogenic UVR, skin, and the immune system. UVB is absorbed by the superficial skin layers and is mainly responsible for direct DNA damage, which, if unrepaired, can lead to mutations in key cancer genes. UVA is less carcinogenic, penetrates deeper in the dermis, and mainly causes indirect oxidative damage to cellular DNA, proteins, and lipids, via photosensitized reactions. UVR not only induces mutagenesis, altering proliferation and differentiation of skin cells, but also has several immunosuppressive effects that compromise tumor immunosurveillance by impairing antigen presentation, inducing suppressive cells, and modulating the cytokine environment. This review focuses upon molecular and cellular effects of UVR, regarding its role in skin cancer development.

Onychomycosis in patients with chronic leg ulcer and toenail abnormalities.

Nails have a limited number of reactive patterns to disease. Accordingly, toenail changes of different etiologies may mimic onychomycosis. OBJECTIVE To determine the prevalence of toenail onychomycosis among patients with leg ulcer and toenail abnormalities attending a dermatology clinic. METHODS A cross-sectional study was conducted through the analysis of clinical records and results of mycological examination. RESULTS A total of 81 patients were included, with a median age of 76.0 years. Most ulcers were of venous etiology, followed by those of mixed and arterial pathogenesis. The mycological evaluation confirmed the diagnosis of onychomycosis in 27.2% of the patients. The etiologic agent was a dermatophyte in 59.1% of isolates in nail samples, while Trichophyton interdigitale was the most frequent fungal species (40.9%). CONCLUSIONS Most toenail abnormalities in patients with chronic leg ulcer were not onychomycosis. This study highlights the importance of systematic mycological examination in these patients, in order to avoid overtreatment with systemic antifungals, unnecessary costs and side effects.

Nodular vasculitis associated with lung adenocarcinoma

Abstract: Nodular vasculitis is an uncommon type of panniculitis. It was firstly associated to the infection by Mycobacterium tuberculosis and named erythema induratum of Bazin, however nowadays it has been associated with several other underlying disorders and it is better described as nodular vasculitis. Concerning its relationship with malignant conditions, there is only one reported case, in association with metastatic colon adenocarcinoma. We report a case of nodular vasculitis as the first manifestation of lung adenocarcinoma. To the best of our knowledge, this is the first reported association of both diseases.

Birt-Hogg-Dubé syndrome.

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

[Why are our patients claiming? Statistics from a dermatology clinic]. / Porque reclamam os nossos doentes? Casuística de uma consulta de dermatologia.

INTRODUCTION: Patient satisfaction is a commonly used indicator for measuring quality in health care. In this context, patients are recognized as important and active agents in improving health services. In Portugal, progress has been made in characterizing concerns and needs of the national health system users, through the analysis of complaints and litigations. OBJECTIVE: To analyse complaints related to care in an outpatient dermatology department in a Portuguese hospital. MATERIAL AND METHODS: All complaints referring to the dermatology department and registered from 2000 to 2010 were analysed. RESULTS: During the eleven-year study period, 106 complaints were recorded, amounting to a rate of 0.4‰, and an increasing propensity to claim. Plaintiffs were mostly women (60.4%). The 'Administrative or Organizational' complaints were more prevalent than those pertaining to 'Healthcare Professionals' (58.5% vs. 41.5%). The former were mainly allusive to the 'Laws and Rules' of the institution,followed by claims related to 'Administrative Procedures'. All of the dissatisfaction records on the 'Healthcare Professionals' group were directed to doctors and medical acts. 'Frustrated Expectation' was the most frequent category in claims towards doctors, followed by'Behavioural' related ones. CONCLUSIONS: The rate of dermatology complaints in the studied department remains low comparatively to the national hospital average.The increase in the number of patients making claims can be attributed to higher demands and enhanced knowledge (or its absence) of their rights. These results emphasize the importance of the recent organizational improvements and highlight the doctor patient relationship. Health education might render a better management of health expectations and resources.

[Purpura: primary systemic amyloidosis manifestation]. / Púrpura: manifestação de amiloidose sistémica primária.

Primary Systemic Amyloidosis (AL) is the most frequent form of systemic amyloidosis and its morbilility is associated with immunoglobulin light chains deposition in vital organs. The mucocutaneous manifestations occur in about 30-40% of the cases and are important in diagnostic suspicion, once they appear in early stages of disease. We report a 71-years-old female patient, with disseminated purpura and cutaneous fragility with 6 months of evolution, accompanied by recent complaints of dysphagy. The first laboratory evaluation didn't show any alterations. The histological and immunohistochemical study of subcutaneous abdominal fat and skin biopsy showed lambda type amyloid protein. In the systemic work-up, we highlight a proteinúria > 1g/24h with Bence Jones proteins and the presence of monoclonal immunoglobulin light chain (lambda type) in serum immunoelectrophoresis. With the diagnosis of primary systemic amyloidosis, treatment with prednisolone and melphalan was started.

Birt-Hogg-Dubé syndrome / Sindrome de Birt-Hogg-Dube

A 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.

Uma mulher de 45 anos com história de carcinoma renal foi observada por pápulas cor da pele, faciais, cervicais e tronculares. Referia história familiar de achados cutâneos semelhantes e irmão com episódios repetidos de pneumotórax. Identificaram-se múltiplos quistos pulmonares por tomografia computorizada. Uma biópsia cutânea revelou fibroma perifolicular. O diagnóstico clínico de síndrome de Birt-Hogg-Dubé (BHDS) foi contudo corroborado pela identificação de uma nova mutação frameshift c.573delGAinsT (p.G191fsX31) em heterozigotia no exão 6 do gene da foliculina. A presença de múltiplos e típicos tumores benignos do folículo piloso, realça o papel do dermatologista no diagnóstico desta rara genodermatose, que está associada a um risco aumentado de tumores de células renais e cistos pulmonares, exigindo seguimento e aconselhamento pessoal e familiar.