Visceral Dissemination of Mucocutaneous Leishmaniasis in a Kidney Transplant Recipient.

Intracellular protozoan of the genus Leishmania, endemic in the Mediterranean basin, are the cause of cutaneous (CL), mucocutaneous (MCL), and visceral leishmaniasis (VL). A 75-year-old woman was admitted nine years after a second kidney transplant (KT), due to persistent pancytopenia and fever. She presented edema and erythema of the nose in the last two years and an exophytic nodular lesion located on the left arm, with areas of peripheral necrosis and central ulceration in the last 18 months. A bone marrow biopsy revealed features compatible with Leishmania amastigotes, and polymerase chain reaction test (PCR) for Leishmania infantum was positive. Moreover, biopsy and PCR for L. infantum of the cutaneous lesion on the patient's left arm and nose and PCR from peripheral blood were positive. Thus, a diagnosis of CL, MCL, and VL was made, and liposomal amphotericin B was initiated, but the patient had an unfavorable outcome and died. This is the first report of a KT recipient presenting with the entire spectrum of leishmaniasis. In Portugal, this infection is rare-so a high degree of clinical suspicion is required for its diagnosis, especially in endemic regions, as visceral leishmaniasis is a potentially life-threatening infection.

Awareness, knowledge, and practice patterns of general practitioner residents and specialists toward hidradenitis suppurativa: a survey study.

INTRODUCTION: Hidradenitis suppurativa (HS) is an unrecognized chronic inflammatory and debilitating disease with severe consequences for patients' quality of life. METHODS: A survey was performed among general practitioner (GP) residents and consultants in order to determine awareness, knowledge, and attitudes about HS. RESULTS: Among 372 respondents, 74% were GP residents in the first 2 years, 22% GP residents in the 3rd and 4th year, and 4% consultants. For a patient with boils and/or recurrent abscesses in folds, 90% considered a diagnosis of HS with no significant difference according to years of experience. These patients were referred to dermatology by 273 residents (80%) and eight consultants (53%), and this difference is statistically significant (p < 0.05). Regarding acute treatment, 84% prescribed topical antibiotics and 76% oral antibiotics. Respecting therapeutic approach, we observed that treatment with non-steroidal anti-inflammatory drugs is higher among older residents (51%) compared to younger ones (36%, p < 0.02) and the prescription of oral clindamycin is higher among consultants (31%) compared to residents (12%, p < 0.04). CONCLUSIONS: Our survey demonstrates that knowledge of HS is lacking among primary care physicians. Communication channels between GPs and dermatologists are often hampered, and so we recommend incorporating medical education into GP residency programs on how to treat mild HS, when to refer, and how to approach HS.

Medication-Related Osteonecrosis of the Jaw in a Patient with Nonuremic Calciphylaxis.

A 62-year-old man was referred to the emergency department of our hospital with pain and swelling in the left mandibular region that had evolved over the previous 2 months (Figure 1). His medical history included nonuremic calciphylaxis and systemic sclerosis (Figure 2). Since the diagnosis of the nonuremic calciphylaxis 5 years before, the patient had been treated with intravenous (IV) sodium pamidronate 60 mg per week for 11 months, without improvement, followed by IV sodium thiosulfate 25 mg twice a week for 18 month. During year 3 of treatment, the calciphylaxis lesions reappeared, and IV sodium pamidronate 60 mg per week was reintroduced to the patient's treatment. The patient remained with double treatment for the next 2 years, but 3 months before the patient's presentation, the IV treatment had been suspended due to an absence of peripheral venous access.

Nonuremic Calciphylaxis: Four Cases Associated with Autoimmune Diseases.

Calciphylaxis is a rare and severe syndrome of vascular calcification with unclear pathogenesis. This disease mainly affects patients with end-stage renal disease; however, it also has been described in nonuremic patients. We report four cases of nonuremic calciphylaxis (NUC) with ulcerated lesions associated with autoimmune disease. These cases support the literature suggesting that bisphosphonates and sodium thiosulfate are effective drugs in the treatment of calciphylaxis regardless of renal function.

Dermatitis neglecta: a challenging diagnosis in psychodermatology.

Dermatitis neglecta is a condition affecting the skin caused by a lack of hygiene. It may be related to psychiatric and neurological disturbances. The appearance of skin lesions results from neglect, which helps distinguish this condition from other similar clinical entities. Resolution of the lesions with adequate cleansing aids a definitive diagnosis.

Psoriasis and comorbidities: general practitioners' awareness.

INTRODUCTION: Systemic inflammatory diseases such as psoriasis, systemic lupus erythematosus (SLE), and rheumatoid arthritis (RA) are associated with an increased prevalence of cardiovascular diseases (CVD) and other comorbidities. The primary aim of this study was to assess the screening practices of general practitioners (GPs) with regard to the most frequent comorbidities in patients with psoriasis. METHODS: We adapted, with permission, a questionnaire that was used by Parsi et al. in 2012, which was then distributed to GP residents and consultants. RESULTS: Overall, 372 questionnaires were collected. Significantly more physicians screen for CV risk factors in patients with RA and SLE than in patients with psoriasis. There was no statistically significant difference between GP residents in the initial and final phase of residency, or between GP residents and consultants regarding awareness of increased prevalence of CVD in psoriasis or comorbidity screening practices in psoriasis patients. CONCLUSIONS: Most GP residents and consultants that participated in this study are not aware of an increased CV risk in patients with psoriasis and assign greater importance regarding this risk to other inflammatory diseases such as RA and SLE.

Cutaneous Morbidity Among Inflammatory Bowel Disease Patients: A Cohort Study.

BACKGROUND AND AIMS: Patients with inflammatory bowel diseases are prone to cutaneous manifestations. The aim of this study was to investigate their prevalence, type and association to demographic and clinical factors. METHODS: This was a cross-sectional study. Information relative to patients of a central Portuguese hospital with a definitive diagnosis of an inflammatory bowel disease, who were prospectively recruited, was collected. RESULTS: The final cohort included 342 patients, 62% of whom had Crohn's disease and 38% had ulcerative colitis. Cutaneous extraintestinal manifestations were present in 44.4% of all patients; this prevalence was lower [14.9%] when excluding cutaneous manifestations secondary to nutrition deficiency or drugs. These skin lesions were classified as granulomatous [0.3%], reactive [4.4%], immunologically associated [10.5%] and secondary to nutritional deficiencies [6.4%] or to bowel-related therapy [29.5%]. Excluding those secondary to nutrition or drugs, cutaneous manifestations were significantly associated with females (odds ratio [OR] 3.210 [1.625-6.340], p = 0.001) and younger patients (OR 0.954 [0.924-0.985], p = 0.004). Additionally, their occurrence was related to patients up to 16 years (OR 13.875 [1.332-144.484], p = 0.028) among the Crohn's disease sub-cohort, whereas in the ulcerative colitis sub-cohort they were more likely to occur in patients with extensive colitis (OR 5.317 [1.552-18.214], p = 0.008). CONCLUSIONS: Nearly half of the patients analysed had at least one cutaneous extraintestinal manifestation. The fact that certain lesions tend to be more common among patients with defined characteristics should alert the physicians and allow an early diagnosis and, when pertinent, a reference to dermatology.

Moderate to severe psoriasis treatment challenges through the era of biological drugs.

Biological therapy has revolutionized moderate to severe psoriasis treatment. However, despite being more effective than conventional systemic treatments, some patients do not respond or lose response to biotechnological treatments or develop drug-antibodies, interfering with its safety and efficacy. There are also clinical forms of the disease and patient profiles for which is pending further scientific evidence for more sustained therapeutic interventions. The continuous and more detailed knowledge of psoriasis pathophysiology has allowed identifying new therapeutic targets, which is expected to help overcome the challenges of individualized psoriasis treatment.

Moderate to severe psoriasis treatment challenges through the era of biological drugs

Abstract: Biological therapy has revolutionized moderate to severe psoriasis treatment. However, despite being more effective than conventional systemic treatments, some patients do not respond or lose response to biotechnological treatments or develop drug-antibodies, interfering with its safety and efficacy. There are also clinical forms of the disease and patient profiles for which is pending further scientific evidence for more sustained therapeutic interventions. The continuous and more detailed knowledge of psoriasis pathophysiology has allowed identifying new therapeutic targets, which is expected to help overcome the challenges of individualized psoriasis treatment.

Cowden syndrome: clinical case and a brief review.

Cowden syndrome is a rare genodermatosis of autosomal dominant inheritance characterized by multiple hamartomas in several organs and an increased risk of malignancies. We present the case of a 53-year-old man with a history of benign and malignant thyroid disease, intestinal polyposis, and Chiari malformation. He had several trichilemmomas, papillomatosis of the oral cavity, macular pigmentation of the glans penis, among other clinical features suggestive of Cowden syndrome. Given the suspicion, genetic study was conducted and PTEN mutation was identified. Cowden syndrome affects 1:200,000 individuals. Mucocutaneous lesions are almost always present and there may be other typical features involving other organs, namely thyroid, colon, and brain. Mucocutaneous lesions may be the initial manifestation of this disorder and usually precede the onset of malignant lesions, making timely diagnosis essential for proper monitoring and screening.

Pigmented lesion of the nipple - a clinicopathological challenge.

Differential diagnoses of pigmented lesions of the nipple include melanocytic nevus, melanosis of the nipple, seborrheic keratosis, pigmented basal cell carcinoma, melanoma and Paget disease. The histologic exam with appropriate immunohistochemistry is a fundamental tool to achieve a correct diagnosis. We present a patient with a pigmented lesion of her right nipple revealing mammary Paget disease and elucidate diagnostic obstacles and prognostic importance of early breast cancer detection.