Repositório RCAAP

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‘Kounis Syndrome’ is an acute coronary artery event due to an artery spasm occurring during immediate hypersensi- tivity reactions or chronic spontaneous urticaria. Recently it has been reported in other systems, including the cerebral vasculature. We present a case series of three patients observed between January 2016 and December 2018 with acute and transient brain injury associated with concomitant exacerbation of chronic spontaneous urticaria, including one patient with multiple recurrences of neurologic symptoms during exacerbations of urticaria. Minor imaging defects were observed in two patients, but there were no apparent vascular risk factors or coagulation abnormalities that might explain neurologic symptoms. Chronic spontaneous urticaria, through activation of mast cells and mediator release, seems capable of inducing cerebral arterial aggression. The authors want to call the attention to this possible association, reinforcing the need to keep urticaria under control to prevent neurological manifestations.

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a recently described chronic autoinflammatory disease, pathophysiologically related to intracellular proteasome/immunoproteasome dysfunction. This review chronicles the recognition of CANDLE syndrome, the developments in the understanding of the pathomechanism, genetic background, and the emerging therapeutic strategies of this condition.

Sebaceous nevus of Jadassohn (SNJ) is an organoid hamartoma of the skin usually seen at or shortly after birth. Its most common location is the scalp, however, it may involve other areas of the head and neck. Classically it presents as a well- -defined plaque composed of multiple confluent yellowish-orange or yellow-brownish papules. The development of neoplasms in SNJ is well documented (10%-30%) as well as its possible association with a neurocutaneous syndrome. We report an exuberant case of facial SNJ in a 2-month-old male patient who underwent surgery at an early age. Recent literature on the subject is briefly addressed.

Ulcerating and mutilating variant of carpal tunnel syndrome occurs in longstanding cases of untreated disease. Pa- tients present with painless ulcers of the second and third fingers, accompanied with other cutaneous and sensory changes. These patients are often misdiagnosed as having a Raynaud disease or systemic sclerosis. Clinical assessment is the gold standard for the diagnosis of carpal tunnel syndrome, but hand radiography and electromyography help supporting the diagnosis. The authors present two cases of this ulcerating variant of carpal tunnel syndrome.

Syringocystadenoma papilliferum is a rare benign adnexal tumor, commonly located in the cephalic segment and usually associated with a sebaceous nevus. Clinically, it may resemble several neoplastic lesions, but histopathology is very characteristic. Surgical removal is the definitive treatment. The occurrence of this lesion on the nipple, as presented below, is extremely rare, although already described. A 47 years’ old female patient reported a vegetating lesion on the right nipple, present for 8 months, occasionally with exudation. On examination, a vegetating nodule with a papillomatous and erosive surface measuring about 2 cm was observed adjacent to the nipple. Surgical excision with a narrow margin was performed. Histopathology showed the typical aspect of papillary syringocystoadenoma.

Primary cutaneous lymphomas are defined as a heterogeneous group of malignant lymphoproliferative neoplasms that attack the skin, without extracutaneous involvement at the moment of the diagnosis. The subgroup "primary cutaneous lymphoma of great cells B, leg type (PCLBCL, LT)" generally attacks one or both lower limbs, however in 10% - 15 % of the cases other areas of the skin may be affected. We present a rare case of PCLBCL, LT with a nodular lesion located in the cervical region to empathize an atypical presentation. Considering its quick growth and high proliferative rate, it is of great importance to know all its possible clinical presentations for a precocious diagnosis and efficient treatment.

Morphea is a rare inflammatory disorder with an unknown etiology. We report the case of a 35-years-old woman presenting with an 18-month history of skin thickening on the extremities and trunk, and inflammatory arthralgia. Complaints started 2-weeks after a fertility treatment. The physical exam and workup confirmed the diagnosis of morphea, and the patient started treatment with deflazacort and methotrexate, with significant improvement. Six years later, the patient was submitted to another fertility treatment with exacerbation of the disease. The temporal association between the fertility treatments and the onset and further worsening of morphea suggest an influence of sex hormones on its pathophysiology.

Z-plasty is a surgical technique classically used to correct scars. It consists of a double transposition of two or more adjacent triangular-shaped flaps resulting in a Z-shaped scar. This procedure is especially advantageous when the surgical incision is not parallel to the skin tension lines as it allows repositioning of the tension vectors, tending to a more favorable cosmetic result and preventing scar retractions. We present the case of a young woman with a left supraciliary basal cell carcinoma whose excision resulted in an extensive surgical defect which was reconstructed using a multiple Z-patch. The authors describe the surgical technique as well as its main advantages and applicability.  

Introduction: Acute bacterial dermo-hypodermatitis (DHAB) is an acute infection of the dermis and hypodermis that most often affects the lower limbs. Although diabetes mellitus has been identified as a risk factor for its development, recent studies have questioned this relationship. The aim of the present study was to compare clinical characteristics of inpatients with DHAB associated or not with diabetes mellitus. Material & Methods: Prospective study of patients hospitalized at the Dermatology Department of the Coimbra Hospital and University Center with the diagnosis of DHAB between January and June 2018. The following parameters were evaluated: 1) demographic / biometric data - gender, age; body mass index; 2) clinical and evolutionary aspects - location of infection, interval between initial symptoms and diagnosis, history of a previous episode; previous diagnosis of diabetes mellitus; 3) laboratory abnormalities - leukocytosis, C-reactive protein (CRP), microorganism screening (blood, abscess pus, wound exudate, blister content); 3) therapy - duration of antibiotic therapy, need for second line therapy, length of hospitalization; 4) local (abscess, necrosis) or systemic complications (bacteremia, drug rash, deterioration of underlying disease and death). Data were analyzed with the SPSS software, mainly looking for the influence of diabetes mellitus on the different parameters evaluated. Statistical significance was set at p <0.05. Results: We included 102 patients, 55 female (53.9%) and 47 male (46.1%), with a mean age of 68.6 ± 13.9 years. The lower limb was the most affected site (73.5%), followed by the upper limb (20.6%) and face (5.9%). In average there were 3.1 ± 2.5 days between initial symptoms and hospitalization. Twenty-four patients had a diagnosis of diabetes mellitus (23.5%), six under insulin treatment (25%). No statistically significant difference was found between the diabetic and non-diabetic group for gender, age, infection location, time from initial symptoms to hospitalization, neither in circulating leukocyte or CRP values. Microorganism screening (blood, abscess pus, wound exudate, blister content) was positive in 2/8 diabetics (25%) and 15/39 non-diabetics (38.5%) (p=0.138), with the same type of microorganism isolated in both groups. Initial antibiotic therapy - cefoxitin plus clindamycin in 64.7% - was replaced in one non-diabetic and 10 diabetic patients (p=0.451) and the total duration of antibiotic treatment and hospitalization between groups was similar. Local complications occurred in 3 diabetics (12.5%) and 15 non-diabetics (19.2%), and systemic complications in 4 diabetics (16.7%) and 12 non-diabetics (15.4%), p=0.553 and p=1.000, respectively. Conclusion: The present study shows that diabetes mellitus in hospitalized patients diagnosed with DHAB is not associated with a worse prognosis, namely in which concerns need for second line antibiotic therapy, longer hospitalization or local/systemic complications.

L-Mesitran Tulle® is a popular non-adherent polyethylene dressing impregnated with the patented L-Mesitran Soft gel that contains Medilan™, which is said to be a “hypoallergenic lanolin”. Lanolin is a well know sensitizer and the prevalence of contact allergy to lanolin alcohols varies from 0.6% - 6.9%. We report the case of a patient who develop eczema around the leg ulcer while using L-Mesitran Tulle® as a wound dressing, highlighting that even this “high purity medical grade of lanolin” (Medilan™) contained in L-Mesitran Tulle® can cause an allergic contact dermatitis.

Introduction: Although corresponding to only 5% of cutaneous neoplasms, melanoma is the tumor of this organ with the highest mortality rate. The constant development of new drugs, particularly those targeting the B-Raf protein, has significantly increased overall patient survival. To date, several studies have evaluated the clinical-pathological correlations with this mutation. However, no study has been published regarding the Portuguese population. Material & Methods: In the present study, the authors investigated the existence of clinico-pathological correlations with the presence or absence of BRAF mutation in a population of patients with metastatic cutaneous melanoma, in order to identify prognostic factors related to this cell signaling pathway. Results: A total of 197 melanoma patients’ clinical processes with BRAF mutation tested, observed at the IPOLFG Dermatology Service between January 2012 and December 2016, were evaluated. The BRAF mutation was documented in 46.7% (n=92) of the patients, 60% of which (n=56) were male. Fifty-two percent (n=48) of the primary tumors were located in the trunk, suggesting the association of the mutation with intermittent sun exposure. In 40% of patients (n=79) metastasis occurred at multiple anatomical sites. The percentage of patients with metastasis in a single anatomical site was 60% (n = 118), with the most frequently involved sites being locoregional ganglion chains (36%, n=43) and lung (26.6%, n=25). Conclusion: The results presented did not reveal differences in the studied Portuguese population, in agreement with those documented for other populations and described in the literature.

Introdução:

Cutaneous malignant melanoma is the third most common type of skin cancer, and its incidence has been rising. Its mortality rate is considerable, due to an aggressive phenotype and great ability of dissemination, mainly in the first years of follow- -up. Late recurrences, those presenting more than 10 years after diagnosis, are rare. The main prognostic factor of cutaneous malignant melanoma is tumor thickness, which also guides management. Thin tumors often have a good prognosis. We report a case of a 66-year-old woman with a history of excision of a thin primary cutaneous malignant melanoma of the dorsum, presenting 16 years later with an unexpected, rapidly progressing and lethal recurrence.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous adverse drug reaction. According on the culprit drug(s), imputability can be confirmed by patch test (PT). Our objective was to evaluate the value of PT in DRESS in the last 10 years, in comparison with our study in the preceding 10 years. Material & Methods: From 2009 to 2018, patients with DRESS performed PT at the Dermatology Department of Coimbra University Hospital, with the main culprit drug(s) and all drugs administered concomitantly or suspected of inducing DRESS flares. Drugs at 1% - 10% pet (Chemotechnique diagnostics®) or from a commercial preparation diluted at 10% pet were patch tested. Results: We studied 41 patients (20 male/ 21 female, mean age 53 years). The main culprits were allopurinol (n=15), antiepileptics (n=14), trimethoprim/ sulfamethoxazole (n=4), salazopyrine (n=3), diclofenac (n=2), antiretrovirals, ezetimibe/simvastatin and strontium ranelate (1 each). In 15 patients other drugs (n=18) were suspected of worsening DRESS, amoxicillin (n=8), ciprofloxacin (n=2), cefoxitin (n=2), levofloxacin, ceftriaxone, ceftazidime, vancomycin, acyclovir and metamizole (1 each). A positive PT to the culprit drug was observed in 10 patients (24.3%), all to antiepileptics. All patients tested for alopurinol had negative results. Positive reactions were observed to drugs related with flares in 12/18 suspected drugs (67%), but not to quinolones. Conclusion: Results were similar to the study conducted 10 years before when 18/56 (32.1%) patients had positive PT, mostly to carbamazepine and other anticonvulsants. Distinct from our previous study, when no tests were performed with the antibiotic series or other drugs used after the initiation of DRESS, we showed that PT can be a valuable tool to diagnose co-sensitisation in DRESS and emphasise the importance of testing all medications taken during the whole episode, even when PT has no value for the main culprit, like allopurinol. Recognising a co-sensitisation can prevent a new DRESS induced by the second drug.

Non-sexually transmitted urethritis are rare and may be caused by uropathogens in the context of urinary tract manipulation or anatomical abnormalities. We present a case of Pseudomonas urethritis in a 71-year-old man who had undergone prior cystectomy and cutaneous urinary diversion. This case illustrates the importance of considering and investigating less frequent causes of urethritis, ensuring that correct treatment is given, in order to avoid possible complications.

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The differential diagnosis of long-lasting erythematous skin nodules in patients living in Africa is broad. We report a clinical case of a 65-year-old Caucasian male, living in a Southern African Country, presenting to our department with an eight- -month history of erythematous skin nodules on the trunk and limbs. The diagnosis was B-pseudolymphoma and we discuss its aetiology and differential diagnosis.

Introduction: Patients with previous non-melanoma skin cancer have an increased risk of developing another skin cancer and some studies suggest that the histological type of the incident tumour can predict the one of the subsequently diagnosed. The aim of this study was to assess a correlation between the histological type of the first and the subsequent non-melanoma skin cancer diagnosed in immunocompetent patients and in different settings of immunosuppression. Methods: A retrospective study was conducted on all patients without previous skin cancer, with the diagnosis of two or more non-melanoma skin cancer between January 1st, 2008 and December 31th, 2017. Results: A total of 413 patients were included. Fifty-one individuals (12.4%) were immunosuppressed. There was a significative association between the histological type of the first and the subsequent non-melanoma skin cancer diagnosed both in immunocompetent and in immunosuppressed patients, with a higher probability of developing a tumour of the same histological type (p<0.001). This association was also significative in patients with the diagnosis of a hematologic malignancy. The mean interval between the two diagnoses was 30 months (range 7-111). Forty-three patients (10.4%) presented a subsequent tumour after more than five years of follow-up. Conclusion: The histological type of the incident non-melanoma skin cancer predicted the risk of developing another tumour of the same type. For the first time, we showed this correlation in patients with a hematologic malignancy. High-risk individuals may benefit from a long-lasting follow-up of at least ten years.

Introduction: Basal cell carcinomas are mostly treated surgically, mostly by surgery with postoperative histopathologic margin evaluation (“conventional surgery”), but large long-term data regarding recurrence by completeness of excisions is limited. Methods: Retrospective cohort study of basal cell carcinomas treated by conventional surgery at different medical specialties in a large tertiary centre, between 2008 and 2014. Survival analysis with a Cox proportional-hazards was performed, stratified by completeness of excision (complete excision/incomplete excision) and adjusted to several potentially confounding covariates. Results: A total of 2876 basal cell carcinomas were identified, of which 2306 (2100 primary, 206 recurrent) were considered eligible for analysis. During the 5-years of follow-up, there were 80 (4%) recurrences among 1980 complete excisions (16/1000 cases-year) and 83 (23.9%) recurrences among 348 incomplete excisions (100/1000 cases-year). Survival analysis was performed with multivariable adjustment. In the final adjusted model, we identified an association between relapse and re-intervention on recurrent tumors [adjusted Hazard Ratio (HR) 2.20 (95% Confidence interval (IC), 1.26-3.84), p=0.006], a wrong preoperative clinical diagnosis/surgery devoid of preoperative biopsy [adjusted HR 2.75 (95% CI, 1.68-4.5), p<0.001], treatment prior to 2012 [adjusted HR 1.47 (95% IC, 1.06-2.05), p<0.021] and surgery on a high-risk location, accordingly to the NCCN stratification [adjusted HR 2.18 (95% CI, 1.08-4.40), p<0.030]. By specific anatomic location, the likelihood of recurrence was especially high in the nose [adjusted HR 3.18 (95% CI 1.71-5.87), p<0.001] and eyelids [adjusted HR 3.08 (95% CI, 1.32-7.17), p=0.009]. There was also a trend towards higher recurrence in aggressive histological subtypes [adjusted HR 1.43 (95% CI 0.99-2.07), p<0.058]. Conclusion: Recurrent basal cell carcinomas, regardless of location, and primary basal cell carcinomas on high-risk locations of the face, especially on the eyelids and nose, should be considered to have a higher and independent likelihood of recurrence, even on “complete excisions” evaluated by histopathology. On the other hand, wait-andsee approaches in incompletely excised BCCs should be considered against a significant 5-year risk of relapse (1 in 10 lesions).