Maria Manuel Brites

HLA-B*58:01 is a risk factor for allopurinol-induced DRESS and Stevens–Johnson syndrome/toxic epidermal necrolysis in a Portuguese population

HLA‐B*58:01 is associated with allopurinol‐induced severe cutaneous adverse drug reactions (sCADR) particularly in Han Chinese, but the risk in European populations has seldom been studied.

Pyoderma gangrenosum – a review of 24 cases observed over 10 years

Background and objectives  Pyoderma gangrenosum (PG) is a disorder, included in the spectrum of neutrophilic and auto‐inflammatory dermatoses, whose clinical aspects and outcome we intend to characterize.

Bullous pemphigoid and comorbidities: a case-control study in Portuguese patients

BACKGROUND aAlthough rare, bullous pemphigoid (BP) is the most common autoimmune blistering disease. Recent studies have shown that patients with bullous pemphigoid are more likely to have neurological and psychiatric diseases, particularly prior to the diagnosis of bullous pemphigoid. OBJECTIVE The aims were: (i) to evaluate the demographic and clinical features of bullous pemphigoid from a database of patients at a Portuguese university hospital and (ii) to compare the prevalence of comorbid conditions before the diagnosis of bullous pemphigoid with a control group. METHODS Seventy-seven patients with bullous pemphigoid were enrolled in the study. They were compared with 176 age- and gender-matched controls, which also had the same inpatient to outpatient ratio, but no history of bullous or cutaneous malignant disease. Univariate and multivariate analyses were used to calculate odds ratios for specific comorbid diseases. RESULTS At least one neurologic diagnosis was present in 55.8% of BP patients compared with 20.5% controls (p<0.001). Comparing cases to controls, stroke was seen in 35.1 vs. 6.8%, OR 8.10 (3.80-17.25); dementia in 37.7 vs. 11.9%, OR 5.25 (2.71-10.16); and Parkinsons disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinsons retained their association with BP. Patients under systemic treatment were eight times more likely to have complications than those treated with topical steroids (p< 0.017). CONCLUSIONS The results of this study substantiate the association between BP and neurological diseases. In addition, they highlight the potential complications associated with the treatment of BP.

Epidemiology of basal cell carcinomas and squamous cell carcinomas in a Department of Dermatology: a 5 year review

BACKGROUND Non-melanoma skin cancer, a common designation for both basal cell carcinomas and squamous cell carcinomas, is the most frequent malignant skin neoplasm. OBJECTIVE Epidemiologic characterization of the population with Non-melanoma skin cancer. METHODS Retrospective analysis of all patients diagnosed with Non-melanoma skin cancer based on histopathologic analysis of all incisional or excisional skin biopsies performed between 2004 and 2008 in a Department of Dermatology. RESULTS A total of 3075 Non-melanoma skin cancers were identified, representing 88% of all malignant skin neoplasms (n=3493) diagnosed in the same period. Of those, 68,3% were basal cell carcinomas. Most Non-melanoma skin cancer patients were female and over 60 years old. Of all Non-melanoma skin cancer, 81,7% (n=1443) were located in sun-exposed skin, and represented 95,1% of malignant skin neoplasms in sun-exposed skin. Non-melanoma skin cancer was the most frequent malignant skin neoplasm in most topographic locations, except for abdomen and pelvis - over 95% of all malignant skin neoplasms in the face, neck and scalp were Non-melanoma skin cancer. Basal cell carcinomas were clearly predominant in all locations, except in upper and lower limbs, lower lip and genitals, where squamous cell carcinomas represented respectively 77,7%, 77,4%, 94,7% and 95,3% of the Non-melanoma skin cancers. CONCLUSION Being the most common skin cancer, Non-melanoma skin cancer should be under constant surveillance, in order to monitor its epidemiologic dynamics, the efficiency of preventive measures and the adaptation of the healthcare resources.

Value of patch tests in clindamycin-related drug eruptions

Background. Patch tests help to confirm the aetiology of the cutaneous adverse drug reactions involving delayed hypersensitivity mechanisms, but the results vary with the pattern of skin reaction and the culprit drug.

Papilomatose cutânea florida e acantose nigricante maligna reveladoras de neoplasia gástrica

4 Abstract: This paper reports the case of a 57-year-old, previously healthy male with no systemic symp- toms who over a short period of time developed multiple wart-like lesions on his trunk, limbs and face, typical lesions of acanthosis nigricans in the major body folds and tripe palms. Diagnostic tests revealed a metastatic gastric adenocarcinoma. Despite the implementation of therapy, which had a transient effect on the tumor and skin lesions, the patient died in 14 months. The association of these three para- neoplastic dermatoses (florid cutaneous papillomatosis, acanthosis nigricans maligna and tripe palms) in the same patient, apparently with a common pathogenic mechanism, is noteworthy. Keywords: Acanthosis nigricans, paraneoplastic syndromes; Stomach neoplasms Resumo: Apresenta-se o caso clinico de um doente de 57 anos, previamente saudavel, sem sintoma- tologia sistemica, que, num curto intervalo de tempo, desenvolve multiplas lesoes semelhantes a verru- gas virais no tronco, membros e face, lesoes tipicas de acantose nigricante nas grandes pregas e uma queratodermia difusa palmar com paquidermatoglifia. Os exames complementares de diagnostico rev- elaram uma neoplasia gastrica metastizada. Apesar da instituicao da terapeutica, com efeito transitorio na neoplasia e nas lesoes cutâneas, o doente viria a falecer em 14 meses. Salientamos a associacao destas tres dermatoses paraneoplasicas num mesmo paciente: papilomatose cutânea florida, acantose nigricante maligna e tripe palms que parecem ter um mecanismo patogenico comum. Palavras-chave: Acantose nigricans; Neoplasias gastricas; Sindromes paraneoplasicas

Prediction of Sentinel Node Status and Clinical Outcome in a Melanoma Centre

Background. Sentinel lymph node biopsy (SLNB) is a standard procedure for patients with localized cutaneous melanoma. The National Comprehensive Cancer Network (NCCN) Melanoma Panel has reinforced the status of the sentinel lymph node (SLN) as an important prognostic factor for melanoma survival. We sought to identify predictive factors associated with a positive SLNB and overall survival in our population. Methods. We performed a retrospective chart review of 221 patients who have done a successful SLNB for melanoma between 2004 and 2010 at our department. Univariate and multivariate analyses were done. Results. The SLNB was positive in 48 patients (21.7%). Univariate analysis showed that male gender, increasing Breslow thickness, tumor type, and absence of tumor-infiltrating lymphocytes were significantly associated with a positive SLNB. Multivariate analysis confirmed that Breslow thickness and the absence of tumor-infiltrating lymphocytes are independently predictive of SLN metastasis. The 5-year survival rates were 53.1% for SLN positive patients and 88.2% for SLN negative patients. Breslow thickness and the SLN status independently predict overall survival. Conclusions. The risk factors for a positive SLNB are consistent with those found in the previous literature. In addition, the SLN status is a major determinant of survival, which highlights its importance in melanoma management.

Mycobacterium chelonae Is an Ubiquitous Atypical Mycobacterium.

The type of cutaneous infection varies mainly according to the patients immune status, and the disseminated form is mostly found in the context of immunosuppression. We report the case of a 62-year-old male who was under long-term systemic corticosteroid therapy and presented with a 7-month history of multiple painless cutaneous lesions at various stages of development: papules, nodules, pustules and hemorrhagic crusts, as well as small erosions and ulcers distributed over the limbs and scalp. Cutaneous biopsy showed a suppurative granulomatous infiltrate with abscess formation. Fite stain revealed numerous extracellular bacilli, suggesting mycobacterial infection, particularly by atypical mycobacteria. Culture of a skin sample revealed Mycobacterium chelonae. The patient started multidrug therapy and showed clinical improvement despite of resistance to one of the antibiotics. This striking presentation underlines the role of immunosuppression with corticotherapy as a major risk factor for these infections. Multidrug therapy is advised and antibiogram is essential in directing treatment.

Synchronous pyoderma gangrenosum and inflammatory bowel disease, healing after total proctocolectomy

We present a female patient observed with painful violaceous plaques with central bullae and pustules on the lower limbs, rapidly transformed into ulcers, associated with bloody diarrhea, recurrent oral erosions and hyperthermia in the previous 3 months. Cutaneous biopsy was consistent with pyoderma gangrenosum, and intestinal diagnostic procedures revealed a non-classifiable inflammatory bowel disease with high x-ANCA titers. Soon after admission the patient was submitted to total proctocolectomy following colonic perforation. Complete ulcer healing occurred three months after surgery, without recurrence. Pyoderma gangrenosum is a rare dermatosis frequently associated with inflammatory bowel disease. This case is particularly interesting for the synchronic clinical presentation of cutaneous and intestinal diseases, but also for the prompt regression of the former after total proctocolectomy.

Histopathology of the Exanthema in DRESS Is Not Specific but May Indicate Severity of Systemic Involvement.

Objective:Exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) has no specific clinical diagnostic hallmark and there are few histopathologic studies. The aim of this study was to describe dermal–epidermal histopathologic features in DRESS and correlate them with the culprit drug, viral reactivation, or systemic organ involvement. Methods:Skin biopsies were independently evaluated by 2 dermatopathologists who characterized the main histological patterns and scored dermal and epidermal changes, which were further correlated with clinical and laboratorial data. Results:In 15 DRESS patients (9 male/6 female patients, mean age 53.3 years), the main observation was lymphocyte exocytosis (1.87 ± 1.25), spongiosis (0.93 ± 0.94), scattered keratinocyte necrosis (1.70 ± 1.44), basal cell vacuolization (2.13 ± 1.42), lymphocyte infiltration around dermal vessels (2.93 ± 0.92) or at the dermal–epidermal junction (2.07 ± 1.12), often with eosinophils and extravasated erythrocytes, swollen endothelial cells, and intravascular neutrophils but no vasculitis. Histopathologic patterns were classified mainly as spongiotic (5), erythema multiforme-like (3), or lichenoid (2). There was a significant positive correlation between the intensity of lymphocyte infiltration and the severity of hepatic cytolysis (r = 0.51; P < 0.05) and eosinophilia (r = 0.51; P < 0.05). No correlation was observed between the intensity and type of dermal inflammation and the degree of epidermal damage or the culprit drug. Human herpes virus type 6–positive patients had a pseudolymphomatous reaction or a perifollicular localization of the infiltrate. Conclusions:Histopathology in DRESS is variable with no specific diagnostic aspect, but there is a possible correlation between the intensity of the lymphocyte infiltrate and DRESS severity, namely, liver cytolysis.