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 Parkinson's disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinson's 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. .

Synchronous pyoderma gangrenosum and inflammatory bowel disease, healing after total proctocolectomy / Pioderma gangrenoso associado a doença inflamatória intestinal, com resolução após proctocolectomia total

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.

Apresentamos uma paciente do sexo feminino observada com múltiplas placas violáceas dolorosas dos membros inferiores, com bolhas e pústulas evoluindo rapidamente para lesões ulceradas, surgindo no contexto de diarreia sanguinolenta, erosões orais recorrentes e febre com três meses de evolução. A biópsia cutânea foi compatível com pioderma gangrenoso; o estudo complementar revelou doença inflamatória intestinal inclassificável com títulos elevados de x-ANCA. Após perfuração cólica, a doente foi submetida a proctocolectomia total, com rápida cicatrização das lesões cutâneas ulceradas em três meses, sem recorrência. O pioderma gangrenoso é uma dermatose rara frequentemente associada a doença inflamatória intestinal. É interessante verificar neste caso a apresentação clínica sincrónica das doenças cutânea e intestinal, bem como a rápida resolução da primeira após proctocolectomia total.

Epidemiology of basal cell carcinomas and squamous cell carcinomas in a Department of Dermatology: a 5 year review / Epidemiologia dos carcinomas basocelulares e espinhocelulares em um Serviço de Dermatologia: revisão de 5 anos

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.

FUNDAMENTOS: O cancro cutâneo não-melanoma, designação conjunta para os carcinomas basocelulares e espinhocelulares, é o tipo de neoplasia cutânea maligna mais frequente. OBJETIVOS: Caracterização epidemiológica da população diagnosticada com cancro cutâneo não-melanoma. MÉTODOS: Foi realizada uma análise retrospectiva dos doentes portadores de cancro cutâneo não-melanoma identificados por análise histológica de todas as biopsias cutâneas incisionais ou excisionais ao longo de 5 anos (2004-2008) num serviço de Dermatologia. RESULTADOS: foram identificados 3075 cancros cutâneos não-melanoma, representando 88% do total de neoplasias malignas diagnosticadas no mesmo período (n=3493). Destes, 68,3% eram carcinomas basocelulares. No seu conjunto, a população de cancro cutâneo nãomelanoma era predominantemente constituída por indivíduos idosos e do sexo feminino, tendo sido observado um aumento consistente da freqüência ao longo do período avaliado (5,25%/ano). A maioria dos cancros cutâneos não-melanoma (n=1443, 81,7%) foi identificada nas áreas de pele foto-exposta, representando 95,1% de todas as neoplasias malignas em áreas foto-expostas. O cancro cutâneo não-melanoma foi a neoplasia mais representativa na generalidade das áreas topográficas, à exceção do abdômen e da pélvis, representando, em particular, mais de 95% das neoplasias malignas da face, da região cervical e do couro cabeludo. O carcinoma basocelular foi o cancro cutâneo não-melanoma predominante em todas as localizações, à exceção dos membros inferiores e superiores, lábio inferior e da área genital, onde o carcinoma espinhocelular representou, respectivamente, 77,7%, 77,4%, 94,7% e 95,3% dos casos. CONCLUSÕES: O cancro cutâneo não-melanoma, como neoplasia maligna cutânea mais freqüente, deverá ser alvo de uma monitorização regular, com vista à determinação da sua dinâmica epidemiológica, da eficácia das medidas preventivas e adequação dos recursos de saúde.

Papilomatose cutânea florida e acantose nigricante maligna reveladoras de neoplasia gástrica / Florid cutaneous papillomatosis and acanthosis nigricans maligna revealing gastric adenocarcinoma

Apresenta-se o caso clínico de um doente de 57 anos, previamente saudável, sem sintomatologia sistêmica, que, num curto intervalo de tempo, desenvolve múltiplas lesões semelhantes a verrugas virais no tronco, membros e face, lesões típicas de acantose nigricante nas grandes pregas e uma queratodermia difusa palmar com paquidermatoglifia. Os exames complementares de diagnóstico revelaram uma neoplasia gástrica metastizada. Apesar da instituição da terapêutica, com efeito transitório na neoplasia e nas lesões cutâneas, o doente viria a falecer em 14 meses. Salientamos a associação destas três dermatoses paraneoplásicas num mesmo paciente: papilomatose cutânea florida, acantose nigricante maligna e tripe palms que parecem ter um mecanismo patogênico comum.

This paper reports the case of a 57-year-old, previously healthy male with no systemic symptoms 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 paraneoplastic dermatoses (florid cutaneous papillomatosis, acanthosis nigricans maligna and tripe palms) in the same patient, apparently with a common pathogenic mechanism, is noteworthy.