Abstract Background Mohs Micrographic Surgery (MMS) is commonly used to treat high-risk basal cell carcinoma (BCC). Objectives Correlate clinicopathologic preoperative features with the number of MMS stages (primary endpoint) and margins (secondary endpoint) required for BCC complete excision. Methods We retrospectively analyzed BCCs treated by MMS in a 2-year period at the study’s institution. Variables studied included the patient gender, age, immune status, lesion size, location, if it was a primary, recurrent, or persistent tumor, histopathologic characteristics, number of surgical stages, and amount of tissue excised. Results 116 BCCs were included. The majority (61.2%, n = 71) required a single-stage surgery for complete clearance, requiring a final margins of 3.11 ± 2.35 mm. Statistically significant differences between locations in different high-risk areas (periocular, perioral, nose, ear) and the number of MMS stages required for complete excision (p = 0.025) were found, with periocular tumours requiring the highest mean of stages (2.29 ± 0.95). An aggressive histopathology significantly influenced the number of MMS stages (p = 0.012). Any significant relation between clinicopathological features and variation in the final surgical margins was found, just certain tendencies (male patients, persistent tumor, periocular location, and high-risk histopathological tumors required larger margins). Neither patient age or tumor dimension correlated significantly with both number of MMS stages and final surgical margins. Study limitations Limitations of this study include its single-center nature with a small sample size, which limits the value of conclusions. Conclusion Main factors related to a greater number of MMS stages were periocular location and high-risk histopathological subtype of the tumor.

Abstract Background: There are conflicting data regarding the prognostic value of the lymphatic basin drainage pattern in melanoma patients and the evidence is scant in the setting of negative sentinel lymph node biopsy. Objective: To investigate whether the pattern of lymphatic basin drainage influences the risk of nodal disease in patients with melanoma of the trunk and negative sentinel lymph node biopsy. Methods: A case series of patients with trunk melanoma and negative sentinel lymph node biopsy was retrospectively evaluated. Clinicopathological features, the pattern of lymphatic drainage and nodal, metastatic, and overall recurrence-free survival were reviewed. Results: Of the 135 patients included, multiple lymphatic basin drainage was identified in 61 (45.2%). Ten of the 74 (13.5%) patients with single drainage developed nodal recurrence, compared with 2 of the 61 (3.6%) patients with multiple drainages (p = 0.04). Nodal recurrence-free survival was significantly longer in the group with multiple drainages than in the group with single drainage (175.6 vs. 138.7 months; p = 0.04). In multivariate analysis, single drainage was associated with a higher risk of nodal recurrence (HR = 4.54; p = 0.05). No significant differences in metastatic and overall recurrence-free survival were found between groups. Study limitations: Retrospective analysis, single-center study, small sample, detailed histopathologic information not always present. Conclusions: In patients with trunk melanoma and negative sentinel lymph node biopsy, multiple lymphatic basin drainage may be an independent risk factor for nodal disease recurrence. This factor may help to identify patients with negative sentinel lymph node biopsy with a higher risk of nodal recurrence.

RESUMO O melanoma metastático tem sido associado a mau prognóstico, apresentando taxas de sobrevivência global aos 5 anos de 10%. Até 2011, os únicos tratamentos disponíveis para o melanoma metastático eram a quimioterapia e a imunoterapia com interleucina-2. O conhecimento mais aprofundado sobre a biologia molecular do melanoma e a identificação de mutações BRAF, que são as mais frequentemente encontradas, permitiram encontrar novos alvos terapêuticos que vieram alterar o prognóstico destes doentes. Atualmente, os tratamentos disponíveis para o melanoma metastático com mutação BRAF são a imunoterapia com inibidores do checkpoint imunológico (anti-PD-1 e anti-CTLA-4) e a terapêutica dirigida com inibidores BRAF e inibidores MEK. No entanto, permanece ainda controverso qual a terapêutica de primeira linha a instituir nestes doentes.

ABSTRACT Metastatic melanoma has been associated with a poor prognosis, with overall survival rates at 5 years of 10%. Until 2011, the only treatments available for metastatic melanoma were chemotherapy and immunotherapy with interleukin-2. The more in-depth knowledge about the molecular biology of melanoma and the identification of BRAF mutations, which are the most frequently found, allowed us to find new therapeutic targets that came to modify the prognosis of these patients. Currently, the treatments available for metastatic melanoma with BRAF mutation are immunotherapy with immunological checkpoint inhibitors (anti-PD-1 to anti-CTLA-4) and targeted therapy with BRAF inhibitors and MEK inhibitors. However, the first-line therapy to be instituted in these patients remains unknown.

Resumo A reconstrução de defeitos cutâneos nasais é um desafio mesmo para um cirurgião dermatológico experiente. Quando o defeito é grande ou demasiado complexo para ser corrigido com retalhos mais simples, os retalhos interpolados são uma boa opção, mas requerem habitualmente dois tempos operatórios. Os retalhos interpolados tunelizados foram desenvolvidos para permitir a reconstrução de defeitos complexos num só tempo cirúrgico. Na reconstrução nasal, os principais retalhos tunelizados são o frontal paramediano e o nasogeniano. Esta revisão incidirá sobre estes dois retalhos: descrição da técnica, aplicações, vantagens e desvantagens.

Abstract The reconstruction of nasal skin defects is a challenge even for an experienced dermatological surgeon. When the defect is too large or complex to be corrected with simpler flaps, interpolated flaps are a good option, but they usually require two surgeries. Tunneled interpolated flaps were developed to allow the reconstruction of complex defects in only one surgical procedure. In nasal reconstruction, the main tunneled flaps are the paramedian forehead and the melolabial flaps. This review will focus on these two flaps: description of the technique, applications, advantages and disadvantages.

Abstract The reconstruction of facial surgical defects is usually challenging for the dermatologic surgeon. Three different cases of facial defects in which tunneled island flaps were used are reported. In 2 cases, wide defects involving the nasal dorsum and ala were repaired using a nasolabial island flap tunneled through the lateral side of the nose. A tunneled island glabellar flap was used for medial canthus reconstruction in the third case. Despite complex pedicle dissection and frequent trapdoor deformation, tunneled island flaps allow reconstruction of wide defects in a single-staged procedure, camouflaging the scar of the donor area in boundaries of cosmetic units and preserving the facial central symmetry.

Introdução: A biopsia renal e fundamental na abordagem diagnostica e no prognostico de doentes com patologias nefrológicas. No sentido de avaliar a epidemiologia das doenças renais na Região Autónoma da Madeira e a sua evolução, analisou-se as biopsias de rim nativo nos ultimos vinte e sete anos. Métodos: Procedeu-se a análise retrospectiva dos registos e laminas histológicas de 1986-2012, com avaliação da evolução temporal na qualidade/indicações para biopsia renal e padrões de patologias nefrologicas. Resultados: A amostra compreende 315 biopsias de rim nativo, sendo a idade media dos doentes de 40.8 ± 18.4 anos, sendo 50.5% (n = 159) do sexo masculino. As síndromes nefrologicas mais frequentes foram a sindrome nefrotica (36.2%, n = 114) e lesão renal aguda (20.0%, n = 63). Das patologias nefrologicas mais frequentes, destacam-se no grupo das glomerulopatias primárias (41.5%, n = 115), a nefropatia de IgA (26.1%, n = 30) e a glomeruloesclerose segmentar e focal (17.4%, n = 20) e nas glomerulopatias secundarias (31.4%, n = 87), a nefrite lupica (51.7%, n = 45) e amiloidose (20.7%, n = 18). Na analise estatistica, realca-se a correlação significativa entre sexo e grupos de sindromes nefrologicas (Fisher’s exact test, p<.01) e entre grupos de sindromes nefrologicas e diagnostico patologicos (χ2, p <.01). Avaliando a evolução temporal, não se objectivaram diferencas estatisticamente significativas em relação ao numero de biopsias renais (χ2, p =.193), numero de glomerulos na amostra (Fisher’s exact test, p =.669), idade (Kruskal-Wallis, p =.216), sindromes nefrologicas (χ2, p =.106) ou grandes grupos de diagnostico patologicos (χ2, p =.649). No entanto, considerando os dignósticos patologicos especificos e a sua variação temporal foram encontradas diferencas significativas (Fisher’s exact test, p <.05) para nefrite lupica e nefropatia membranosa com uma incidencia crescente e para a amiloidose com uma tendência oposta. Discussão: A analise do registo de biopsias renais de uma população com características particulares, isolada geograficamente, permitiu mostrar concordância com outros números epidemiológicos referentes a outras subpopulações europeias, permitindo simultaneamente uma compreensão das directrizes locais sobre biopsias renais.

Introduction: Renal biopsy plays an essential role either in the diagnosis or in the prognosis of patients with renal disease. In order to assess its epidemiology and evolution in Madeira Islands, we analysed twenty-seven years of native kidney biopsies. Methods: We performed a retrospective analysis of clinical records, including histological revision from 1986 to 2012, totalling 315 native kidney biopsies. They were assessed regarding the temporal evolution both for the quality/indications for renal biopsy and for the patterns of kidney disease. Results: A total of 315 native kidney biopsies were analysed. The patients’ mean age was of 40.8 �± 18.4 years and 50.5%(n = 159) were males. The most common indications for renal biopsy were nephrotic syndrome (36.2%, n = 114) and acute kidney injury (20.0%, n = 63). Among primary glomerular diseases (41.5%, n = 115) the most common were IgA nephropathy (26.1%, n = 30) and focal-segmental glomerulosclerosis (17.4%, n = 20) and among secondary glomerular diseases (31.4%, n = 87), lupus nephritis (51.7%, n = 45) and amyloidosis (20.7%, n = 18). Statistical analysis revealed significant correlation between gender and major pathological diagnosis (Fisher’s exact test, p <.01) and between indications for renal biopsy and major pathological diagnosis (χ2, p <.01). Regarding the temporal evolution, no statistically significant differences were found in the number of renal biopsies (χ2, p =.193), number of glomeruli per sample (Fisher’s exact test, p =.669), age (Kruskal-Wallis, p =.216), indications for renal biopsy (χ2, p =.106) or major pathological diagnosis groups (χ2, p =.649). However, considering the specific clinico-pathological diagnoses and their temporal variation, a statistically significant difference (Fisher’s exact test, p <.05) was found for lupus nephritis and membranous nephropathy with an increasing incidence and for amyloidosis with an opposite tendency. Discussion: The review of the native kidney biopsies from a population with particular characteristics, geographically isolated, such as those from Madeira Islands, showed parallel between epidemiological numbers referring to other European subpopulations, allowing simultaneously a comprehensive approach to our renal biopsy policies

Pemphigoid gestationis is a rare, autoimmune blistering dermatosis of pregnancy. No increase in fetal or maternal mortality has been demonstrated, but a greater prevalence of premature and small-for-gestationalage babies has been reported. Topical and systemic corticosteroids and antihistamines are the manstay of treatment. The authors report a case of a 27-year-old woman at 28-weeks gestation with sudden onset of pruriginous vesicles and blisters in the abdomen and limbs. Systemic corticosteroids were introduced and maintained throughout gestation to prevent flares and tapered after the birth of a healthy child.

O lóbulo auricular é uma estrutura anatómica com uma importância estética significativa. O desafio inerente à sua reconstrução relaciona-se com a dificuldade em obter um resultado duradouro e cosmeticamente aceitável. Os autores apresentam duas opções: a técnica de Gavello e o retalho bilobado, após excisão de neoplasias malignas do lóbulo auricular. A técnica de Gavello, recorre a um retalho bilobado com base anterior, para constituir o novo lóbulo auricular. O retalho bilobado de D'Hooghe, com lobos pré e pós-auriculares, permite a reconstrução de defeitos de pequenas dimensões. As duas técnicas descritas, apesar de antigas, mantém-se actuais pela execução de grau de dificuldade média, em tempo cirúrgico único, sob anestesia local com a obtenção de resultados cosmeticamente aceitáveis.

The earlobe is an anatomical structure that has a significant aesthetic role. Its surgical repair places a challenge due to the difficulty of obtaining a natural appearing and durable outcome. The authors present two options: the Gavello technique and the bilobed flap, after the excision of malign neoplasms of the earlobe. The Gavello technique makes use of a bilobed flap with an anterior base to mold the new earlobe. D'Hooghe's bilobed flap with a pre and post-auricular lobe allows the reconstruction of small earlobes. Both techniques, although old, acquire an important and current interest in earlobe reconstruction, by reason of the moderate degree of difficulty, the use of a single time surgical act and under local anesthesia, with a proper aesthetic result.

A Úlcera de Lipschütz, ou ulcus vulvae acutum, é uma entidade rara mas provavelmente subdiagnosticada, que se apresenta como úlcera vulvar aguda dolorosa numa mulher jovem. A etiologia é desconhecida embora relatos recentes a tenham associado à primo-infecção pelo vírus Epstein-Barr. O diagnóstico é estabelecido após exclusão de doenças sexualmente transmissíveis, causas autoimunes, traumáticas e outras etiologias de úlceras genitais. Relatamos o caso de uma jovem que desenvolveu quadro gripal e dolorosas úlceras genitais. Os exames complementares excluiram doenças sexualmente transmissíveis e as outras causas habituais de ulceração genital. As lesões cicatrizaram sem sequelas e sem recorrências. Este caso representa um diagnóstico diferencial importante e raro de ulceração genital.

Lipschütz Ulcer, or ulcus vulvae acutum, is a rare and probably underdiagnosed entity that usually presents as an acute painful vulvar ulcer in young women. The etiology is unknown, although recent reports have associated it with the Epstein-Barr virus. The diagnosis is made by exclusion after ruling out sexually transmitted diseases, autoimmune causes, trauma, and other etiologies of genital ulcerations. We report a case of a young woman who developed flu-like symptoms and painful vulvar ulcers. Complementary examinations ruled out sexually transmitted diseases and the other usual causes of genital ulcers; lesions healed with no sequelae or recurrences. This case represents a rare important differential diagnosis of genital ulceration.

A abordagem de defeitos cirúrgicos da região perioral, em particular das comissuras labiais, é um desafio importante na prática cirúrgica dermatológica. Apresentamos o caso de um doente do sexo masculino, de 69 anos, com extenso carcinoma basocelular ulcerado da região perioral direita, com envolvimento da comissura labial. Foi realizada excisão radical da lesão e reconstrução do defeito cirúrgico com retalho romboidal duplo das regiões geniana e mandibular homolaterais. O resultado cosmético final foi satisfatório, com preservação da funcionalidade das estruturas labiais. O retalho romboidal duplo é uma alternativa viável para a reconstrução de defeitos cirúrgicos da comissura labial, de execução relativamente simples, com baixo risco de complicações, que oferece óptimos resultados funcionais e cosméticos, sem distorção da anatomia labial.

Closure of perioral surgical defects involving the oral commissures is highly challenging. We describe a 69-year-old male patient with a large basal cell carcinoma of the right perioral region, extending to the right oral commissure. This lesion was radically excised, and the resulting surgical defect was closed using a homolateral double opposing rhomboidal flap. The final result was esthetically very satisfactory, with total preservation of lip function. Double opposing rhomboidal flaps are viable surgical options for the reconstruction of surgical defects involving the perioral area and oral commissures. In this relatively simple procedure, donor skin is obtained from the nearby cheek and mandibular areas, under low risk of surgical complications, preserving lip function without distortion of the labial anatomy.

O carcinoma de células escamosas é a neoplasia maligna mais frequente dos lábios, e em cerca de 90% dos casos, localiza-se no lábio inferior, por causa da maior exposição cumulativa à radiação ultravioleta. Os autores apresentam duas técnicas cirúrgicas para a reconstrução de grandes defeitos do lábio inferior, resultantes da excisão cirúrgica tumoral, exemplificando-as e comparando-as através de dois casos clínicos

Squamous cell carcinoma is the most common malignant neoplasm of the lips, and in about 90% of cases it is located on the lower lip due to higher cumulative exposure to ultraviolet radiation. The authors present two surgical techniques for reconstruction of large lower lip defects, resulting from surgical excision of tumors, exemplifying and comparing them with two clinical cases

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