João Alves

Variants of dermatofibroma - a histopathological study

Several variants of dermatofibroma have been described. They are essentially distinguished by their clinical and histopathological features. To review the mainfeaturesof these variants, a retrospective study of skin biopsies and tissue excisions of dermatofibromasperformed in the dermatology and venereology service at the Hospital Garcia de Orta between May 2007 and April 2012 was carried out. During that period, 192 dermatofibromas were diagnosed in 181 patients, the lesions being more common in women. Median age of the study population was 48 years. The most common lesion site was the limbs (74% of patients). The histopathological types found were common fibrous histiocytoma (80%) and the aneurysmal (5.7%),hemosiderotic (5.7%), epithelioid (2.6%), cellular (2.1%), lipidized (2.1%), atrophic (1.0) and clear cell (0.5%) variants. Based on these findings, this review focuses on the clinical and histological features of the various variants of dermatofibroma in terms of their clinical presentation, distinct histopathological features, differential diagnosis and prognosis.

Malignant Lues in an immunocompetent patient

Malignant lues is a rare form of secondary syphilis mostly associated with HIV infection. It is an uncommon presentation of syphilis even rarer in immunocompetent patients. We present the case of a 57-year-old homosexual man referred to our department due to a 4-month history of a disseminated, slightly painful, nodular-ulcerative cutaneous eruption associated with low-grade fever, malaise and aesthenia. Regarding the clinical features and serological and histopathological findings, the diagnosis of syphilis maligna was assumed. Serology for HIV was repeatedly negative. This case is interesting, not only because a very uncommon form of secondary syphilis was identified but also for being diagnosed in an immunocompetent patient. Lack of awareness of this type of presentation delays the diagnosis and treatment, leading to an increase in morbidity and spread of infection.

Ovarian carcinoma presenting as cutaneous nasal metastasis

Metastatic ovarian cancer uncommonly presents with skin metastasis. When present, skin metastases of ovarian cancer are usually localized in the vicinity of the primary tumor. We report a case of a 58-year-old woman with a rapid growing erythematous, well-defined nodule localized on the left nasal ala. A skin biopsy was performed and histopathological and immunohistochemical findings were compatible with a cutaneous metastasis of adenocarcinoma. A systematic investigation revealed a bilateral ovarian cystadenocarcinoma associated with visceral dissemination, likely associated with nose cutaneous metastasis. We report a very uncommon case because of the presentation of ovarian carcinoma as cutaneous metastasis. To our knowledge, this atypical localization on the nose has not been described yet in the literature.

Fixed eruption induced by sunlight

Fixed drug eruption (FDE) was first described by Brocq in 1894 [1]. Since then, a number of drugs have been implicated in the development of well-demarcated, erythematous, cutaneous or mucosal plaques that recur at the same site each time the respective drug is administered [2, 3]. The term fixed food eruption was introduced in 1996. Several foods have since then been described as possible triggers of fixed druglike eruptions [4, 5]. In 1982, sunlight was also reported as triggering factor in an eruption clinically similar to FDE [6]. Recently, in 2010, a sunlight-induced dermatosis, designated as fixed sunlight eruption, was described [7]. We report the case of a 22-year-old woman, who presented with a persistent hyperpigmented eruption on the hips and thighs that had first appeared three years earlier. Initially, cutaneous lesions – burning and pruritic, erythematous plaques – had occurred after sun exposure and resolved within one week, leaving pigmented patches. The patient stated that acute lesions had since then always recurred 3 to 4 hours after sun exposure of the affected sites. She had no significant past medical history and denied the intake of any kind of medication or any unusual diet, either before the first event or prior to subsequent episodes. At initial presentation, physical examination revealed isolated, well-demarcated, hyperpigmented, round to oval macules, measuring 2 to 8 cm, located on the inner thighs, hips and buttocks (Figure 1); skin type III (according to Fitzpatrick). The patient was reevaluated three days after recreational sun exposure of both affected and unaffected skin that had resulted in a flare-up of preexisting lesions, displaying an erythematous-violaceous hue with red borders and a purple center (Figure 2). Histopathology of an active lesion showed features consistent with fixed drug eruption or fixed druglike eruption (Figure 3). Laboratory analysis – including hepatitis B and C, HIV, ANA, double-stranded DNA, SSA/Ro, SSB/La, anti-mitochondrial antibodies, and lupus anti-coagulant – was within normal limits. To prove the causative effect of sunlight, phototesting was performed. The patient was instructed not to take any medication or change her usual diet in the days leading up to the test. As UVA-UVB source, we used a full body phototherapy unit (Waldmann UV7001K, Villingen-Schwenningen, Germany) with UVB narrowband (TL/01) fluorescent bulbs and F 85/100 W-PUVA lamps (Waldmann). After determining the patient’s minimal erythema dose to UVB (MED-B) (350 mJ/cm2), affected and unaffected skin was exposed to 10 % of MED-B (35 mJ/cm2). While there was a flare-up of preexisting lesions four hours after exposure, no new lesions developed on previously unaffected skin. Once acute lesions had resolved a week later, the patient was exposed to UVA (5 J/cm2), followed by reactivation of lesions five hours later. Here again, exposure of unaffected skin did not trigger any lesions. A biopsy taken from one of the reactivated lesions two days later yielded the same histologic findings as before. Visible light phototesting was performed a week later using a slide projector (Toshiba TDP-S8 800H; Toshiba, Irvine, CA, USA) with a high-pressure mercury lamp (200 W). Thirty-minute exposure, at a distance of 15 cm from both

Scleredema associated with Sjögren's syndrome *

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

Perforating Granuloma Annulare — An Unusual Subtype of a Common Disease

Perforating granuloma annulare (GA) is a rare subset of GA with an unknown etiology and chronic course. Herein, we report the case of 72 year-old women with a 3-month history of a post-traumatic, persistent, erythematous and exudative plaque located on her left leg. Differential diagnosis included mycobacterial infection, subcutaneous mycosis, perforating dermatoses, pyoderma and squamous cell carcinoma. The histopathology was highly suggestive of a perforating GA. The patient was treated with betamethasone dipropionate cream applied once daily and a complete resolution of the lesion was observed in three weeks. Despite being a very rare subtype of a common disease, perforating granuloma annulare has clinical and histopathological characteristic features that facilitate the differential diagnosis, avoiding unnecessary procedures and inadequate and potentially more invasive treatments.

Solitary ulcerated plaque on the face - an unusual presentation of cutaneous plasmacytosis?

Cutaneous and systemic plasmacytosis is a rare disorder characterized by cutaneous polyclonal plasma cell infiltration frequently associated with polyclonal hypergammaglobulinemia and lymphadenopathy. We report a case of a 67-year-old woman with an inflammatory ulcerated plaque in the left masseter region. A skin biopsy showed dense perivascular infiltrate of mature plasma cells in the dermis without atypia and immunoglobulin light chain restriction. After physical examination and further investigation, we ruled out systemic disease. Our patient was successfully treated only with hydrocortisone cream application. Few cases of isolated benign primary cutaneous plasmacytosis have been described, particularly in children. After excluding the diagnosis of a reactive process to an infection, which is unlikely in this case, we suspected of a rare manifestation of primary cutaneous plasmacytosis in adults with distinct presentation and clinical course.

Proctite e Infeção Anoretal por Chlamydia trachomatis e Neisseria gonorrhoeae em HSH – Estudo Retrospetivo

Introducao : As infecoes sexualmente transmissiveis (IST) em homens que tem sexo com homens (HSH) tem aumentado nas ultimas decadas, nomeadamente as provocadas por Neisseria gonorrhoeae (NG) e por Chlamydia trachomatis (CT), ambas responsaveis por proctites e infeccoes anorectais. Material e Metodos : Analise retrospetiva da frequencia de proctite e infeccao anoretal por NG e CT em 2420 HSH observados na Consulta de Doencas Sexualmente Transmissiveis do Centro de Saude da Lapa, entre Janeiro de 2008 e Dezembro de 2013. Sao avaliados dados sociodemograficos, comportamentais, clinicos e laboratoriais. Resultados : Foram encontrados sinais e/ou sintomas de proctite em 1,6% (38/2420) dos HSH, nao tendo sido identificado qualquer agente infeccioso em 39,4% (15/38) destes doentes. Em 38 (16%) dos 238 HSH a infeccao por NG teve localizacao anoretal; este local anatomico revelou-se infetado por CT em 23 (14,6%) dos 158 casos positivos, tendo sido identificado o serovar L2b em 7 dos 23 (30,4%). As infecoes anorectais foram mais frequentes em doentes com antecedentes de IST, multiplos parceiros sexuais e imigrantes. Salienta-se a elevada percentagem de HSH infetados e assintomaticos ou com sinais/sintomas pouco sugestivos de proctite: 55,3% (21 / 38) e 47,8%, (11 / 23) dos infetados por NG e CT, respetivamente. Conclusoes : A frequencia da infecao anoretal assintomatica por CT e NG nos HSH que recorrem a consulta DST do CS Lapa e semelhante a descrita para consultas similares noutros paises e justifica a necessidade da pesquisa daqueles agentes e de estirpes LGV de CT quando e referido contacto anal recetivo.

Total earlobe reconstruction with a superiorly based preauricular flap

An 86-year-old woman was referred for treatment of a lentigo maligna melanoma on the left earlobe, confirmed by cutaneous biopsy. The resulting surgical defect involved the earlobes full thickness. The authors outline a simple method for reconstructing the entire lobe in one stage, without grafts, whilst offering a pleasant appearance. Earlobe deformity may be congenital or, more often, acquired due to trauma, burns or surgery. Ear lobules are an important reference point for facial symmetry and they serve decorative purposes like wearing earrings. Losing them represents an obvious aesthetic abnormality. Several methods developed to reconstruct this deformity have presented various advantages and disadvantages.

MELANOMA MALIGNO: ANÁLISE RETROSPECTIVA 2006-2013

Introduction: The incidence of malignant melanoma has been rising. Therefore, the knowledge of its prevalence is very important. The authors intended to study the prevalence, demographic variables and the clinical features of the malignant melanoma diagnosed in their department.Material and methods: A retrospective study of the MM diagnosed between 2006 and 2013 was performed. The data was statistically an analysed.Results: three hundred and forty one malignant melanomas were diagnosed in 302 patients, 189 in women. The average annual growth rate was 8.57%, with 27 MM in 2006 and 48 in 2013. The older age groups predominated. Overall the trunk was the most frequent location. The Breslow thickness averaged 1.18mm. A statistically significant increase in the proportion of thin malignant melanomas ( 2mm remained unaltered. Nodal disease (stage III) was evident in 36 patients. Only two patients had metastatic disease (stage IV).Discussion: The number of malignant melanomas diagnosed grew above the reported national and international incidence growth. The average Breslow thickness, above 1 mm, remains high. Nonetheless, the proportional increase in malignant melanomas <1mm points towards a positive evolution.Conclusion: Our data, though representing a limited population, may lead to conclusions on the efficacy of the prevention strategies regarding malignant melanoma.Introduction: The incidence of malignant melanoma has been rising. Therefore, the knowledge of its prevalence is very important. The authors intended to study the prevalence, demographic variables and the clinical features of the malignant melanoma diagnosed in their department. Material and methods: A retrospective study of the MM diagnosed between 2006 and 2013 was performed. The data was statistically an analysed. Results: three hundred and forty one malignant melanomas were diagnosed in 302 patients, 189 in women. The average annual growth rate was 8.57%, with 27 MM in 2006 and 48 in 2013. The older age groups predominated. Overall the trunk was the most frequent location. The Breslow thickness averaged 1.18mm. A statistically significant increase in the proportion of thin malignant melanomas ( 2mm remained unaltered. Nodal disease (stage III) was evident in 36 patients. Only two patients had metastatic disease (stage IV). Discussion: The number of malignant melanomas diagnosed grew above the reported national and international incidence growth. The average Breslow thickness, above 1 mm, remains high. Nonetheless, the proportional increase in malignant melanomas <1mm points towards a positive evolution. Conclusion: Our data, though representing a limited population, may lead to conclusions on the efficacy of the prevention strategies regarding malignant melanoma.