An Unusual Cause of Papules on the Face.

Dear Editor, Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, locally proliferating disorder that affects predominantly the head and neck region (1,2). There seems to be a higher incidence in middle-aged Caucasian women (2,3). A 28-year-old female patient with no relevant personal or family medical history and only taking an oral contraceptive, presented to our department with multiple, well delimited, infracentimetric erythematous papules with a smooth surface on the left frontal, temporal, and preauricular regions (Figure 1). The lesions had appeared 7 months earlier, with progressive growth in number and dimensions since. The patient reported pruritus and denied previous trauma, topical application of any sort, insect bite at these locations, and any other accompanying symptoms. A thorough physical examination revealed no additional abnormalities. An excisional biopsy of one of the left temporal papules revealed a prominent lymphoid component, with a dense multinodular infiltrate in the superficial and deep dermis, with reactive germinative centers of considerable dimensions (Figure 2). Large and atypical lymphocytes were confined to the germinative centers, with reactive characteristics. Lymphocytes surrounding the germinative centers were predominantly small, accompanied by a significant number of scattered eosinophils. CD3 and CD20 immunohistochemical staining revealed B-cells predominantly in the nodular areas corresponding to the germinative centers, while T-cells displayed a diffuse peripheral distribution. There was severe neovascularization, with thick-walled vascular channels lined by enlarged plump endothelial cells with an "epithelioid" appearance. These findings supported the diagnosis of angiolymphoid hyperplasia with eosinophilia (ALHE). Laboratory workup did not show any abnormalities, including eosinophilia or elevation of immunoglobulin E levels. Due to pruritus and aesthetic concerns, surgical excision of the larger and most symptomatic papules was performed. The patient was assured of the benign nature of the disease and informed about the possible development of new lesions. Kept under clinical surveillance, the patient remained free of new lesions at 6-month follow-up. ALHE generally presents as solitary or multiple erythematous or hyperpigmented dome-shaped papulonodules. Lesions can be pruritic or painful and do not tend to resolve spontaneously (4). The pathogenesis of ALHE remains controversial, although some theories have been suggested. The most widely accepted hypothesis is that it is an angioproliferative process, accompanied by an inflammatory infiltrate, reactive to several stimuli (3). Some authors believe it is an allergic reaction, but no specific sole agent has been identified (5). Others claim ALHE may represent a T-cell lymphoproliferative disorder of benign or low-grade malignant nature (6). Some recent studies suggest that ALHE pathogenesis may be related to a vascular malformation secondary to a subcutaneous arteriovenous shunt (1-3). Histologically there are both vascular and inflammatory components, with an abnormal vascular proliferation and diffuse lymphocytic infiltrates with eosinophils. The vascular component is formed by capillaries clustered around arterial or venous vessels, dilated and atypical, with a protruded endothelium (3). The main differential diagnosis of ALHE is Kimura's disease, and there has been some discussion regarding the relationship between these two entities due to their clinical and histopathological similarities. However, most studies currently agree that they are distinct diseases. The differential diagnosis also includes angiosarcoma, particularly the epithelioid variant, epithelioid hemangioendothelioma, Kaposi sarcoma, pyogenic granuloma, and cutaneous metastasis (3). ALHE usually requires treatment as spontaneous regression, although reported in the literature, is rare (1,3). Many options have been suggested, with variable levels of success, but there is no definitive treatment for this condition (2). Surgical excision is the preferred choice, but recurrence may happen if the excision is incomplete (1). Mohs micrographic surgery with excision of abnormal vessels at the base of the lesion may be more effective in reducing recurrences (4). Other treatments reported include laser therapy (pulsed dye, CO2, copper vapor), systemic or intralesional corticosteroid injection, cryotherapy, imiquimod, tacrolimus, isotretinoin, radiotherapy, interferon alfa 2a, anti-interleukin-5 antibody, photodynamic therapy, and methotrexate (1). In the present case the diagnosis of ALHE was established through the conjunction of clinical and histological findings. Although a rare entity, its predominantly facial involvement in young adults and the absence of a satisfactory treatment can produce a significant impact that can include the quality of life of the patients.

Case for diagnosis. Multinucleated cell angiohistiocytoma.

Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Varicella zoster virus reactivation and the increased risk of cerebrovascular accidents: the unexpected role dermatologists can play.

Varicella zoster virus (VZV) primary infection usually causes varicella and its reactivation may lead to different clinical manifestations depending on the site of viral reactivation and its subsequent tissue spread. There is a growing recognition of the association between VZV reactivation and ensuing cerebrovascular accidents (CVA). The virus can spread to cerebral arteries, causing a wide clinical spectrum related to VZV vasculopathy. Herein we present an 80-year-old man with a previously undiagnosed immunosuppressive condition, admitted with disseminated herpes zoster, who subsequently developed an acute ischemic CVA and showed a substantial neurologic recovery under antiviral therapy.

Ashy dermatosis with involvement of mucous membranes.

Ashy dermatosis is a rare condition, of unknown aetiology, in which mucous membranes are typically spared. The authors report the case of a 57-year-old female with a history of asymptomatic gray-bluish macules located on the trunk and oral mucosa. There were no relief changes on examination. Skin biopsies from the oral mucosa and trunk were performed and both were compatible with ashy dermatosis. The patient started treatment with oral clofazimine but due to the absence of clinical improvement the drug was discontinued three months later. This case report illustrates an atypical case of ashy dermatosis owing to the involvement of mucous membranes, which is rarely described in the literature.

Multiple papulonodular lesions on central area of the face: what is your diagnosis?

A healthy 31-year-old woman presented with a 20-year history of asymptomatic skin-colored papules and nodules on the central area of the face. Her maternal grandmother, aunts, mother, and sister also had similar lesions. Clinical, histopathological, and genetic features allowed the diagnosis of multiple familial trichoepithelioma. The patient and family were referred to the genetic department for genetic counselling. Close follow-up for the possibility of secondary basal cell carcinoma is warranted.

Birt-Hogg-Dubé Syndrome - report of two cases with two new mutations.

INTRODUCTION: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant genodermatosis characterized by cutaneous fibrofolliculomas and/or trichodiscomas, lung cysts, spontaneous pneumothorax and renal tumors. However, its clinical expression is highly variable. This syndrome is caused by germline mutations in the folliculin gene (FLCN) on chromosome 17p11.2. MAIN OBSERVATIONS: Two men, 60 and 39-year-old, presented with a several year history of asymptomatic whitish papules scattered over the face and neck. Skin biopsies revealed fibrofolliculomas. The clinical diagnosis of BHDS was corroborated by identification of new heterozygotic mutations in FLCN gene, in exon 6 (C.573_574delinsT) and in exon 9 (c.1015C>T), respectively. Computed tomography scan of the thorax and abdomen showed pulmonary cysts with no suspicious kidneys lesions, and, in the case of the second patient, a mass in left adrenal gland. Laparoscopic left adrenalectomy was performed and histopathological examination was compatible with a malignant perivascular epithelioid cell tumor. CONCLUSIONS: The presence of multiple fibrofolliculomas should raise the suspicion of BHDS. Patients with this syndrome, regardless of the detected mutation, should be carefully monitored to ensure that potentially serious disease-related conditions can be detected early.

Ashy dermatosis with involvement of mucous membranes

Abstract: Ashy dermatosis is a rare condition, of unknown aetiology, in which mucous membranes are typically spared. The authors report the case of a 57-year-old female with a history of asymptomatic gray-bluish macules located on the trunk and oral mucosa. There were no relief changes on examination. Skin biopsies from the oral mucosa and trunk were performed and both were compatible with ashy dermatosis. The patient started treatment with oral clofazimine but due to the absence of clinical improvement the drug was discontinued three months later. This case report illustrates an atypical case of ashy dermatosis owing to the involvement of mucous membranes, which is rarely described in the literature.

Drug-induced photosensitivity: Photoallergic and phototoxic reactions.

Drug-induced photosensitivity refers to the development of cutaneous disease due to the interaction between a given chemical agent and sunlight. Photosensitivity reactions can be classified as phototoxic or photoallergic. Sometimes, there is an overlap between these two patterns, making their distinction particularly difficult for the clinician. We review the drugs that have been implicated as photosensitizers, the involved mechanism, and their clinical presentations. The main topical agents that cause contact photosensitivity are the nonsteroidal antiinflammatory drugs, whereas the main systemic drugs inducing photosensitivity are antimicrobials, nonsteroidal antiinflammatory agents, and cardiovascular drugs. Drug-induced photosensitivity remains a common clinical problem and is often underdiagnosed.

[Career Satisfaction of Medical Residents in Portugal]. / Satisfação com a Especialidade entre os Internos da Formação Específica em Portugal.

INTRODUCTION: The satisfaction with the medical profession has been identified as an essential factor for the quality of care, the wellbeing of patients and the healthcare systems' stability. Recent studies have emphasized a growing discontent of physicians, mainly as a result of changes in labor relations. OBJECTIVES: To assess the perception of Portuguese medical residents about: correspondence of residency with previous expectations; degree of satisfaction with the specialty, profession and place of training; reasons for dissatisfaction; opinion regarding clinical practice in Portugal and emigration intents. MATERIAL AND METHODS: Cross-sectional study. Data collection was conducted through the "Satisfaction with Specialization Survey", created in an online platform, designed for this purpose, between May and August 2014. RESULTS: From a total population of 5788 medical residents, 804 (12.25 %) responses were obtained. From this sample, 77% of the responses were from residents in the first three years. Results showed that 90% of the residents are satisfied with their specialty, 85% with the medical profession and 86% with their place of training. Nevertheless, results showed a decrease in satisfaction over the final years of residency. The overall assessment of the clinical practice scenario in Portugal was negative and 65% of residents have plans to emigrate after completing their residency. CONCLUSION: Portuguese residents revealed high satisfaction levels regarding their profession. However, their views on Portuguese clinical practice and the results concerning the intent to emigrate highlight the need to take steps to reverse this scenario.

Introdução: A satisfação com a profissão médica tem sido apontada como um fator essencial para a qualidade assistencial, o bemestar dos doentes e a estabilidade dos sistemas de saúde. Estudos recentes têm vindo a enfatizar um crescente descontentamento dos médicos, principalmente como consequência das alterações das relações laborais.Objetivos: Avaliar a perceção dos médicos de formação específica em Portugal, sobre as expectativas e grau de satisfação com a profissão, especialidade e local de formação; razões da insatisfação e intenção de emigrar.Material e Métodos: Estudo transversal. A colheita de dados foi efetuada entre Maio e Agosto de 2014 através de um Inquérito online sobre a âÄúSatisfação com a EspecialidadeâÄù.Resultados: De uma população total de 5788 médicos, foram obtidas 804 respostas (12,25% do total de médicos internos). Desta amostra, 77% das respostas correspondem a internos dos três primeiros anos de formação. Verificou-se que 90% dos médicos se encontram satisfeitos com a especialidade, tendo-se encontrado também níveis elevados de satisfação com a profissão (85%) e local de formação (86%). Por outro lado, constatou-se que estes diminuíam com a progressão ao longo dos anos de internato. A avaliação global sobre o panorama da prática médica foi negativa e 65% dos médicos responderam que consideram emigrar após conclusão do internato.Conclusão: Os médicos internos em Portugal apresentam níveis positivos de satisfação com a sua profissão. No entanto, a sua opinião sobre o panorama da Medicina e os resultados relativos à intenção de emigrar alertam para a necessidade de tomada de medidas para inverter este cenário.