Abstract Patients with lepromatous or borderline leprosy may present two types of vasculonecrotic reactions: Lucio’s phenomenon (LP) and necrotic erythema nodosum leprosum (nENL). These are serious conditions, which mostly lead to life-threatening infectious and thrombotic complications. The authors report the case of a patient with leprosy recurrence associated with an atypical type II reaction with LP characteristics on histopathology.

Abstract Secondary osteoma cutis is a phenomenon that may occur in several conditions. When it occurs in a melanocytic nevus it is named osteonevus of Nanta, an event considered uncommon and characterized by the presence of bone formation adjacent or interposed with melanocytic cells. There are reports of its occurrence in various melanocytic lesions, being more frequently associated with intradermal nevus. We report a case of osteonevus of Nanta in combined nevus, possibly the first description of this association.

Abstract We report a rare case of subcutaneous phaeohyphomycosis caused by Cladophialophora bantiana in an immunocompetent patient in Amazonas, Brazil. This dematiaceous fungus has been mainly associated with life-threatening infections affecting the central nervous systems of immunosuppressed patients. We present the clinical, laboratory, and therapeutic aspects, and in vitro susceptibility test results for different antifungal drugs. A brief review of the cases reported in the literature over the past 20 years has also been discussed. According to the literature review, the present case is the first report of subcutaneous phaeohyphomycosis due to C. bantiana in an immunocompetent patient in Latin America.

Abstract: A few cases of pigmented squamous cell carcinoma affecting the skin and the ocular and oral mucosa of the elderly have been described in the literature. The disease manifests itself as papular and nodular erythematous or pigmented lesions. The main clinical differential diagnoses are pigmented basal cell carcinoma and melanoma. Histopathological examination is characterized by proliferation of atypical squamous cells with formation of horn pearls permeated by dendritic melanocytes. For this reason, a careful diagnosis is necessary to rule out other tumors that show melanin pigment. Surgical removal is the indicated treatment and the prognosis is similar to the classical squamous cell carcinoma. We report a case of pigmented squamous cell carcinoma with diagnosis confirmed by histological and immunohistochemical examinations.

Abstract Rosacea fulminans or pyoderma faciale is a rare cutaneous disorder that usually affects women usually between the ages of 15-46. The disease is characterized by sudden onset of papules, pustules, cysts, and painful coalescing nodules with red-cyanotic centrofacial erythema. Although its etiology remains unknown, hormonal, immunological, and vascular factors have been reported. Early diagnosis and prompt treatment should minimize unsightly scars. We report a case of a 33-year-old female patient treated with traditional doses of doxycycline, with improvement of the lesions and regression of the condition in two months.

Abstract Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

Abstract Lichen sclerosus is a chronic inflammatory disease, usually located in the genital area. The etiology of lichen sclerosus is multifactorial, with participation of genetic, autoimmune, infectious and hormonal factors. Bullous clinical form stems from hydropic degeneration of the basal membrane, constituting a less frequent variant of the disease. In this work, we report the case of a female patient, 55 years old, who in the last three years presented whitish plaques, with horny spikes, located on back and arms. Some of these lesions evolved with hemorrhagic blisters, which after histopathological examination confirmed the diagnosis of bullous and hemorrhagic lichen sclerosus. The patient was treated with high-potency topical corticosteroid for two months, resulting in remission of bullous and hemorrhagic lesions.

BACKGROUND: Leprosy is characterized histologically by a spectrum of different granulomatous skin lesions, reflecting patients' immune responses to Mycobacterium leprae. Although CD4+CD25+ FoxP3+ T regulatory cells are pivotal in the immuneregulation, presence, frequency, and distribution of Tregs in leprosy, its reactional states have been investigated in few studies. OBJECTIVES: This study aimed to verify the frequency and distribution of regulatory T cells in different clinical forms and reactional states of leprosy. METHODS: We performed an immunohistochemical study on 96 leprosy cases [Indeterminate (I): 9 patients; tuberculoid tuberculoid: 13 patients; borderline tuberculoid: 26 patients; borderline borderline: 3 patients; borderline lepromatous: 8 patients; lepromatous lepromatous: 27 patients; reversal reaction: 8 patients; and erythema nodosum leprosum: 2 patients]. RESULTS: FoxP3-positive cells were present in 100% of the cases with an average density of 2.82% of the infiltrate. Their distribution was not related to granulomatous structures or special locations. There was a statistically significant increment of FoxP3 expression in patients with leprosy reversal reactions when compared with patients presenting with type I leprosy (P= 0.0228); borderline tuberculoid leprosy (P = 0.0351) and lepromatous leprosy (P = 0.0344). CONCLUSIONS: These findings suggest that Tregs play a relevant role in the etiopathogenesis of leprosy, mainly in type I leprosy reaction.

The livedoid vasculopathy is an obstructive vascular process of etiology not yet fully known, being possibly associated with several prothrombotic events. It is clinically characterized by the presence of painful and recurring purpuric lesions, which usually suffer ulceration and evolve with formation of white atrophic scars usually located in the lower limbs. Two cases are here reported of painful ulcerated lesions on the lower limbs, in which the identification of VL enabled the diagnosis of systemic diseases.

A miíase é uma doença causada por infestação de larvas de moscas nos tecidos humanos ou de outros animais vertebrados. É dermatose comum em países tropicais e subtropicais e tem como fatores predisponentes: doenças crônicas, imunodeficiência, má higiene, senilidade, doenças psiquiátricas, cânceres cutâneos e de mucosas ulcerados. Relata-se caso de paciente hígido que após trauma sobre lesão pré-existente, apresentou tumoração na região dorsal parasitada por larvas de moscas. O exame histopatológico realizado para o diagnóstico da neoplasia, de modo surpreendente, evidenciou a presença de uma larva parcialmente degenerada com características de Dermatobia hominis, sugerindo associação de miíase primária e secundária em carcinoma basocelular.

Myiasis is a disease caused by infestation of fly larvae in human and other vertebrate tissues. It is a skin condition common in tropical and subtropical countries and its predisposing factors are: chronic diseases, immunodeficiency, poor hygiene, senility, psychiatric disorders, skin cancers and ulcerated mucosae. We report the case of a healthy patient who after traumatic injury of a preexisting lesion showed a tumor on the dorsal region parasitized by fly larvae. The histopathological examination performed for the diagnosis of skin neoplasm surprisingly revealed the presence of a partially degenerated larva with characteristics of Dermatobia hominis, suggesting an association of primary and secondary myiasis on basal cell carcinoma.

FUNDAMENTOS: As doenças de pele estão associadas a alta morbidade, baixa mortalidade e baixa proporção de hospitalização. Entretanto, podem causar considerável interferência no bem-estar físico e emocional do indivíduo. Várias delas atingem grandes contingentes populacionais, havendo necessidade de intervenções específicas para seu controle. OBJETIVO: Descrever a frequência das dermatoses diagnosticadas em serviço de dermatologia na cidade de Manaus, capital do estado do Amazonas. MÉTODOS: Coletaram-se dados registrados sobre sexo, idade, procedência e diagnósticos referentes à primeira consulta dos pacientes atendidos entre janeiro de 2000 e dezembro de 2007. RESULTADOS: Das 56.024 consultas registradas, obtiveram-se 56.720 diagnósticos dermatológicos, sendo mais comuns as doenças sexualmente transmissíveis (25,12%), as dermatoses alérgicas (14,03%), as dermatoses não especificadas (13,01%), a hanseníase (6,34%) e acne, seborreia e afins (5,05%). A frequência foi semelhante para ambos os sexos, a faixa etária de 20-29 anos foi predominante e Manaus foi a procedência mais referida. CONCLUSÕES: O padrão das doenças cutâneas identificadas neste estudo pode servir como linha de base para que gestores do sistema de saúde da região desenvolvam estratégias de prevenção e controle das dermatoses mais comuns, com ênfase nas doenças sexualmente transmissíveis, doenças cutâneas alérgicas, hanseníase e acne

BACKGROUNDS: Fundaments: Skin diseases are associated wih high morbidity, low mortality and low rate of hospitalization. However, they can cause considerable interference in physical and emotional well-being of the individual. Several of them reach large population, requiring specific interventions for their control. OBJECTIVE: To describe the frequency of skin disease diagnosed in the dermatology service in Manaus, capital of Amazonas State. METHODS: We collected data on registered sex, age, origin and diagnostics for the first consultation of patients attended between January 2000 and December 2007. RESULTS: Of the 56.024 recorded visits, we obtained 56.720 cases of dermatological diagnoses, being the most common sexually transmitted diseases (25,12%), allergic skin disesases (14,03%), unspecified dermatoses (13,01%), leprosy (6,34%) and acne, seborrhea and related diseases (5,05%). The frequency was similar for both sexes, aged 20-29 years predominated and Manaus the origin most reported. CONCLUSIONS: The pattern of skin diseases identified in this study may serve as a baseline to managers of health system in the region develop strategies for prevention and control of dermatoses, with emphasis on sexually transmitted diseases, allergic skin diseases, leprosy and acne

O rabdomiossarcoma é o tumor de partes moles mais comum na infância, sendo raro o acometimento exclusivamente cutâneo. Apresenta-se caso de criança com nódulo doloroso na face, cuja análise histopatológica e imunoistoquímica confirmou tratar-se de rabdomiossarcoma, o qual foi conduzido por equipe multidisciplinar. Os tumores de partes moles são responsáveis por 6% de todos os tumores infantis; destes, 53% são rabdomiossarcomas, que podem acometer qualquer sítio. A manifestação como nódulo dérmico é incomum, representando um desafio diagnóstico, já que não possui características clínicas que o diferenciem de outras patologias.

Rhabdomyosarcoma is the most common soft tissue tumor in childhood; however, it rarely affects only the skin. This case report describes a child with a painful nodule on her face. Histopathology and immunohistochemistry confirmed the diagnosis of rhabdomyosarcoma, and a multidisciplinary team then followed up the patient. Soft tissue tumors are responsible for 6% of all childhood tumors, and 53% of these are rhabdomyosarcomas, which may affect any part of the body. Presentation in the form of skin nodules is rare and represents a diagnostic challenge, since there are no clinical characteristics that differentiate this condition from other pathologies.

FUNDAMENTOS: Doenças Sexualmente Transmissíveis em crianças permanecem um problema de saúde pública pouco estudado, sendo ainda necessários esclarecimentos sobre seu manejo e a relação destas com o abuso sexual infantil. OBJETIVOS: Descrever o perfil clínico-epidemiológico das Doenças Sexualmente Transmissíveis em crianças atendidas em centro de referência na cidade de Manaus. MÉTODOS: Realizou-se estudo descritivo exploratório para verificar características clínicas, epidemiológicas e laboratoriais das Doenças Sexualmente Transmissíveis em crianças atendidas durante o período de janeiro/2003 a dezembro/2007. RESULTADOS: Foram incluídas no estudo 182 crianças que apresentavam DST. A maioria era do sexo feminino (65,4%) e de cor parda; a média de idade foi de 8,5 anos; 89% eram procedentes da cidade de Manaus; os pais foram os principais acompanhantes na consulta; verruga genital foi o principal diagnóstico em ambos os sexos; e, 90,1% apresentavam apenas uma DST. CONCLUSÃO: As frequências e características clínicas das DST nas crianças do estudo não diferiram do encontrado na literatura. Embora, com base em sinais e sintomas referentes tão somente às DST nas crianças, não se tenham parâmetros fidedignos de confirmação de abuso, deve-se sempre estar alerta para esta possibilidade, visto que estas doenças podem ser sinalizadoras de ofensas sexuais, por vezes, dissimuladas e repetidas.

BACKGROUND: Sexually transmitted diseases in children remain a public health concern that is relatively ignored. Further data are required on the management of these diseases and their association with child sexual abuse. OBJECTIVES: To describe the clinical and epidemiological profile of sexually transmitted diseases in children receiving care at a referral center in the city of Manaus, Amazonas, Brazil. METHODS: A descriptive, exploratory study was conducted to evaluate the clinical, epidemiological and laboratory characteristics of the sexually transmitted diseases (STD) found in children who received care at this clinic between January 2003 and December 2007. RESULTS: A total of 182 children with STDs were included in the study. The majority were female (65.4%), dark-skinned and with a mean age of 8.5 years. Furthermore, 89% were from the city of Manaus and their parents were usually responsible for having brought them to the clinic. Genital warts constituted the principal diagnosis in children of both sexes and 90.1% of the children had only one STD. CONCLUSIONS: The frequencies and clinical characteristics of the STDs in the children in this study were similar to data reported in the literature. Although the signs and symptoms of the STDs found in these children do not, in themselves, constitute reliable parameters by which to confirm abuse, professionals should always be alert to this possibility, since these diseases may represent a sign of sexual offenses that may be dissimulated and repetitive.

A doença de Rosai-Dorfman, também denominada histiocitose sinusal com linfadenopatia maciça, é histiocitose de células não Langerhans, idiopática e de curso benigno. Descrita em 1969, caracteriza-se por linfadenomegalia não dolorosa, sendo a cadeia cervical a mais envolvida, além de febre, perda de peso e sudorese. O envolvimento extranodal ocorre em 43% dos casos, em que múltiplos sítios podem ser acometidos. Já foram descritos casos exclusivamente extranodais, inclusive formas limitadas à pele. Relata-se um caso de doença de Rosai-Dorfman extranodal cutânea pura, devido à raridade dessa apresentação clínica.

Rosai-Dorfman disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a non-Langerhans cell histiocytosis with a benign course and unknown etiology. It was described in 1969 as a painless cervical lymph node enlargement in association with fever, weight loss and sweating. Extranodal disease has been reported in 43% of cases, with involvement of multiple organs. Purely extranodal Rosai-Dorfman disease has been already reported, including forms restricted to the skin. This paper reports a case of purely cutaneous Rosai-Dorfman disease, which is of interest in view of the rarity of this condition.

FUNDAMENTOS: A teledermatologia, pouco utilizada no Brasil e com iniciativas incipientes no Amazonas, necessita de estudos para demonstrar sua factibilidade e eficácia. OBJETIVOS: Avaliar a eficácia de um método assíncrono de teledermatologia, utilizando recursos tecnológicos simples e de baixo custo. MÉTODOS: Cento e setenta e quatro pacientes foram examinados por quatro dermatologistas; dois efetuaram diagnóstico presencial (A1 e A2) e dois por meio de imagens das lesões e história clínica (B1 e B2). Foi investigada a concordância entre as avaliações presenciais e por imagens. RESULTADOS: A concordância do diagnóstico principal entre os examinadores presenciais (A1 e A2) foi de 83,3% e entre os de imagens (B1 e B2), de 81%. A concordância entre o diagnóstico principal estabelecido pelo método presencial e o obtido por meio de imagens variou de 78,2% a 83,9%. CONCLUSÃO: O diagnóstico de doenças dermatológicas realizado por imagens digitais demonstrou concordância ótima quando comparado àquele realizado com a presença física do paciente.

BACKGROUND: Teledermatology is seldom used in Brazil, although some incipient initiatives have been implemented in the state of Amazonas. Further studies are still required to confirm the feasibility and efficacy of this diagnostic tool. OBJECTIVES: To evaluate the efficacy of an asynchronous method of teledermatology using simple, inexpensive technological resources. METHODS: One hundred and seventy-four patients were examined by four dermatologists, two clinic-based dermatologists, who diagnosed the patients following live examination (A1 and A2), and two consultant specialists in image dermatology, who reached diagnoses by examining images of the lesions and the patients' clinical histories (B1 and B2). The agreement between live examination and examination of images was evaluated. RESULTS: Agreement between the two live examiners (A1 and A2) with respect to the principal diagnosis was 83.3% compared to 81.0% between the physicians who diagnosed by examining images (B1 and B2). Agreement between the principal diagnosis established by live examination and that obtained from examining images ranged from 78.2% to 83.9%, CONCLUSION: Agreement between diagnoses of dermatological diseases reached following examination of digital images and those reached following live examination of the patient was excellent.