Hamartoma folicular basaloide / Basaloid follicular hamartoma

O hamartoma folicular basaloide (HFB) é um tumor anexial raro e benigno, que se assemelha ao carcinoma basocelular (CBC), e pode apresentar manifestações clínicas diversas. Uma mutação no gene PTCH, envolvido na síndrome de Gorlin-Goltz, poderia estar associada à patogênese dessa neoplasia. Descreve-se caso de menina, sete anos, apresentando múltiplas pápulas na face.

Basaloid follicular hamartoma (BFH) is a rare and benign adnexal tumor that resembles basal cell carcinoma (BCC) and may present with different clinical manifestations. A mutation in the PTCH gene, involved in Gorlin-Goltz syndrome, could be associated with the pathogenesis of this neoplasm. We describe the case of a 7-year-old girl with multiple papules on her face.

Cutaneous metastasis on the nasal tip: first clinical sign of pulmonary carcinoid tumor

Abstract Skin metastases are rare and may occur in the context of a known metastatic disease or be the first clinical sign of an underlying primary tumor. In the case of carcinoid neoplasms, determining whether the cutaneous tumor is primary or secondary and identifying the tumor origin in metastatic cases is not always an easy task. This is the report of a case of cutaneous metastasis presenting as the first clinical manifestation of a previously unknown pulmonary carcinoid tumor, including the discussion of histopathological and immunohistochemical findings that allowed an adequate diagnosis of the tumor etiology and reinforces the importance for dermatologists and dermatopathologists to be familiar with these findings.

Relapse in leprosy and drug resistance assessment in a tertiary hospital of the state of Espírito Santo, Brazil

Abstract INTRODUCTION: Leprosy recurrence is the reappearance of the disease after treatment with current schemes and discharged for cure and may have variable incubation periods. METHODS: This is a descriptive observational study of leprosy recurrence in Espírito Santo diagnosed between January 2018 and January 2020. RESULTS: One hundred and ninety-two cases were available, of which 30 were diagnosed with leprosy recurrence. CONCLUSIONS: In 25 cases, the incubation period was 5-15 years after the first treatment, favoring bacillary persistence. In the remaining 5 cases, the disease had recurred after 15 years, pointing to reinfection as none of them exhibited drug resistance.

Diagnostic delay in a patient with reactional lepromatous leprosy treated as testicular tuberculosis

Abstract Leprosy is a chronic neglected and stigmatizing disease. Due to involvement of the peripheral nerves, it can result in physical disabilities, deformities, and emotional damage if not diagnosed and treated promptly. This is the case of a young patient with testicular pain and swelling and no improvement after a specific therapeutic scheme for tuberculosis. Clinical and laboratory reevaluation revealed hypoesthetic skin patches associated with post-burn crusted ulcers on the left arm, thickening of ulnar nerves, atrophy of interosseous muscles of the hands, positive skin smear microscopy, and testicular histopathology with numerous bacilli forming globi. These findings indicated lepromatous leprosy with type II reaction.

Exuberant case of cutaneous metastasis of breast cancer

Abstract: Cutaneous metastasis is the main cause of morbidity and mortality of cancer patients, denoting a guarded prognosis. The clinical spectrum of the disease is broad and can mimic benign conditions. The diagnosis depends on thorough clinical examination and complementary exams, with emphasis on the histopathological study and immunohistochemistry. Because it indicates a systemic relapse, it is necessary to intervene with intravenous chemotherapy, to which local therapies can be associated. We report the case of a 65-year-old woman with cutaneous metastasis from breast cancer treated 14 years back, manifested by extensive firm and infiltrated red-purple plaques on the sides of the trunk, with a course of six months. After evaluation, she was referred to the regional cancer center. She passed away one month and a half later.

Diffuse cutaneous melanosis: rare complication of metastatic melanoma

Abstract Diffuse cutaneous melanosis is a rare complication of metastatic melanoma related to a worse prognosis. There are few cases reported in the literature. Its pathogenesis has not been completely elucidated, although studies have suggested certain mechanisms for its occurrence. It is clinically manifested as a blue-gray discoloration of the skin and mucous membranes in a cephalo caudal progression and usually associated with melanuria. Skin and mucosa histopathology reveals only the presence of melanophages in the dermis, mainly perivascular, and free interstitial melanin. We report the case of a 68-year-old male with metastatic melanoma, diffuse hyperpigmentation of the skin and melanuria.

Bacillary angiomatosis with bone invasion

Abstract Bacillary angiomatosis is an infection determined by Bartonella henselae and B. quintana, rare and prevalent in patients with acquired immunodeficiency syndrome. We describe a case of a patient with AIDS and TCD4+ cells equal to 9/mm3, showing reddish-violet papular and nodular lesions, disseminated over the skin, most on the back of the right hand and third finger, with osteolysis of the distal phalanx observed by radiography. The findings of vascular proliferation with presence of bacilli, on the histopathological examination of the skin and bone lesions, led to the diagnosis of bacillary angiomatosis. Corroborating the literature, in the present case the infection affected a young man (29 years old) with advanced immunosuppression and clinical and histological lesions compatible with the diagnosis.

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito Santo) / Síndrome de Sweet: achados clínico-patológicos nos pacientes atendidos de 1997 a 2009 no Hospital Universitário Cassiano Antônio Moraes - Vitória (Espírito Santo)

Sweet's syndrome or acute febrile neutrophilic dermatosis is rare in Brazil. It is clinically characterized by painful erythematous nodules, papules or plaques that occur mainly on the neck and upper limbs. Its cause may be unknown (idiopathic form) or it may be associated with malignancies, usually hematologic, or drugs. The authors describe 16 cases of the syndrome. The median age was 36 years, and all patients were white and presented solid lesions, predominantly on the upper limbs and trunk. Histopathological examination of the dermis of all patients revealed predominance of moderate to intense, superficial and deep, diffuse inflammatory infiltrate, mainly consisting of polymorphonuclear neutrophils, with leukocytoclasia. It also revealed changes in the epidermis and hypodermis (neutrophilic hypodermitis), but with no signs of vasculitis in most patients, which was not considered an important finding for diagnosis. Presence of exocytosis of neutrophils was common, favoring the diagnosis of Sweet's syndrome when accompanied by diffuse interstitial neutrophilic dermatitis.

A Síndrome de Sweet ou dermatose neutrofílica febril aguda é rara no Brasil, caracterizada clinicamente por pápulas, placas ou nódulos eritematosos, dolorosos, principalmente na região cervical e membros superiores. Pode surgir sem fator desencadeante (forma idiopática) ou associar-se a neoplasias, comumente hematológicas, ou a drogas. Os autores descrevem 16 casos da Síndrome, idade mediana de 36 anos, todos da raça branca, com lesões sólidas predominantes nos membros superiores e no tronco. O histopatológico da derme observou, em todos os pacientes, predomínio de infiltrado inflamatório difuso, superficial e profundo, de moderado a intenso, composto principalmente por polimorfonucleares neutrófilos, com leucocitoclasia, além de alterações na epiderme e na hipoderme (hipodermite neutrofílica), mas sem sinais de vasculite na maioria dos pacientes, não sendo considerada achado importante para o diagnóstico. A presença de exocitose de neutrófilos foi frequente, favorecendo o diagnóstico de Síndrome de Sweet quando acompanhada por dermatite intersticial neutrofílica difusa.

Kaposi's Sarcoma: clinical and pathological aspects in patients seen at the Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil / Sarcoma de Kaposi: achados clínico-patológicos nos pacientes atendidos no Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil

BACKGROUND: Kaposi's sarcoma is a neoplasm of endothelial origin that is divided into four distinct types according to the clinical characteristics and the affected population: Classic (in elder men of Jewish or Mediterranean origin); Epidemic (in patients affected by AIDS); Endemic (in black African men) and Iatrogenic (in patients under immunosuppressive regimens). Human herpesvirus 8 infection is essential but not sufficient for the sarcoma development. OBJECTIVE: To describe the epidemiological, clinical and histopathological aspects of patients with KS seen at the Dermatology Clinic -Cassiano Antônio Moraes University Hospital - Federal University of Espirito Santo, Vitória - ES. METHODS: A descriptive and retrospective study based on clinical charts of patients with KS seen at the Dermatology Clinic from 1986 to 2009. RESULTS: The majority of the 15 cases were male patients (93,3%) and white (60%). Epidemic Kaposi's sarcoma occurred in 80%, and the Classic form in 20%, with no cases in the Endemic or Iatrogenic groups. All the histopatho logical exams of the cutaneous lesions were reviewed and a proliferation of fusiform cells, extravasated erythrocytes and vascular rifts among the largest vessels, assuming the "vessels in vessels" typical aspect, were seen. CONCLUSION: The number of cases of Kaposi's Sarcoma was linear throughout the years of the study, especially of the epidemic form, although the incidence and prevalence of AIDS increased in the state of Espírito Santo. Therefore, if we consider the relation between KS and AIDS, a decreasing line of Kaposi's sarcoma could be seen, especially after the introduction of HAART.

FUNDAMENTOS: O Sarcoma de Kaposi é neoplasia de origem endotelial, dividida em quatro formas clínicas: clássica (homens idosos de origem judaica e mediterrânea), epidêmica (associada ao HIV), endêmica (negros africanos) e iatrogênica (relacionada à imunossupressão). A infecção pelo herpes vírus humano tipo 8 (HHV-8) é necessária, mas insuficiente para que todas as formas possam ocorrer. OBJETIVOS: Avaliar os aspectos epidemiológicos, clínicos e características histopatológicas das lesões dos pacientes com Sarcoma de Kaposi consultados no Serviço de Dermatologia do Hospital Universitário Cassiano Antônio Moraes - Universidade Federal do Espírito Santo, Vitória - ES. MÉTODOS: Estudo retrospectivo, descritivo, realizado pela análise dos prontuários dos pacientes diagnosticados com Sarcoma de Kaposi, durante janeiro de 1986 a dezembro de 2009, no Serviço de Dermatologia. RESULTADOS: Dos 15 pacientes estudados, houve maioria do sexo masculino (93,3%) e predomínio da raça branca (60%). A forma epidêmica foi a mais freqüente (80%), seguida pela clássica (20%). Não foram observadas as formas: endêmica e iatrogênica. A revisão das lâminas das biópsias cutâneas foi feita nos 15 casos, e demonstrou derme com proliferação de células fusiformes, extravasamento de hemácias e fendas vasculares em torno de vasos maiores, com aspecto clássico de "vasos em torno de vasos". CONCLUSÕES: O número de casos de Sarcoma de Kaposi foi linear ao longo do estudo, especialmente da forma epidêmica. Por outro lado, a incidência e a prevalência da AIDS no Espírito Santo foram crescentes. Portanto, considerando-se a relação entre o sarcoma de Kaposi e a AIDS houve decréscimo do primeiro, mais acentuado após a era HAART.

Edema agudo hemorrágico da infância: relato de três casos / Acute hemorrhagic edema of infancy: report of three cases

O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.

Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.

Melanoma maligno:estudo casuístico retrospectivo de 1982 a 1992, no Hospital Universitário da UFES / Malignant melanoma: retrospective study in the period 1982-1992, in the University Hospital of UFES

Entre 35. 695 biópsias realizadas no decênio 1982-92, no HUCAM, foram encontrados 55 casos de melanoma maligno (MM), correspondendo a 0,15 por cento dos diagnósticos histopatológicos. A distribuiçäo dos casos de MM quanto a cor e idade näo diferiram do existente na literatura. Observou-se alta freqüência de melanoma metastático e de casos em fase avançada com baixo índice de concordância (43,33 por cento) e o alto índice de inceerteza (33,33 por cento) em relaçäo ao diagnóstico clínico. Estes dados sugerem falha no diagnóstico precoce dos MM pelos médicos e/ou uma desinformaçäo da populaçäo quanto ao risco das lesöes pigmentadas. Através dos dados obtidos verifica-se que existe necessidade de divulgaçäo dos critérios clínicos de diagnósticos de melanoma maligno cutâneo (MMc) dentro da classe médica e de uma campanha de educaçäo para a populaçäo em geral, talvez associado a um programa de exame sistematizado dos indivíduos com alto risco para MM

Líquen esclero-atrófico bolhoso em aréolas mamárias: relato de um caso / Bullous lichen sclerosus et atrophicus on mammary areolae

Relato de um caso de paciente do sexo feminino branca, 23 anos, con líquen esclero-atrófico (LSA) bolhoso acometendo bilateralmente as aréolas mamárias. As lesöes inciaram após a menarca, como manchas hipocrômicas em ambas aréolas mamárias, sendo tratada, nos primeiros três anos, como vitiligo. Foi feita revisäo da literatura e uma breve discussäo sobre o diagnóstico diferencial da doença

Granuloma anular generalizado actínico ou granuloma actínico generalizado?: relato de um caso / Actinic generalized granuloma anmelare or generalized actinic granuloma?: report of a case

Relato de um caso com lesöes populoanulares disseminadas em áreas expostas à luz solar, apresentando, à histopatologia, reaçäo granulomatosa com células gigantes tipo corpo estranho e intensa elastose solar que respondeu bem ao tratamento com difosfato de cloroquina. Discutem-se as possibilidades diagnósticas entre granuloma actínico ou granuloma anular ou se ambos representam uma mesma doença