RESUMO Relatamos o caso de um paciente com evolução e diagnóstico tardios de cisto de íris no olho direito, provavelmente secundário a trauma, com complicações importantes e baixa irreversível da visão, tendo sido admitido no Hospital Universitário Antônio Pedro já com leucoma total da córnea e câmara anterior indevassável. A biomicroscopia ultrassônica se mostrou imprescindível para o estudo anatômico, sendo o paciente finalmente submetido à enucleação, para melhora estética e elucidação diagnóstica. Concluímos que a atalamia e a desestruturação do segmento anterior, consequentes ao crescimento cístico, levaram a um gradativo aumento da pressão intraocular e lesão do nervo óptico, com consequente perda da visão.

ABSTRACT The authors report the case of a male adult presenting significant ocular complications and irreversible visual impairment, resulting from the long-term progression and late diagnosis of an iris cyst in the right eye, probably secondary to trauma. The patient was admitted to Hospital Universitário Antonio Pedro with a total corneal opacity that blocked direct visualization of the anterior chamber. Ultrasound biomicroscopy was crucial for the anatomic study, and the patient was submitted to enucleation for aesthetic improvement and clarifying diagnosis. We concluded athalamia and deformation of the anterior segment, due to expansion of the cyst, led to gradual elevation of the intraocular pressure and damage of the optic nerve, resulting in visual loss.

Abstract: Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Abstract: Sweet's syndrome is an uncommon benign skin disorder, whose pathogenesis remains unknown. Its classic form is more common in women and presents itself as papular-nodular, painful and erythematous or violaceous lesions. It mainly affects the face, neck, and upper limbs. Fever and neutrophilic leukocytosis are also common features. Although it is considered a systemic disease marker in more than half of patients, the association of this condition with Crohn's disease is rare, with few cases reported in the literature, of which, none in Brazil. We report the case of a patient with Crohn's disease who developed the classical features of Sweet's syndrome.

Abstract Pruritic folliculitis of pregnancy is a rare disease of unknown etiology. It occcurs primarily during pregnancy, usually with spontaneous resolution postpartum. It is characterized by a benign dermatosis, with papular and pustular follicular lesions that first appear on the torso and occasionally spread throughout the body. We report the case of a patient in the 27th week of pregnancy, with a two-month evolution of pruritic and papular erythematous lesions on her lower back. Differential diagnosis includes other pregnancy-specific dermatoses: gestational pemphigoid, pruritic urticarial papules and plaques of pregnancy (PUPPP), prurigo of pregnancy, and (PUPPP) and prurigo of pregancy. Histopathological tests showed changes consistent with pruritic folliculitis of pregnancy. This case is relevant due to its rare nature and its clinical and histopathological characteristics.

RESUMO Escleredema de Buschke (EB) é doença rara do tecido conjuntivo caracterizada por endurecimento difuso e não depressível da pele, principalmente nas regiões cervical, deltoideanas e dorso. Enquadrado no grupo das mucinoses cutâneas, tem etiologia desconhecida e associação com: infecções bacterianas ou virais, alterações hematológicas e diabetes mellitus. O exame histopatológico evidencia derme espessada com fibras colágenas calibrosas separadas por fendas que correspondem a depósito de mucopolissacárides, observados por colorações especiais. Diversos tratamentos são relatados na literatura sem resultados bem definidos. Descrevemos caso de EB em paciente com diabetes mellitus tipo 2.

SUMMARY Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

Paracoccidioidomycosis (PCM) is the most common endemic mycosis in Latin America. The etiological agents, which comprise two species, Paracoccidioides brasiliensis and P. lutzii, are thermodimorphic fungi that usually affect previously healthy adults. They primarily involve the lungs and then disseminate to other organs. Such mycosis is rare in organ transplant recipients; there have been only three cases reported in literature, until now. We report a case of PCM in a renal transplant recipient with an unusual dermatological presentation.

We describe a case of melanoma with the presence of comedo-like openings at dermoscopy. These structures, typical of seborrheic keratosis, represent an uncommon finding in melanoma. We emphasize the importance of searching for specific dermoscopic criteria for melanocytic lesions during the examination of a pigmented lesion, despite possible observations of characteristic structures of non-melanocytic lesions, in order to increase the accuracy in the diagnosis of melanoma.

A terapêutica com drogas antitireoidianas pode ser acompanhada de efeitos colaterais. Apresentamos um caso de paciente com diagnóstico de Doença de Graves que na vigência da terapêutica com metimazol desenvolveu um quadro extenso de vasculite de membros inferiores. A partir da suspensão do metimazol e da introdução de prednisona em doses imunossupressoras as lesões cutâneas entraram em involução.

Treatment with antithyroid drugs may be accompanied by side effects. We present a patient diagnosed with Grave's Disease who developed extensive vasculitis in the lower limbs during methimazole use. After suspension of the methimazole and the introduction of prednisone in immunesupressor doses the cutaneous lesions started to involute.

A doença de Fox-Fordyce é uma dermatose inflamatória rara que afeta predominantemente mulheres jovens e caracteriza-se por múltiplas pápulas foliculares cor da pele ou acastanhadas, muito pruriginosas, localizadas nas áreas ricas em glândulas apócrinas. A histopatologia demonstra espongiose focal da porção superior do infundíbulo, com fibrose perifolicular e infiltrado linfohistiocítico. O diagnóstico é baseado na clínica e no exame histopatológico. Muitas opções de tratamento já foram descritas, porém nenhuma delas é excelente. Optamos pelo adapaleno 0,1% tópico e foi observada melhora satisfatória dos sinais e sintomas da doença.

The Fox-Fordyce disease is a rare inflammatory dermatosis that affects mainly young women and is characterized by multiple follicular papules, skin color or brownish, very itchy, localized in areas rich in apocrine glands. Histopathology shows focal spongiosis of the upper infundibulum with fibrosis and perifollicular lymphohistiocytic infiltrate. The diagnosis is based on clinical and histopathological examination. Many treatment options have been described; however none of them is excellent. We chose the topic adapalene 0.1% and a satisfactory improvement of the signs and symptoms of the disease was observed.

FUNDAMENTOS: A síndrome de Sweet corresponde a um conjunto de alterações cutâneas, sistêmicas e histopatológicas como resposta a diversos estímulos, semelhantes ao eritema nodoso, ao eritema multiforme e à vasculite leucocitoclásica. São descritas condições associadas como infecção, gravidez, uso de drogas e malignidades. OBJETIVOS: Avaliar as alterações clínicas e histopatológicas da síndrome, relacionando-a a outras condições. MÉTODO: Estudo retrospectivo de 73 casos com avaliação microscópica de lesões cutâneas, dados clínicos e laboratoriais. RESULTADOS: Houve predomínio de mulheres (83,0%), brancas (49,2%), entre a quarta e a sexta décadas de vida (73,8%). Placas eritematosas (76,9%), pápulas (43,0%), pseudovesiculação (PV) (38,4%) e lesões em alvo (18,5%) foram as principais alterações. Entre as condições associadas, infecções de vias aéreas (15,4%) e uso de drogas (10,8%) foram as mais frequentes. Outras associações, representadas por um caso cada, foram: linfoma de Hodgkin, gravidez, colite ulcerativa, policitemia vera e lúpus eritematoso em paciente com Aids. Neutrófilos com leucocitoclasia (98,6%), degeneração do colágeno (87,7%), edema (74,0%) e PV (38,4%) foram os principais achados microscópicos. Eosinófilos estiveram presentes (41,1%) de raros a abundantes e, em geral, não relacionados ao uso de drogas. O infiltrado inflamatório atingiu a derme profunda em 47,9% das vezes e encontrou-se paniculite em 80,0% dos casos nos quais a hipoderme estava representada (10 casos). CONCLUSÕES: Os achados, de modo geral, coincidem com os da literatura, destacando-se: frequente participação de eosinófilos sem correlação com a ingestão de medicamentos, paniculite e rara associação com linfoma de Hodgkin, sendo este o quinto relato de tal ocorrência, segundo o conhecimento dos autores.

BACKGROUND: Sweet's syndrome refers to a set of cutaneous, systemic and histopathological alterations that occur in response to different stimuli, in a similar way to that occurring in erythema nodosum, erythema multiforme and leukocytoclastic vasculitis. The syndrome has been described in association with conditions such as infections, pregnancy, the use of certain medications and malignancy. OBJECTIVES: To evaluate the clinical and histopathological alterations occurring in this syndrome and to assess the association between these alterations and other conditions. METHODS: A retrospective study of 73 cases was conducted, evaluating data on the microscopic examination of skin lesions, as well as clinical and laboratory data. RESULTS: The majority of the patients were female (83.0%), white (49.2%) and between 30 and 60 years of age (73.8%). The principal alterations found were: erythymatous plaques (76.9%), papules (43.0%), pseudo-vesiculation (PV) (38.4%) and target lesions (18.5%). With respect to the associated conditions, upper respiratory tract infections (15.4%) and the use of medication (10.8%) were the most common. Other associations, albeit represented by only one case each, were: Hodgkin's lymphoma, pregnancy, ulcerative colitis, polycythemia vera and lupus erythematosus in a patient with acquired immune deficiency syndrome (AIDS). The principal microscopic findings were: neutrophils with leukocytoclasia (98.6%), collagen degeneration (87.7%), edema (74.0%) and PV (38.4%). The presence of eosinophils (41.1%) ranged from rare to abundant and was usually unrelated to the use of medication. Inflammatory infiltrate reached the deep epidermal layer in 47.9% of cases and panniculitis was found in 80.0% of cases in which the hypodermis was affected (10 cases). CONCLUSIONS: In general, these findings are in agreement with results published in the literature, emphasizing the frequent finding of eosinophils unrelated to drug use, panniculitis and the rare association with Hodgkin's lymphoma. This is the fifth report of an association between Sweet's syndrome and Hodgkin's disease.

A síndrome de Sweet pode estar associada a malignidades hematológicas, principalmente, à leucemia mieloide aguda, porém existem poucos relatos demonstrando a associação com a policitemia vera. Relata-se o caso de doente do sexo masculino, de 65 anos, portador de policitemia vera,que evoluiu com aparecimento de síndrome de Sweet na sua forma paraneoplásica.

Sweet’s syndrome may be associated with hematological malignancies, particularly with acute myelogenous leukemia, but there are few reports of its association with polycythemiavera. We describethe case of a 65-year-old male patient, diagnosed with polycythemiavera, which developedinto paraneoplastic Sweet's syndrome.

O estudo da medula óssea em 20 casos de necrópsia de pacientes portadores da Síndrome da Imunodeficiência Adquirida sugere que as alterações medulares são freqüentes e de valor diagnóstico no reconhecimento da Síndrome. É admitida a existência de um padrão lesional diagnóstico onde predominam os seguintes elementos: hipercelularidade, ausência ou redução da granulopoiese, plasmocitose, histiocitose e displasia megacariocítica.

The bone marrow of 20 autopsy cases of patients with Acquired Immune Deficiency Syndrome was examined. The study suggests that the bone marrow changes are frequent and of diagnostic value to recognize the syndrome. It is assumed that there is a diagnostic lesional pattern in which the following elements are predominant: hypercellularity, absent or reduced granulopoiesis, plasmocytosis, histiocytosis and megakariocytic dysplasia.