Paracoccidioidomycosis: Epidemiological and Clinical Aspects in 546 Cases Studied in the State of Espírito Santo, Brazil.

Paracoccidioidomycosis (PCM) is an endemic disease in the southeast region of Brazil, which includes the state of Espírito Santo (ES). This historic case series analyses 546 patients treated in this state from 1978 to 2012. Patients aged from 7 to 83 years, 509 males and 37 females, yielding a ratio of 13.7:1. Most of the patients (81.4%) originated from rural areas in ES, 71.0% being farmers. A higher concentration of cases was observed in municipalities located along the western range of the state. Sixty patients (11.0%) had an acute/subacute form of the disease, 485 (88.9%) had a chronic form, and one had a subclinical form. The most affected organs included the lungs, oropharyngeal mucosa, lymph nodes, skin, and larynx. The diagnosis was confirmed by histopathology in 252 (46.2%) cases, direct examination in 168 (30.7%), both exams in 111 (20.3%) and serology in 15 (2.8%). Tuberculosis, acquired immune deficiency syndrome, leishmaniasis, and intestinal parasites were the most frequently associated infectious diseases. From 328 patients followed up, total regression of the lesions was observed in 17.4%, partial regression in 77.4%, and no regression in 17 (5.2%) cases. Regarding the number of cases in this series, ES emerges as an important endemic area for PCM in Brazil.

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito 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.

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.

Pitiríase rósea: [revisão] / Pityriasis rosea: [review]

Pitiríase rósea é doença inflamatória aguda da pele, que regride espontaneamente, normalmente sem deixar seqüelas, em período que varia de quatro a oito semanas. Clinicamente é caracterizada pelo aparecimento de típicas lesões eritêmato-pápulo-escamosas. Atinge todas as idades, embora seja mais comumente observada entre 10 e 35 anos. Apesar de exaustivas pesquisas, sua etiologia ainda permanece desconhecida. São discutidos alguns aspectos epidemiológicos, anatomoclínicos, diagnósticos diferenciais, doenças associadas, com ênfase no tratamento e etiologia da doença.

Pityriasis rosea is a skin disease characterized by acute inflammation that fades away spontaneously without scar tissue formation for an average period of 4 to 8 weeks. Clinically, it is characterized by the occurrence of typical erythematous papulosquamous lesions distributed mainly over the trunk and extremities. It affects people of all ages, but is more often observed in the age range of 10 to 35 years. Even though it is extensively studied, its etiology remains unknown. In this study, the authors analyze some epidemiologic aspects, differential diagnosis, related diseases, and give special attention to treatment and possible etiology.

Focal dermal hypoplasia with exuberant fat herniations and skeletal deformities.

Focal dermal hypoplasia or Goltz syndrome is a rare congenital and mesoectodermal dysplasia with multisystemic involvement. Although the genetic alterations responsible for focal dermal hypoplasia are not fully known, there is predominance in affected females, suggesting dominant X-linked inheritance. Besides the skin, other structures frequently involved are the skeletal system, eyes, teeth, hair, and nails. Skeletal abnormalities are predominantly observed in the hands and feet. We report a 9-year-old girl who had typical linear skin atrophy on the trunk, exuberant "fat herniations," several skeletal abnormalities, and exuberant "lobster claw" deformity. In addition, she had the typical longitudinal striations in femur metaphyses. With regard to family history, her mother had one male stillbirth with several deformities. This typical focal dermal hypoplasia patient is considered valuable in light of the affected male stillbirth and parents with nonaffected phenotypes that together provides evidence for mother-to-daughter spontaneous transmission.

Manifestaçöes cutâneas em transplantados renais: um estudo prospectivo / Cutaneous manifestations in renal transplantation patients: a prospective study

Foi realizado um estudo das manifestaçöes cutàneas em pacientes transplantados renais que teve como objetivo avaliar a incidência e o comportamento dessas dermatoses e sua resposta ao tratamento específico. Os 14 pacientes estudados foram submetidos a um protocolo que incluía, quando necessário, exame histopatológico e micológico. Observaram-se manifestaçöes alérgicas (7,1%), pré-tumorais (7,1%), vasculares (14,3%), tumorais (14,3%), bacterianas (21,4%), virais (28,6%), fúngicas (87,7%) entre outras (incluindo as iatrogênicas näo diferem daquelas descritas na literatura mundial. As manifestaçöes clínicas e sua evoluçäo com o tratamento específico ainda näo estäo em fase de estudos, e nossos resultados, embora parciais, indicam que as lesöes cutàneas säo mais atípicas e rebeldes à terapêutica quando os pacientes apresentam déficit funcional renal, independente da dosagem das drogas imunodepressoras

Distribuiçäo geográfica da Leishmaniose tegumentar Americana no Estado do Espírito Santo-Brasil / Geographic distribution of American tegumentary leishmaniasis in Espírito Santo State-Brazil

Com base nos registros de 730 pacientes portadores de Leishmaniose Tegumentar Americana (LTA) referentes ao período de janeiro de 1972 a dezembro de 1982, os autores realizaram um estudo da distribuiçäo geográfica dessa parasitose no Estado do Espírito Santo. Os pacientes procediam de 36 dos 53 municípios que compöem o Estado, destacando-se Viana e Cariacica como os de maior prevalência, somando juntos 442 casos (60,54%), com 259 destes residindo numas poucas localidades, contínuas entre si, formando uma área endêmica, com transmissäo ocorrendo provavelmente no peri e intra-domicílio. Esta área de alta endemicidade, pertencente principalmente ao vale do rio Formate, estende-se também ao município de Domingos Martins através da localidade de Biriricas. Nos demais municípios a LTA caracterizou-se como uma doença profissional de ocorrência antiga no Estado