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.

Com base nos registros de 730pacientes portadores de L eishm aniose Tegum entar A m ericana (L TA

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.

A 12‐year‐old Brazilian girl was seen in our department with a single asymptomatic nodule on the dorsum of the tongue (Fig. 1). The nodule had been present for approxi‐mately 4 years. It measured 1–2 cm in diameter, was firm, flesh‐colored, nontender, nonpulsatile, and slightly mobile. The patient acknowledged that she had been treated for tapeworms 3 years previously, but she could not specify the treatment. Physical examination was otherwise unre‐markable. A clinical diagnosis of lipoma, cyst, or granular cell tumor was suspected. Biopsy of the nodule was per‐formed. Histopathologic examination revealed a cysticercus of Taenia solium in a “bladder cyst” (Fig. 2) surrounded by a discrete inflammatory reaction. Laboratory tests included stool examination that was negative for ova and parasites and a normal complete blood cell count. Chest roentgeno‐gram, EEG, ECG, and brain, liver, spleen, and bone scans were normal. The patient was seen 2 years later and had no evidence of cysticercosis.

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.

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.