Cerebriform Appearance Research Articles

CT findings in a dog with chronic giant hypertrophic gastritis: Ménétrier-like disease.

A 9-year-old male's entire Boston Terrier was presented with persistent hyporexia and weight loss for 6 weeks prior to referral admission. A CT scan showed marked hypertrophy of the rugal folds protruding into the gastric lumen, which had a cerebriform appearance. Changes were consistent with the ultrasonographic findings, which showed marked hyperechogenicity of the gastric mucosa, mucosal cysts, and otherwise normal wall layering. Histopathology of the fundic gastric mucosa showed foveolar hyperplasia and cystic dilation which were findings consistent with Ménétrier-like disease. Based on the reviewed literature, this is the first case report describing CT findings of Ménétrier-like disease in a dog.

Giant Cerebriform Nevus Sebaceous of Jadassohn in an Infant

Nevi are hamartomatous growth of skin or mucosa that usually present at the time of birth or soon after. Nevus sebaceous (NS) is an epidermal nevus comprised predominantly of sebaceous glands. It presents at the time of birth, as yellow-tan-colored plaque, which usually remains constant till puberty and thereafter it increases in size due to the hormonal influence on sebaceous glands. Here, we are reporting a case of giant cerebriform NS of Jadassohn in a 2-month-old male child. Such giant and cerebriform appearance of NS is rarely seen.

Prevalence of respiratory epithelial adenomatoid hamartomas (REAH) associated with nasal polyposis: an epidemiological study – how to diagnose

ObjectivesTo highlight the prevalence of respiratory epithelial adenomatoid hamartomas in the olfactory cleft of patients with nasal polyposis. To demonstrate characteristics indicative of hamartoma on the CT scans of paranasal sinuses during surgery and in histopathological exams. MethodsCross-sectional study carried out in Hospital das Clínicas da UFMG and Núcleo de Otorrino BH. We performed 114 nasal endoscopic surgeries for polyposis, between February 2015 and November 2019. We assessed the olfactory cleft width in all preoperative CT scans. Upon seeing an indication of hamartoma on the CT scan, we took a tissue sample from the olfactory cleft during the surgery and sent for histopathological exam. We referred the samples to a pathologist experienced in the anatomopathological diagnosis of respiratory epithelial adenomatoid hamartomas. ResultsOf the 114 patients with polyposis, 54 (47.4%) had olfactory cleft enlargement and, 100% of them had tissue with a dense and hardened polypoid aspect, with a slight cerebriform appearance in this region during the surgery. Histology confirmed a respiratory epithelial adenomatoid hamartoma. ConclusionThis observation suggests that the presence of hamartomas in polyposis is common, but underdiagnosed. Level of evidenceStep 3 (Level 3).

043 Role of Prostaglandin E-major Urinary Metabolite Levels in Identifying The Phenotype of Pachydermoperiostosis

Pachydermoperiostosis (PDP) is an autosomal recessive hereditary disease, hat has three diagnostic features: digital clubbing, periostosis, and pachydermia including cutis verticis gyrata (CVG). CVG is a condition in which folds of the hypertrophic scalp skin create a cerebriform appearance. The phenotypic spectrum of PDP has been categorized to the complete and incomplete form depending on presence or absence of CVG respectively. Two causative genes: HPGD and SLCO2A1 have been identified. Prostaglandin E2 (PGE2) is metabolized to stable prostaglandin E-major urinary metabolite (PGE-MUM), and finally excreted in the urine as PGE-MUM.

Dermoscopic findings of genital keratotic lesions: Bowenoid papulosis, seborrheic keratosis, and condyloma acuminatum

Dermatologists often encounter keratotic or warty lesions in the genital area. Establishing a clear diagnosis may seem challenging, particularly when the differential diagnosis includes bowenoid papulosis, seborrheic keratosis, and condyloma acuminatum. This study aimed to compare the dermoscopic features of bowenoid papulosis (BP), seborrheic keratosis, and condyloma acuminatum in the genital area. All lesions histopathologically confirmed underwent clinical assessment and dermoscopic observation. Dermoscopically, glomerular vessels were predominant in bowenoid papulosis, whereas seborrheic keratosis was the least vascular-patterned disease. Most cases of bowenoid papulosis presented mucosal pigmentation and classified as "flat". Seborrheic keratosis had a pigmented, cerebriform appearance. Condyloma acuminatum was characterised by a finger-like appearance, highly vascular-patterned features surrounded by whitish halos. Dermoscopic findings can be useful for differentiating the entity of genital keratotic lesions ahead of an invasive method. When dermoscopic features favor BP, different from genital warts, it should be removed completely but conservatively.

Role of Prostaglandin E-Major Urinary Metabolite Levels in Identifying the Phenotype of Pachydermoperiostosis

Pachydermoperiostosis (PDP) is an autosomal recessive hereditary disease that has three diagnostic features: digital clubbing, periostosis, and pachydermia, including cutis verticis gyrata (CVG) (Castori et al., 2005). CVG is a condition in which folds of the hypertrophic scalp skin create a cerebriform appearance. The phenotypic spectrum of PDP has been categorized into three distinct forms. First, the complete form involves all the three major symptoms, including CVG. Second, the incomplete form has all the three symptoms but solely lacks CVG.

Cerebriform intradermal nevus presenting as cutis verticis gyrata: A rarity

Cutis verticis gyrata (CVG) is a rare skin disorder characterized by cerebriform appearance of the scalp with convoluted folds and deep furrows. Cerebriform intradermal nevus (CIDN) is one of the rare causes of CVG. We report a case of CIDN presenting as CVG on the left parietal area of scalp since birth in a 15-year-old girl. She had no neurological or ophthalmologic manifestations or any other cutaneous lesions.

648 Dermoscopic findings and HPV genotypes of genital keratotic lesions: Bowenoid papulosis, seborrheic keratosis, and condyloma acuminatum

Dermatologists often encounter keratotic lesions in the genital area. Although making a clear diagnosis can be difficult, it is important for the treatment and prognosis closely related to the patient's quality of life. Dermoscopy has proven to be a useful, non-invasive tool. However, there is still a lack of dermoscopic data comparing bowenoid papulosis (BP), seborrheic keratosis (SK), and condyloma acuminatum (CA). More than 40 human papillomaviruses (HPV) genotypes infect the genital area and manifest as various intraepithelial neoplasms. This study is conducted to find distinctive dermoscopic features and HPV genotype distribution of BP, genital SK, and CA. Dermoscopically, glomerular vessels were predominant in BP that appeared in 7 cases (70.0%). Hairpin vessels were the most common vascular structures that accounted for 12 cases of CA (66.7%). SK was the least vascular-patterned disease as no vessel was observed in eight cases of SK (66.7%). Mucosal pigmentation was observed in 6 cases (60.0%) of BP. Seven cases of BP (70.0%) were classified into 'flat'. SK showed cerebriform appearance in seven cases (58.3%). Most CA cases had knob-like or finger-like appearance and whitish halo. All of BP ad CA presented positive results in HPV DNA detection, while seven cases (58.3%) of SK had positive results. For the high-risk genotype, principally HPV 16, BP showed the highest detection rate with 90.0%. SK and CA showed 58.3% and 44.4%, respectively. For the low-risk genotype, principally HPV 6 followed by HPV 11, CA presented the highest detection rate, with 88.9%. BP and SK showed 40.0% and 8.3% detection rate, respectively. The coexistence of the high-risk and the low-risk HPV was seen in three cases (30.0%) of BP, one case (8.3%) of SK, and six cases (33.3%) of CA. Dermoscopy can be useful for differentiating the entity of genital keratotic lesions ahead of an invasive method and a physician should consider the morphologic plasticity of HPV-related keratosis in the genital area or the genital wart in the expanded concept.

Hypertrophic gastropathy associated with gastric sarcoma in a dog

A 14-y-old spayed female Labrador Retriever was presented with an 8-mo history of chronic vomiting. Abdominal ultrasound and gastrointestinal endoscopy revealed a mass protruding into the gastric lumen, with cytologic features suggestive of sarcoma. A partial gastrectomy was performed; the gastric body and antrum were thickened, with a cerebriform appearance of the mucosal surface. Histologic examination revealed a submucosal neoplastic proliferation of fusiform cells variably arranged in irregular bundles and scattered whorls. Fusiform cells strongly reacted to antibodies against vimentin, S100, and neuron-specific enolase; glial fibrillary acidic protein was moderately and multifocally expressed. Pancytokeratin, KIT, α-smooth muscle actin, and desmin were nonreactive. Histologic and immunohistochemical findings suggested a diagnosis of gastric sarcoma with features referable to a non-GIST (gastrointestinal stromal tumor), non-smooth muscle NIMT (non-angiogenic, non-lymphogenic intestinal mesenchymal tumor). The overlying gastric mucosa was thickened by elongated and dilated gastric glands, predominantly lined by intensely periodic acid-Schiff-stained mucous cells. This altered mucosal architecture was suggestive of Ménétrier-like disease. Although this disease has been hypothesized to predispose to gastric adenocarcinoma in dogs, an association with gastric sarcoma has not been documented previously in the veterinary literature, to our knowledge.

Proteus Syndrome: Report of a Case with Vascular Malformation Successfully Treated with Endovenous Radiofrequency Ablation

We reported a case of a 10 year-old Thai boy diagnosed as Proteus syndrome, presented with progressive enlarging of extremities with asymmetric distorting of the feet and toes. Skin examination showed marked thickening of the soles without characteristic cerebriform appearance, and well-defined purplish plaques with capillary-microcystic lymphatic malformation on the right hip and thigh. MRI/MRA of extremities showed disproportionate overgrowth with combined lymphatic-venous malformation at the right VNUS® Radiofrequency Generator Model RFG2 (VNUS Medical Technologies Inc., San Jose, California, USA and foam sclerotherapy after ablation as an adjunctive treatment for the venous malformation of the right leg with favorable outcomes.

Lobulated Bowen's Disease with a Clear Cell Change

Bowen's disease usually manifests as a slowly enlarging erythematous scaly patch or plaque. An uncommon variant of Bowen's disease showing a verrucous appearance has been reported and a distinct variant with a prominent clear cell change on histopathology, in addition to a verrucous surface change, was also reported. We describe novel form of Bowen's disease having a cerebriform appearance and showing histopathologically a significant clear cell change and propose that the clinical term “lobulated Bowen's disease” would be compatible for the description of this unique clinical variant. From a histopathological point of view, the precise definition and etiopathogenesis of the clear cell change in Bowen's disease should be elucidated.

Cutis verticis gyrata secondary to giant cerebriform intradermal nevus in a pediatric patient

Cutis verticis gyrata (CVG) is a long-lasting and progressive condition in which there is hypertrophy and folding of the scalp skin. Cerebriform intradermal nevus (CIDN) is a rare cause of CVG. Here, we report a case with CIDN of the scalp in a 12-year-old girl. Dermatological examination showed a cerebriform appearance with accentuating folds and deep furrows, thickening in the temporal regions. We want to emphasize that CIDN should not be forgotten in the differential diagnosis of the causes of CVG.

Nem tudo que parece ser, é lesão: aspectos anatômicos, não lesões, artefatos, lesões sem significado clínico e alterações post mortem encontrados na necropsia de suínos domésticos e selvagens (Sus scrofa)

A interpretação das alterações encontradas na necropsia é uma etapa importante para o sucesso do diagnóstico final. Este trabalho tem como objetivo descrever e ilustrar os aspectos anatômicos, não lesões, artefatos, lesões sem significado clínico e alterações post mortem encontradas em suínos domésticos e selvagens. Além disso, também se recomenda técnicas de colheita de tecidos para o diagnóstico de doenças que acometem essa espécie. Os principais aspectos anatômicos e não lesões descritos são fímbrias linguais, quadrilátero esofágico, toro pilórico e demarcação do padrão lobular do fígado (sistema gastrintestinal); tonsilas do palato mole, tecido linfoide associado ao estômago, placas de Peyer do intestino delgado e dobras da margem do baço (sistema hematopoiético); mediastino proeminente do testículo e aréolas da placenta (sistema reprodutor); atelectasia pulmonar e apêndice decidual (feto); e glândulas carpais (sistema tegumentar). Os artefatos de eutanásia abordados são petéquias na superfície do pulmão e rim, falsa anemia por sangria, hemorragia subdural por concussão cerebral, pseudo-infartos do baço e aspecto cerebriforme do intestino delgado. As lesões de pouco significado clínico descritas são cistos renais, linfonodos com pigmento de ferro, papilomas e hemangiomas no escroto, ossos no mesentério e hiperemia da mucosa gástrica. As alterações post mortem comumente encontradas são livor mortis, músculos pálidos, pseudomelanose e líquido serosanguinolento nas cavidades torácica e abdominal em fetos.

Giant Seborrheic Keratosis of the Genitalia Clinically Mimicking a Genital Wart

Seborrheic keratosis is one of the most prevalent benign skin tumors. It is most frequently determined in body, extremities, head and neck. Genital region located seborrheic keratosis is rarely encountered and it is frequently misdiagnosed as genital wart. HPV has been blamed in etiology. Genital HPV positivity at varying rates in genital and extra-genital located SK’s has been reported. In this study, we reported a 35 year-old male case applied to the hospital with common verrucous masses in external genital region. Dermoscopic examination showed fissures and ridges, cerebriform appearance comedo-like openings, consistent with SK. The histopathology confirmed the diagnosis of SK. In histopathologic examination, the acanthosis that include basaloid cell proliferation consisting of thick keratohyalin granules and a keratinized plug structures drew attention on analysis of the sections. HPV was determined on biopsy material through the PCR method. HPV6 and HPV16, HPV6 as the dominant, were determined in performed HPV typing. In our case, HPV growth is a finding supporting the role of HPV in etiology.

Recurrence of Nevus Lipomatosus Cutaneous Superficialis after CO2 Laser Treatment

Recurrence of Nevus Lipomatosus Cutaneous Superficialis after CO2 Laser Treatment

Giant seborrheic keratosis of the genitalia

Genital seborrheic keratosis (SK) is a rare entity, which can be easily misdiagnosed as genital warts. Dermoscopy is a useful tool to make diagnosis of SK in such cases. We report a 50-year-old woman with a large polypoidal growth on the external genitalia. Dermoscopic examination showed fissures and ridges, cerebriform appearance, and comedo-like openings consistent with SK. The histopathology confirmed the diagnosis of SK.

Folliculotropic T-Cell Lymphocytosis as a Distinct Form of Pilotropic T-Cell Dyscrasia

Cutaneous T-cell dyscrasia represents a heterogeneous group of persistent clonal and usually epitheliotropic T-cell infiltrates with a seemingly low risk for progression to mycosis fungoides (MF). Mucin-poor folliculotropic T-cell lymphocytosis is the least well characterized with only a few anecdotal case reports. Cases of folliculotropic lymphocytosis were retrieved via a natural language search from 2 dermatopathology databases. Comprehensive light microscopic, phenotypic, and molecular studies were conducted. There were 12 females and 12 males, with mean age of 41 years. There were 2 main clinical presentations: facial erythematous infiltrative papules and/or nodules vs extremity and truncal solitary, large, scaly plaques. The mean duration of the lesions was 3 years. Progression to follicular MF was noted in 1 case. Migration of small lymphocytes into the superficial portions of the hair follicle unaccompanied by other inflammatory cells or mucin was observed; lymphocytes with a cerebriform appearance could be seen. The CD4/CD8 ratio was more than 5:1 with significant reductions in the expression of CD62L and CD7. Of 20 cases tested, 5 showed monoclonality. Folliculotropic T-cell lymphocytosis is a form of pilotropic T-cell dyscrasia distinct from MF and alopecia mucinosa; the course seems indolent in most cases.

Experimental infections with Paracoccidioides brasiliensis obtained from armadillos: comparison to clinical isolates

Paracoccidioides brasiliensis causes paracoccidioidomycosis (PCM) that is one of the most prevalent systemic human mycoses in Latin America. Armadillos show a high incidence of PCM infection and could, therefore, be a natural reservoir for this fungus. In this study were compared the virulence profiles of isolates obtained from nine-banded armadillos (Dasypus novemcinctus) (PbT1 and PbT4) and isolates from PCM patients (Pb265 and Bt83). Pathogenicity was evaluated by fungal load and analysis of colony morphology. Immunity against the fungus was tested by delayed type hypersensitivity test (DTH) and antibody quantification by ELISA. The higher virulence of PbT1 and PbT4 was suggested by higher fungal load in spleen and lungs. Armadillo isolates and Bt83 presented a cotton-like surface contrasting with the cerebriform appearance of Pb265. All isolates induced cellular and humoral immune responses in infected BALB/c mice. DTH reactions were similarly induced by the four isolates, however, a great variability was observed in specific antibody levels, being the highest ones induced by Bt83 and PbT4. The present work confirms that armadillos harbor P. brasiliensis, whose multiplication and induced immunity in experimentally infected mice are heterogeneous, resembling the behavior of isolates from human PCM. This study reinforces the possibility that armadillos play an important role in the biological cycle of this pathogen.

Proteus syndrome with giant myelolipoma in the pelvis

Proteus syndrome is a very rare and complex disorder with malformations and overgrowth of multiple tissues. This disorder was designated Proteus syndrome by Wiedemann et al. to denote its variable clinical expression. Our patient presented with macrodactyly, cerebriform appearance and a huge abdominal mass. A biopsy under laparotomy was performed, and histopathological examination revealed myelolipoma. Tumor resection was performed. To our knowledge, no case of Proteus syndrome presenting a myelolipoma in the retroperitoneal cavity has been reported before.

Essential primary cutis verticis gyrata

OBJECTIVE: To report a rare case of a child with essential primary Cutis verticis gyrata. REPORT: Nine-year-old boy with extensive hypertrophy of scalp skin, with a cerebriform appearance. No underlying neurologic and ophthalmologic disorders were found, and no other cases were described in his family. COMMENTS: The diagnosis of primary Cutis verticis gyrata was established by thickening of the scalp and absence of neurologic and ophthalmologic abnormalities. Differential diagnosis comprises secondary conditions such as: cerebriform intradermal nevus, pachydermoperiostosis, acromegaly, and inflammatory diseases of the scalp. This is the first report of a child with this form of Cutis verticis gyrata.