ABSTRACT Rubinstein-Taybi syndrome is a rare disorder characterized by craniofacial dysmorphisms, thumb and hallux alterations, associated with growth and mental deficiency. We describe a 25-year-old male patient, classically presenting the phenotypic features of the syndrome, who sought allergologic evaluation to assess the possibility of food allergy due to the presence of eosinophilic esophagitis. Evaluation with specific tests were normal. Further investigation showed a history of severe systemic infections that had not yet been properly screened, and laboratory investigation showed a deficiency in the production of Immunoglobulin G and its subclasses, as well as specific antibodies. He had no cytopenias, and lymphocyte immunophenotyping was normal. We aim to show that considering the recurrence of infections, notably severe ones requiring hospitalization, an adequate immunological evaluation should be performed in these patients.

RESUMO A síndrome de Rubinstein-Taybi é um raro distúrbio caracterizado por dismorfismos craniofaciais, alterações dos polegares e hálux, associado a deficiência do crescimento e mental. Descrevemos paciente masculino, 25 anos, apresentando classicamente as características fenotípicas da síndrome, que procurou avaliação alergológica, para a possibilidade de alergia alimentar, devido a presença de quadro de esofagite eosinofílica. Avaliação com exames específicos foram normais. Aprofundamento da investigação mostrou história de graves infecções sistêmicas ainda não devidamente rastreadas, e investigação laboratorial evidenciou deficiência na produção de Imunoglobulina G e de suas subclasses, bem como de anticorpos específicos. Não apresentava citopenias, e imunofenotipagem de linfócitos foi normal. Objetivamos mostrar que considerando a recorrência de infecções, notadamente graves necessitando de hospitalização, uma adequada avaliação imunológica deve ser realizada nesses pacientes.

Abstract Varicella is a common viral infection in childhood and usually has a benign evolution. However, the evolution can be severe in immunosuppressed children. Vaccination can prevent its occurrence and especially the development of severe and/or complicated cases. Methotrexate is a systemic therapeutic option for many inflammatory diseases, and its immunosuppressive action exposes its users to a higher susceptibility to infection. The present report describes a case of severe varicella infection in a child immunosuppressed with methotrexate.

ABSTRACT Propolis is a lipophilic resin extracted from plants by bees. The purpose of this case report was to show the importance of this substance as cause of allergic contact cheilitis. A 21-year-old female patient complained of pruritic perioral eczema for 5 years. In the past months it also affected the neck. After diagnosing contact dermatitis, she was submitted to a patch test with a Latin American baseline series. The result was strongly positive for propolis (++) and weakly positive for perfume mix I (+). After the test, the patient revealed she had been using propolis drops, per oris, for 10 years. The worsening of the condition was due to increased dose, aiming “to improve immunity”, during the coronavirus disease 2019 (COVID-19) pandemic. The contact allergy to propolis might be increasing due to the widespread use of natural products. Propolis is a sensitizer to be considered in patients with long-lasting cheilitis.

Abstract: Mycetoma is a chronic suppurative disease of the skin and subcutaneous tissue, characterized by a symptomatic triad: tumor, fistulas and grains. It can be caused by fungi (eumycetoma) and bacteria (actinomycetoma), with similar clinical features. Diagnosis is based on the clinical presentation and identification of the etiological agents in the tissue, by mycological/bacteriological, histopathological and immunohistochemical tests. It is important to specify the fungal or bacterial etiology, because the treatments are different. An approach that involves early diagnosis, the use of systemic antibiotics or antifungal agents, including surgical removal of lesions, is the basis for the treatment of these diseases. In this review, the most commonly used diagnostic methods and treatments will be discussed. Also, we will review the history of the disease through epidemiological and etiological aspects.

Abstract: Basal cell carcinoma and Squamous cell carcinoma, referred to as non-melanoma skin cancer, are the most common malignancies in humans. Their incidence is increasing worldwide every year. In Brazil, even with the advent of educational campaigns on photoprotection and laws that banned tanning beds, they are the most frequent neoplasias, representing a public health problem recognized by the Ministry of health.

Idiopathic Atrophoderma of Pasini and Pierini (IAPP) is a rare, exclusively cutaneous disease. It is more frequent in females, with incidence peak in the second and third decades of life. The etiopathogenesis remains unknown. IAPP most commonly affects the back, abdomen and proximal regions of the limbs. Lesions may be rounded, oval or circular; single or multiple. The evolution is variable and the course is initially progressive. Collagen changes such as atrophy, thinning, condensation and sclerosis may be observed in the papillary dermis. This paper describes a case of Idiopathic Atrophoderma of Pasini and Pierini with histopathologic findings.

O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, incomum na infância. Relatamos um caso de lactente feminina, com 3 meses de idade, que apresentou dados clínicos e laboratoriais confirmatórios para o diagnóstico de penfigoide bolhoso. Os autores utilizaram a coloração de imuno-histoquímica para o colágeno tipo IV que permitiu a diferenciação do penfigoide bolhoso de outras buloses subepidérmicas. Oportunamente, revisamos as características clínicas, imunológicas, terapêuticas e prognósticas da patologia na criança.

Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.

A síndrome de hiperimunoglobulinemia E, ou síndrome de Job é uma imunodeficiência primária rara, caracterizada por abcessos cutâneos recorrentes, infecções de repetição do trato respiratório, títulos elevados de IgE, eosinofilia, alterações ósseas e dentárias. Relata-se o caso de um paciente do sexo masculino, 14 anos, portador da doença, com manifestações desde típicas a algumas relativamente esporádicas, fazendo-se uma revisão da síndrome e dos achados clínicos associados.

The hyperimmunoglobulin E syndrome, or Job's syndrome is a rare primary immunodeficiency characterized by recurrent skin abscesses, recurrent respiratory tract infections, and high levels of IgE, eosinophilia, bone and dental changes. We report the case of a fourteen-year-old male patient presenting this disease, with both typical and also some relatively sporadic manifestations. We performed a literature review on the syndrome and its associated clinical findings.

FUNDAMENTOS: o crescente aumento do número dos transplantados renais tem favorecido o aparecimento mais frequente das dermatoses e permitido o estudo em sucessivos trabalhos. OBJETIVOS: avaliar a frequência das dermatoses em pacientes transplantados renais. MÉTODOS: captação de pacientes transplantados renais durante o período de 1° de março de 2009 a 30 de junho de 2010 com suspeita de dermatoses. RESULTADOS : foram avaliados 53 pacientes (28 homens e 25 mulheres), entre 22 e 69 anos (com uma média de 45 anos), a maioria procedente de Ceilândia, Samambaia e São Sebastião/DF, entre 5 e 10 anos de transplante renal (37,7%), sendo 62,3% receptor de doador vivo e 83% em uso de prednisona. As dermatoses mais prevalentes foram as de etiologia fúngica (45,3%) e viral (39,6%). Das neoplasias malignas não-melanoma, apesar da baixa incidência, predominou o carcinoma basocelular (seis casos). Com relação s dermatoses de origem fúngica, ocorreram 12 casos de onicomicoses, cinco casos de pitiríase versicolor e quatro casos de foliculite pitirospórica. Para realização do diagnóstico, na maioria dos casos (64,2%), foi utilizado os exames laboratoriais ( micológicos e histopatológicos). CONCLUSÃO: as manifestações cutâneas em pacientes transplantados renais são geralmente secundárias imunossupressão. As dermatoses infecciosas, principalmente as de etiologia fúngica, são frequentes em pacientes transplantados renais, e sua ocorrência aumenta progressivamente, conforme o tempo transcorrido, a partir do transplante, sendo importante o acompanhamento.

BACKGROUND: The increasing in the number of kidney transplant recipients has favored, more frequently than before, the emergence of dermatoses and warranted their study through subsequent publications. OBJECTIVES: to evaluate the frequency of dermatoses in kidney transplant recipients. METHODS: kidney transplant recipients with suspected dermatoses between March 1st 2009 and June 30th 2010. RESULTS: 53 patients (28 males and 25 females), aged between 22 and 69 (mean age = 45 years) were evaluated. Most of them came from the cities of Ceilândia, Samambaia and São Sebastião/DF, and had already been transplanted for 5 to 10 years before (37.7%); 62.3% were recipients of living donors and 83% were prednisone-treated. The most prevalent dermatoses were of fungal (45.3%) and viral (39.6%) etiologies. Among the non-melanoma malignant neoplasms, the basal cell carcinoma prevailed (six cases), in spite of the low incidence. Concerning fungal dermatoses, 12 cases of onychomycosis, five of pityriasis versicolor and four of pityrosporum folliculitis were reported. For diagnosis, in most cases (64.2%), laboratory examinations (mycological and histopathological) were performed. CONCLUSION: cutaneous manifestations in kidney transplant recipients are generally secondary to immunosuppression. The infectious dermatoses, especially those of fungal origin, are frequently found in kidney transplant recipients and their occurrence increases progressively according to the time elapsed from the transplantation, which makes follow-up important.

Aureobasidium pullulans is a causal agent of phaeohyphomycosis, occasionally found in men and animals. As an agent of different opportunistic fungal processes, it may cause fungemia, systemic infections and abscesses in different viscera. This paper aims to report a case of a patient with infection of the lymphatic system by A. pullulans. A 23-year-old patient being treated for erythema nodosum leprosum presented a 60-day complaint of daily fever, hoarseness, odynophagia and weight loss. Laboratory tests showed pancytopenia with severe neutropenia, cervical adenomegaly and solid contrast uptake lesion in the oropharyngeal region. Due to neutropenia and sepsis the patient was initially treated with cefepime and vancomycin, but there was no clinical improvement. Lymph node puncture-aspiration showed yeast-form fungus identified as A. pullulans by sequencing ITS region. The patient was treated with amphotericin B deoxycholate, leading to complete recovery of bone marrow function and regression of adenomegaly and the oropharyngeal lesion.

Esteatocistoma múltiplo é um raro transtorno genético autossômico dominante que se caracteriza por múltiplos cistos dérmicos de tamanho variável e assintomáticos. Descreve-se o caso de um paciente do sexo masculino, de 23 anos, com quadro clínico e evolutivo típicos dessa desordem.

Steatocystoma multiplex is a rare genetic disorder, autosomal dominant, that is characterized by multiple asymptomatic dermal cysts which vary in size. It is described here the case of a 23 year-old male patient with a typical clinical and evolutional progression of this disease.

A cromomicose é micose subcutânea crônica sistêmica caracterizada por resposta inflamatória crônica granulomatosa. No entanto, existem poucos dados a respeito do padrão de subtipos de leucócitos na cromomicose e sobre as vias envolvidas no recrutamento destas células. O objetivo deste trabalho foi avaliar os tipos celulares, bem como a expressão de quimiocinas, receptores de quimiocinas e enzimas em lesões de cromomicose. O infiltrado inflamatório foi caracterizado por meio de técnica imuno-histoquímica utilizando os seguintes marcadores CD68 (macrófagos), S100 (células de Langerhans), CD3, CD4, CD8, CD45RO, CD20 e CD56 (linfócitos) e CD15 (neutrófilos). A expressão de MIP-1alfa (Proteína Inflamatória do Macrófago-1alfa), receptores de quimiocinas (CXCR3 e CCR1) e enzimas (superóxido dismutase-SOD e óxido nítrico sintase induzida-iNOS) foi avaliada pelo mesmo método. Observou-se um aumento de todas as populações celulares avaliadas em relação às amostras controle. As populações de células CD15+ e CD56+ foram significativamente menores que células CD3+, CD4+, CD20+ e CD68+. A análise estatística revelou uma correlação positiva entre o número de fungos com as células CD3, CD45RO e iNOS-positivas. A expressão de MIP-1alfa foi também associada às populações de células CD45RO, CD68, iNOS e CXCR3 positivas. Nossos resultados apontam para um possível papel de MIP-1alfa e da persistência fúngica na infiltração de células inflamatórias nos sítios de cromomicose.

Chromoblastomycosis (CR) is a subcutaneous chronic mycosis characterized by a granulomatous inflammatory response. However, little is known regarding the pattern of leukocyte subsets in CR and the pathways involved in their recruitment. The objective of this study was to assess the cellular subsets, chemokine, chemokine receptors and enzymes in CR. The inflammatory infiltrate was characterized by immunohistochemistry using antibodies against macrophages (CD68), Langerhans'cells (S100), lymphocytes (CD3, CD4, CD8, CD45RO, CD20 and CD56) and neutrophils (CD15). The expression of MIP-1alpha (Macrophage inflammatory protein-1alpha), chemokine receptors (CXCR3 and CCR1) and enzymes (superoxide dismutase-SOD and nitric oxide synthase-iNOS) was also evaluated by the same method. We observed an increase in all populations evaluated when compared with the controls. Numbers of CD15+ and CD56+ were significantly lower than CD3+, CD4+, CD20+ and CD68+ cells. Statistical analysis revealed an association of fungi numbers with CD3, CD45RO and iNOS-positive cells. Furthermore, MIP-1alpha expression was associated with CD45RO, CD68, iNOS and CXCR3. Our results suggest a possible role of MIP-1alpha and fungi persistence in the cell infiltration in CR sites.

É descrito um caso de pseudomicetoma dermatofítico, recidivante, em paciente com lesões no couro cabeludo. O exame histopatológico de uma das lesões mostrou, nos tecidos, os agregados micelianos ou pseudogrânulos, característicos da enfermidade. Dos tecidos frescos de outras lesões foi isolado o dermatófito Trichophyton tonsurans. Exames micológicos adicionais falharam em demonstrar a existência de tinea capitis, possível fonte do pseudomicetoma. A raridade dessa forma de dermatofitose profunda justifica a presente publicação.

A case of dermatophytic pseudomycetoma, in a patient with a history of recurrent lesions in the scalp, for over two years, is reported. Histopathological examination of the affected tissues revealed the mycelial aggregates or pseudogranules characteristic of the disease. Cultures of fresh tissues samples detected a fungus identified as Trichophyton tonsurans. Additional examinations failed to demonstrate lesions of tinea capitis, a probable source of the deep infection. Dermatophytic pseudomycetoma is an extremely rare form of dermatophytosis, and the rarity of such cases is the motive for the present communication.