Abstract Background The prevalence of atopic eczema is unknown in many countries. The International Study of Asthma and Allergies in Childhood (ISAAC) is an epidemiological landmark in the study of allergic diseases. Objective To validate and assess the reproducibility of the ISAAC Written Atopic Eczema Questionnaire (WAEQ) for children aged between 6 and 7 years by telephone contact. Methods Observational study through interviews with guardians of children aged 6 to 7 years using the ISAAC atopic eczema module questionnaire in three different phases separated by 2 weeks: telephone interviews in the first and third contacts and in-person interviews under supervision in the second contact. Reproducibility was estimated using the Kappa index and validation using the sensitivity and specificity coefficients. Results Data from 88 children (32 from the atopic eczema group) were analyzed. Reproducibility showed almost perfect agreement for the questions “Recurrent pruritic lesions” and “Lesions in typical locations” (Kappa between 0.81-0.82), while a substantial agreement was observed for all other indicators (Kappa variation between 0.66 and 0.78). The validation showed high specificity (≥ 80.4%) and sensitivity (≥ 87.5%) for all questions, except those related to chronicity and medical diagnosis (34.4% and 40.6%, respectively). Study limitations Non-random selection, no sample size calculation, participants from a tertiary hospital and study period coincident with the Coronavirus pandemic. Conclusions Our results showed that the ISAAC atopic eczema module questionnaire by telephone interviews has good reproducibility and high agreement with the clinical diagnosis of atopic eczema. It may be an appropriate alternative tool in epidemiological studies of childhood atopic eczema, especially in periods of social isolation.

Monilethrix is a rare hereditary condition generally considered to be an autosomal dominant disorder with variable penetrance. A case of a 6-year-old girl without a familial background for this disease is reported. The diagnosis was made by optic microscopy and dermoscopy. A therapeutic trial with topical minoxidil was conducted.

Kwashiorkor é um tipo de desnutrição proteico-energética em que há deficiência dietética de proteína, embora a ingestão de calorias se mantenha adequada. As manifestações cutâneas incluem pele xerótica, com aspecto de esmalte descascado, típica coloração avermelhada a branco-acinzentada dos cabelos, o sinal da bandeira e edema mais evidente, nos membros inferiores e na face, dando aspecto de lua cheia. O presente artigo relata o caso de um paciente adulto, do sexo masculino, previamente submetido à duodenopancreatectomia para tratamento de pancreatite crônica associada ao pseudotumor em cabeça de pâncreas que evoluiu com alterações cutâneas de kwashiorkor após tuberculose pulmonar.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.

Os autores relatam um caso clínico sobre Síndrome SAPHO, na faixa etária pediátrica, com enfoque dermatológico. Essa entidade deve ser considerada, nos pacientes que tenham dor na parede torácica anterior ou outros sintomas musculoesqueléticos, acompanhados por lesões dermatológicas, como pustulose palmoplantar e acne fulminans. As manifestações cutâneas específicas, diagnóstico e o tratamento utilizado serão apresentados

The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus. This entity should be considered in patients who have pain in the anterior chest wall or other musculoskeletal symptoms, accompanied by palmoplantar pustulosis and acne fulminans. The specific cutaneous manifestations, diagnosis and the treatment will be presented

A síndrome de extravasamento capilar sistêmico é rara e causada por aumento da permeabilidade capilar. Várias etiologias estão envolvidas. No nosso serviço de Dermatologia, a principal é a psoríase instável. Diversos tratamentos são usados e muitos ainda estão em estudo. Nosso objetivo foi apresentar esta situação clínica potencialmente fatal que ocorre em nossa especialidade

The systemic capillary leak syndrome is rare and caused by increased capillary permeability. Several etiologies are involved. In our Department of Dermatology the main one is unstable psoriasis. Several treatments are used and many are still under study. Our objective was to present this potentially fatal medical condition that occurs in our specialty

A Síndrome de Bloch-Sulzberger (Incontinência Pigmentar) é uma genodermatose rara, que afeta, principalmente, o sexo feminino, pois costuma ser letal em pacientes do sexo masculino intraútero. Caracteriza-se, principalmente, pelas manifestações dermatológicas, podendo também apresentar anomalias dentárias, oftalmológicas e neurológicas. As lesões cutâneas apresentam 4 fases distintas: vesiculosa, verrucosa, pigmentar e atrófica; que podem seguir uma sequência irregular, havendo até sobreposição das mesmas

Bloch-Sulzberger syndrome (incontinentia pigmenti) is a rare genodermatosis that affects predominantly females, since it is generally lethal to male fetuses in utero. It is characterized principally by skin lesions, but may also involve dental, ophthalmological and neurological abnormalities. The skin lesions are present in four different phases: vesicular, verrucous, hyperpigmented and atrophic/hypopigmented. Their sequence is irregular and overlapping of stages is common

Relatamos um caso de higroma cístico na face de uma criança de quatro meses de idade. Havia história de aumento de volume congênito na hemiface direita que involuiu consideravelmente após sinais de infecção. O higroma cístico ou linfangioma é uma malformação congênita rara do sistema linfático, presente ao nascimento em 50% dos casos. Localiza-se preferencialmente na região cervical e na face. Geralmente evolui com crescimento lento e progressivo, podendo comprimir e infiltrar estruturas adjacentes. Sua regressão espontânea ocorre em apenas 6% dos casos.

This report describes the case of a cystic hygroma on the face of a four-month old child. There was a history of congenital swelling of the right hemiface that decreased considerably following signs of infection. The cystic hygroma or lymphangioma is a rare congenital malformation of the lymphatic system that is present at birth in 50% of cases. It is usually located on the neck or face. It generally grows slowly and progressively and may compress and infiltrate adjacent structures. Its spontaneous regression occurs in only 6% of cases.

A hiperceratose epidermolítica é doença genética da queratinização e inicialmente foi descrita sob a sinonímia de eritrodermia ictiosiforme congênita bolhosa. Caracteriza-se por apresentar herança autossômica dominante, podendo ocorrer mutação espontânea em 50% dos casos. Usualmente apresenta-se ao nascimento com bolhas, eritema e descamação, evoluindo para hiperceratose com ou sem eritrodermia associada. A histopatologia é típica, destacando-se camada córnea intensamente espessada, com degeneração vacuolar na porção superior da epiderme. Têm sido relatados casos esporádicos por mutação pós-zigótica durante a embriogênese, caracterizando o padrão em mosaico do envolvimento cutâneo, com áreas de hiperceratose alternando com pele sã, seguindo as linhas de Blaschko. As mutações têm sido relatadas nos genes das citoqueratinas 1 e 10. Os autores apresentam o caso de um adolescente de 15 anos de idade, que aos 10 meses de vida apresentou vesículas e bolhas que evoluíram para lesões hiperceratósicas acometendo os membros, o tronco e a região cervical. Tais lesões seguem as linhas de Blaschko e se alternam com pele sã. O exame histopatológico foi compatível com hiperceratose epidermolítica. O paciente recebeu tratamento com acitretin, obtendo boa resposta.

Epidermolytic Hyperkeratosis is a genetic disorder of keratinization, initially described as bullous ichthyotic erythroderma. It is transmitted as an autosomal dominant trait, although 50% of cases may occur as spontaneous mutations. This disease frequently presents with blistering, redness and peeling at birth. With time it may develop into generalized hyperkeratosis with or without erythroderma. It has a distinctive histopathologic feature of vacuolar degeneration of the upper epidermis and associated thick stratum corneum. Sporadic epidermolytic hyperkeratosis due to postzygotic, spontaneous mutation during embryogenesis may appear in a mosaic pattern of skin involvement showing areas of hyperkeratosis distributed in streaks along Blaschko's lines, and alternating with normal skin. Mutations occur in either keratin 1 and 10. The authors present the case of a 15 year-old adolescent, who since the age of ten has presented vesico-bullous lesions that gradually became hyperkeratotic. They affected the limbs, the body and the cervical region following Blaschko's lines, and alternating with normal skin. The histopathologic study was compatible with epidermolytic hyperkeratosis. He was treated with acitretin, which obtained a good response.