Maria Cecília Rivitti Machado

Prevenção e cuidados com a pele da criança e do recém-nascido

Neonatal skin suffers a progressive adaptation to the extrauterine environment and special care is needed during this period. This skin is very sensitive, thin and fragile. Immaturity of the epidermal barrier reduces the defense against the excessive proliferation of microbes and makes the skin more vulnerable to trauma and percutaneous drug toxicity. Because of the peculiar characteristics of newborn, infant and childrens skin, the use of cosmetic products designed for hygiene and protection requires caution. In order to preserve the integrity of neonatal and childs skin, this article reviewed basic preventive care practices in relation to hygiene, bathing, cleansing agents, topical products and their percutaneous toxicity.

Elejalde syndrome: report of a case and review of the literature.

Abstract:  Elejalde syndrome is a rare autosomal recessive condition, with only 10 reported cases through 2001. It is characterized by silvery hair, pigment abnormalities, and profound central nervous system dysfunction. The differential diagnosis includes Griscelli and Chediak‐Higashi syndromes, which present with silvery hair, pigment abnormalities, central nervous system alterations, and severe immunologic dysfunction. We report a 6‐year‐old girl with Elejalde syndrome and review Elejalde, Griscelli, and Chediak‐Higashi syndromes.

Clinical presentation and treatment of diaper dermatitis: part II

Diaper irritant contact dermatitis is the most prevalent diaper dermatitis and, probably, the most common cause of cutaneous disease in infants. Wearing diapers causes over-hydration and increase of local temperature and humidity. As a consequence, the skin becomes susceptible to friction from movement under the diaper. Occlusion, maceration and possibly Candida and bacteria may all play a role. Oils, soaps and powders can be irritants and aggravate the eruption. The best thing to do is prevention. Treatment is simple and depends on dermatitis type and severity.

Polarized light microscopy of hair shafts aids in the differential diagnosis of Chédiak-Higashi and Griscelli-Prunieras syndromes

PURPOSE To study and compare the appearance of hairs from patients with Chédiak-Higashi and Griscelli-Prunieras syndromes under light and polarized light microscopy. METHOD Hairs from 2 Chédiak-Higashi and 2 Griscelli-Prunieras patients were obtained and examined under normal and polarized light microscopy. RESULTS Under light microscopy, hairs from Chédiak-Higashi patients presented evenly distributed, regular melanin granules, larger than those seen in normal hairs. Under polarized light microscopy, shafts exhibited a bright and polychromatic refringence appearance. In contrast, hair from Griscelli-Prunieras patients, under light microscopy, exhibited bigger and irregular melanin granules, distributed mainly near the medulla. Under polarized light microscopy, shafts appeared monotonously white. CONCLUSION Light microscopic examination of hair shafts of patients with Chédiak-Higashi or Griscelli-Prunieras syndrome reveals subtle differences that are useful in identifying both disorders, but not in distinguishing between them. We provide evidence that polarized light microscopy of hair shafts, an approach that has not been previously described, aids in differentiating between these syndromes. We propose hair study by polarized light microscopy as a helpful complementary diagnostic method for differential diagnosis between CHS and GPS, especially when the more sophisticated molecular studies are not available.

Apresentação, evolução e tratamento dos hemangiomas cutâneos: experiência do ambulatório de Dermatologia Infantil do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

FUNDAMENTOS: Os hemangiomas, proliferacoes benignas do tecido endotelial, sao os tumores mais comuns da infância, e a maioria possui apresentacao e crescimento tipicos. OBJETIVOS: Relatar a experiencia do Ambulatorio de Dermatologia Infantil do Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo em relacao a apresentacao, evolucao e ao tratamento dos hemangiomas da infância, correlacionando-os com dados da literatura. METODOS: Foram avaliados retrospectivamente, mediante analise de prontuarios e registros fotograficos, 40 casos de hemangiomas atendidos no periodo de marco de 1994 a novembro de 2004. RESULTADOS: Houve predominio do sexo feminino na razao de 3:1, e maior numero de pacientes brancos (75%). O hemangioma estava presente ao nascimento em 77% dos pacientes. As lesoes eram unicas em 85% dos casos, e a maior parte se localizava na regiao da cabeca e pescoco (57%). A conduta expectante foi a mais adotada, e o seguimento dos pacientes mostrou inicio da involucao da lesao antes dos 12 meses de idade em 65% dos casos. A resolucao total ocorreu ate tres anos de idade em 50% dos pacientes, ate os seis anos em 58%, ate os nove anos em 83% e ate os 11 anos em 100%. CONCLUSOES: A casuistica dos autores e superponivel a da literatura mundial em relacao as variaveis sexo e cor dos pacientes, numero e localizacao dos hemangiomas, porem com maior porcentagem de lesoes presentes ao nascimento

Identification of XP Complementation Groups by Recombinant Adenovirus Carrying DNA Repair Genes

lyzed in skin-draining lymph nodes (LN; Figure 2l). Upon MC903 treatment, the induction of Th2 cytokines (IL4, IL13, IL10, IL6, and IL31) in WT LNs was not observed in TSLP / LNs, indicating that the MC903-triggered Th2 response is abolished in TSLP / mice. No change was seen for IFNg transcript levels in WT or TSLP / LNs, from either MC903or ethanol-treated mice, suggesting that MC903 application does not involve a Th1 response in LNs. In conclusion, we demonstrate unequivocally here that TSLP produced by keratinocytes is absolutely required in pathogenesis of AD triggered by topical application of the vitamin D3 analogue MC903. We also show that in this AD model, induction of IL4, IL13, IL31, and eotaxin-2 is fully TSLP dependent, whereas that of IL6, IL10, and monocyte chemotactic protein-2 is only partially TSLP dependent, and that of IFNg is TSLP independent. Finally, our floxed TSLP mice will be helpful for selective ablation of TSLP in other cell types (Soumelis and Liu, 2004; Ziegler and Liu, 2006; Holgate, 2007; Sokol et al., 2008), and therefore to further elucidate the physiological and pathological function of this cytokine.

Fisiopatologia da dermatite da área das fraldas: parte I

Irritant contact dermatitis is the most prevalent diaper dermatitis and, probably, the most common cause of skin disease in infancy. The wearing of diaper leads to overhydration, increased local temperature and humidity. Constant maceration and prolonged contact with urine and stools makes the skin under the diaper more susceptible. There is often secondary infection due to Candida or bacteria, such as Bacillus faecallis, Proteus, Pseudomonas, Staphylococcus e Streptococcus. Oils, soaps, powders and ointments can be irritants and aggravate the rash. It is important to know the pathophysiology of the disease for appropriate treatment and prevention

Translation, cross-cultural adaptation and validation of the Quality of Life Evaluation in Epidermolysis Bullosa instrument in Brazilian Portuguese.

The Quality of Life Evaluation in Epidermolysis Bullosa (QoLEB) questionnaire was developed for use in English‐speaking individuals.

Increased Melanocytic Nevi in Patients with Inherited Ichthyoses: Report of a Previously Undescribed Association

Abstract:  Ichthyosis is a heterogeneous cornification disorder. Melanocytic lesions have not been previously described in association with ichthyosis. Their clinical importance lies in the fact that they may simulate melanoma clinically and dermoscopically, as seen in epidermolysis bullosa. The objective of this study was to evaluate the clinical, dermoscopic, and histopathologic features of nevi and lentigines in 16 patients with autosomal recessive congenital ichthyosis—lamellar ichthyosis and nonbullous ichthyosiform congenital erythroderma. Patients underwent general clinical examination dermoscopy. The more suspicious lesions were excised and to histopathologic examination. Most patients (n = 13) reported no personal or familial history of melanoma or atypical nevi. All of the patients had at least five atypical melanocytic lesions. Ten of the 16 patients had at least one atypical nevus or lentigo. This study suggests that increased atypical melanocytic nevi may be a feature of long‐standing congenital ichthyoses. Whether this finding is disease‐related or a coincidental observation is difficult to ascertain. As an unequivocal discrimination from malignant melanoma in vivo is not always possible, regular clinical follow‐up of patients with ichthyosis and increased or unusual nevi is recommended.

Generalized morphea in a child with harlequin ichthyosis: a rare association

INTRODUCTION Harlequin ichthyosis (HI) is a severe and rare hereditary congenital skin disorder characterized by excessive dryness, ectropion and eclabion. The association of ichthyosis with systemic sclerosis has been described in only three children. No patient with generalized morphea (GM) associated with harlequin ichthyosis was described. CASE REPORT A 4-years and 6-months girl, diagnosed with harlequin ichthyosis based on diffuse cutaneous thickening, scaling, erythema, ectropion and eclabium since the first hours of birth was described. She was treated with acitretin (1.0mg/kg/day) and emollient cream. At 3 years and 9 months, she developed muscle contractures with pain on motion and limitation in elbows and knees, and diffuse sclerodermic plaques on the abdomen, back, suprapubic area and lower limbs. Skin biopsy showed rectified epidermis and mild hyperorthokeratosis, reticular dermis with perivascular and periadnexal infiltrates of lymphocytes and mononuclear cells, and reticular dermis and sweat gland sclerosis surrounded by a dense collagen tissue, compatible with scleroderma. The patient fulfilled the GM subtype criteria. Methotrexate and prednisone were introduced. At 4 years and 3 months, new scleroderma lesions occurred and azathioprine was associated with previous therapy, with no apparent changes after two months. DISCUSSION A case of harlequin ichthyosis associated with a GM was reported. The treatment of these two conditions is a challenge and requires a multidisciplinary team.