摘要:

Abstract:With few exceptions, epidermolysis bullosa simplex (EBS) is transmitted as an autosomal dominant trait. All cases of autosomal recessive EBS reported to date have been associated with significant extracutaneous disease {including anemia, marked growth retardation, dentition abnormalities, and/or concurrent neuromuscular disease}, early infant mortality, or often, rather extensive cutaneous involvement. In some, the cutaneous morphology has even suggested junctional or dystrophic disease. We cared for a kindred in which four individuals were affected with an autosomal recessive form of EBS with cutaneous findings most often suggestive of Weber‐Cockayne disease. Except for scattered oral erosions in one patient, there was no evidence of associated extracutaneous disease. These findings demonstrate the increasing complexity of even the simplex forms of inherited epidermolysis bullosa. This obviously has implications for genetic counsellin

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00256.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:White dermographism constitutes an abnormal vascular reaction characteristically demonstrable in atopic dermatitis; however, there is no information about it in the infantile phase of atopic dermatitis. Therefore we examined 73 infants younger than 3 years of age with eczematous dermatitis for the demonstrablity of white dermographiam after mechanical strocking of the lesional skin. None of the 40 healthy control infants showed white dermographism on their normal skin. In contrast, an age‐dependent increase was demonstrated in patients with infantile eczema, from 11% in those 1 to 2 months of age to 85% in those older than 7 months. There was no correlation between the demonstrabllity of white dermographism in early infancy and the prognosls of infantile eczema. Based on our study of various types of dermatitis experimentally induced in adult volunteers, we think that, in addition to the immaturity of infantile skin, the presence of acute dermatitic changes may be related to Inabllity to demonstrate with dermographism in the early phase of infantile eczem

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00257.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:Severe allergic protein‐losing enteropathy has been described in childhood atopic dermatitis (AD). Minor morphologic and functional abnormalities of the gastrointestinal mucosa, including altered permeability to oligosaccharides, are not uncommon in this condition, but the prevalence of occult enteric protein loss is unknown. We measured the random fecal α1‐antitrypsin (AAT) concentration, an indicator of enteric protein loss, in children with and without AD and found no significant difference between the groups. In half of the patients with AD, gastrointestinal permeability to oligosaccharides was also measured, and no relationship between this and fecal AAT was f

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00258.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:Based on clinical features, infantile seborrheic dermatitis (ISD) can be classified as follows: true seborrheic dermatitis (SD), psoriasiform seborrheic dermatitis (Psor SD), and erythrodemic seborrheic dermatitis. We reviewed the records of 72 children who had been affected by ISD several years earlier to investigate the evolution of disease, to evaluate the patients for the presence of new skin lesions, and to study family histories with respect to these conditions. In addition, we attempted to determine if there is any connection among the initial features of ISD and the types of skin lesions, and atopy or psoriasis. On reexamination, our patients previously diagnosed as having SD showed a larger variety of new skin lesions than those who had Psor SD, and 15% had developed atopic dermatitis. Atopic dermatitis was not present at follow‐up in the children with previously diagnosed Psor SD. Psoriatic lesions were present in similar percentages in both groups at the time of reevaluation. Investigation of family history was not useful in predicting later development of psoriasis or atopic dermatitis. We conclude that the initial clinical features of ISD may be of prognostic value, and that SD and Psor SD are probably two different clinical entitie

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00259.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:The presence of Pityrosporum ovale was investigated in four groups of infants age 1 to 24 months, 15 with infantile seborrheic dermatitis, 15 with infantile atopic dermatitis, 15 with other infantile dermatoses, and 15 healthy infants. Samples were taken from the scalp, face, presternal area, and inguinal area.Pityrosporum ovalewas detected by smears and/or cultures in 73% of infants with seborrheic dermatitis, 33% with atopic dermatitis, 33% with other dermatoses, and 53% of healthy infants. The percentages of positive smears and/or cultures from four body sites in each patient group were 42% for seborrheic dermatitis, 20% for atopic dermatitis, 20% for other infantile dermatoses, and 23% for healthy infants. The majority of infants with positive cultures or positive direct examination for P. ovale were between 1 and 8 months of age. The organism was isolated in 28% of samples taken from the scalp, 32% from the face, 30% from the presternal area, and 15% from the inguinal area. Patients with infantile seborrheic dermatitis were treated with 2% topical ketoconazole cream for two weeks. Eleven of these children were clinically cleared and 13 became mycologically negative.Pityrosporum ovale was significantly more frequent in infants with seborrheic dermatitis than in those with atopic dermatitis, in other infantile dermatoses, or in healthy infants, both in the total number of infants with positive smears and/or cultures and in the number of positive samples per body area (P<0.05).

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00260.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:A Child with epidermolysis bullosa dystrophica, recessive type (EBDR) developed significant anemia at 9 years of age and was treated with long‐term transfuslon therapy. At age 17 he had symptoms of congestive heart failure secondary to dilated cardlomyopathy. Treatment with digoxin and vasodilators for the past year has failed to improve his cardiomyopathy significantly. Chronic iron overload and secondary hemosiderosis may have contributed to his problems, and we propose that chelation therapy be used in any child received long‐term transfusion ther

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00261.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:An infant with congenital ichthyosis and deafness developed Hirschsprung's disease. No evidence of keratitis was present. No previous cases of ichthyosis have been associated with aganglionic megacolon. Although no corneal changes were observed, we believe that the clinical features of ichthyosis and deafness suggest the diagnosis of Kid (keratitis, ichthyosis, deafness) syndrome. Whether corneal changes would have occurred is unknown, since the infant died of mainutrition and infectious complications.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00262.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:A newborn male Thai infant had numerous brownish red nodules of various sizes scattered over both palms and soles, left thigh, abdomen, chin, and left upper eyelld. An extremely large tumor mass was present on the right sole. Many lesions showed spontaneous ulceration. No extraculaneous involvement was found. All lesions involuted spontaneously within two to three months, some with scar formation. histology showed large numbers of mononuculeated and multinucleated histiocytic cell infiltrations, 10% of which contained Birbeck's granules. Areas of necrosis and calcification were also seen in the largest tumor. No recurrence was observed after follow‐up for three year

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00263.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:Neutrophilic eccrine hidradenitis (NEH) is a recently recognized dermatosis occurring in patients receiving chemotherapy for a variety of malignancies. We report the second pediatric case. An 11‐year‐old boy with non‐Hodgkin's lymphoma developed widespread erythematous papulopustules beginning two days after receiving high‐dose cytarabine (Ara‐C) in preparation for a bone marrow transplant. The lesions spontaneously regressed in two weeks. Histologic examination revealed a neutrophilic infiltrate around and within the eccrine ducts and secretary coils. Bacterial, fungal, and viral cultures were negative. these findings are characteristic of NEH. The condition should be differentiated from infectious dermatoses that may require

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00264.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Abstract:A total of 900 consecutive newborns delivered at the Nehru Hospital, Chandigarh, India, over a period of 7 months were examined for presence of skin lesions within 48 hours of birth. Commonly observed skin lesions were Epstein pearls (88.7%), mongollan spots (62.2%), milia (34.9%), sebaceous hyperplasia (31.8%), salmon patches (28.4%), and er‐ ythema toxicum neonatorum (20.6%). These figures are comparable with earlier reports. Impetigo neonatorum occurred in 11.3% of Infants, and was frequent in our hot and humid climate from May to August. Traumatic skin lesions were most often present in babies who had forceps deliver‐ies. Three hundred ten (34%) babies were available for follow‐up up to six weeks. Additional skin lesions observed were omphalitis (16 babies), oral thrush (9) and postinflammatory hypomelanosis (8). Three infants had atopic dermatitis, two each had seborrheic dermatitis, diaper dermatitis, pityriasis velrsicolor, and nevus achromicus. One each had vltillgo, ichthy‐osis vulgaris, urticaria, and strawberry hemangloma. These observations highlight the importance of repeat examination for the appearance of skin lesions during the neonatal

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1989.tb00265.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY