摘要:

Abstract:Aberrant embryologic development of the neural crest may clinically result in one of several patterns of malformation. Four examples of the neurocristopathy syndrome are described that collectively feature a very broad range of expression in these infants. Because of the many expressions, the syndrome may not be recognized. We believe that greater familiarity with the disorder will aid in the discovery of unsuspected anomalies and provide clues about predisposing etiologies.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00660.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:Uncombable‐hair syndrome is a rare disorder of scalp hair that starts early in childhood. It is characterized clinically as unruly hair and by scanning electron microscopy as longitudinally grooved hairs with a triangular cross section. Because of the controversy of inheritance and prior reports of similar changes in normal and other hair disorders, we performed a quantitative scanning electron microscopic assessment of four patients with uncombable‐hair syndrome, first‐degree relatives, and nonaffected controls. Our results indicate that longitudinal grooving of scalp hair is a common occurrence, and that for clinical changes to be apparent, approximately 50% of hairs must be aff

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00661.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:Eight patients were treated for perianal streptococcal dermatitis. The condition previously was described as perianal cellulitis, a term that is confusing in light of the clinical features and distinctive appearance of the disorder.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00662.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:A retrospective study was carried out in 40 infants, age 1 to 24 months, with urticaria. Acute urticaria was seen most frequently (85%), followed by recurrent (10%) and chronic disease (5%). Several clinical features such as frequency of angloedema and hemorrhagic lesions appeared to be specific to urticaria in infants. An underlying cause was identified or suspected in 65% of cases: foods in 25%, and drugs and infections in 37.5%. Under 6 months of age, all infants had acute urticaria and 75% had cow's milk allergy. After 6 months of age, the main causes were drug intake (mostly aspirin and amoxicillin) and/or infections (mainly viral) (50%). Atopy was not overrepresented (20%), although a possible link between atopy and recurrent urticaria was noted. After a follow‐up of 2 to 7.5 years, 96% of patients were symptom free. Thus, our results indicate that clinical and etiologic features of urticaria in infants are somewhat different from those of adults and childre

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00663.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:To determine the occurrence of hereditary bullous epidermolyses (EB) in Croatia, Yugoslavia, from 1960 to 1987, cases were gathered from the hospital files of dermatologic and pediatric clinics and departments throughout the area. The diagnosis of EB type was made on the basis of clinical features, patients' histories, and light microscopy and electron microscopy findings. Fifty families with 58 patients were registered; 44 patients were examined personally by one of the authors. The most frequent type of EB in Croatia was recessive dystrophic EB Hallopeau‐Siemens, occurring in 35 of the 58 individuals. Regional accumulation of cases within the Varaždin area was noted (13 patients). Prevalence of EB in Croatia is 0.956 cases per 100,000 inhabitants. One case of recessive dystrophic EB Hallopeau‐Siemens occurred in about every 52,000 live bi

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00664.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:Thirty‐five children affected by a recurrent summer and spring papular eruption on the elbows, knees, and backs of the hands were examined retrospectively in order to evaluate the frequency of atopy. It was found that 45.7% were suffering from atopic diseases (atopic eczema, allergic rhinitis, asthma). This high proportion confirms the fact that an underlying atopy may predispose to the occurrence of this dermatosi

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00665.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:A 4‐year‐old child with dystrophic epidermolysis bullosa inverse is described. Clinical features were blistering of the skin, erosions, scarring and milia formation. The areas involved included the trunk, with preference for the axillary and inguinal folds, the neck and sacral area, and proximal extremities. Notably, the hands and feet were completely spared, with only mild nail dystrophy. Ultrastructural analysis revealed dermolytic blistering and absent or rudimentary anchoring fibrils. Collagen VII, the main structural protein of these fibrils, was present in the skin, as shown by indirect immunofluorescence. These findings suggest that a mutation that prevents appropriate supramolecular aggregation of collagen VII into anchoring fibrils may underlie this subtype of dystrophic epidermolysis bullosa in some patie

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00666.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:Plaques of leukemia cutis were the first manifestation of juvenile chronic myelogenous leukemia in a 2‐year‐old. This is a rare and usually fatal form of childhood leukemia. Only three cases of leukemia cutis associated with juvenile chronic myelogenous leukemia have been publis

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00667.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:Rapp‐Hodgkin syndrome is an uncommon, autosomai dominant condition characterized by distinctive craniofacial anomalies, cleft lip or palate, poor dentition, poor hair growth, dystrophic nails, and hypohidrosis, and hypospadias in boys. Since the originai report in 1966, fewer than 20 other patients have been described. We report a new sporadic case, a 12‐year‐oid boy who had erythrodermia and scaling skin at birth, and later developed most of the symptoms and findings previously desc

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00668.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstract:We report a 15‐month‐old boy with xeroderma pigmentosum, a history of repeated infections, and immune deficiency who developed a fatal pneumonia with parainfluenza type 1. Immunologic evaluation revealed a severe combined immunodeficiency with hypoglobulinemia, C3 deficiency, anergic response to skin testing, and an abnormal lymphocytic response to mitogens. We suggest that patients with xeroderma pigmentosum be evaluated carefully for immune deficiencies, should repeated infections oc

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb00669.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY