摘要:

Abstract:Most patients with X‐linked dominant chondrodysplasia punctata (X‐DCDP) have whorled, thick, or spiky adherent hyperkeratosis on their whole body. Histopathologically, there are large keratotlc plugs of hair follicles where calcium deposits. This is the characteristic finding in this disease. We performed histopathologic examination of skin specimens from two newborn babies with X‐DCDP. The desquamated thick scales from the hyperkeratotic lesions had calcium in the center of the keratotic plugs. Histopathologic examination of thick scales is easy and helpful in diagnosing X

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01176.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:A 3‐month‐old boy had reticulated achromic and atrophic skin lesions on the right buttock and thigh, as well as partial syndactyly of the third and fourth toes of the right foot. With hematoxylin and eosin stain, an epidermal defect, abnormally located fat cell lobules, and absence of the upper and midportions of the dermis were observed, conforming to a typical histopathologic picture of focal dermal hypoplasia. Electron microscopic examination of the atrophic site showed loose collagen bundles, collagen fibers with loss of regular bands, abnormal fibroblasts, and disruption of the basement membrane zone. These defects in the basement membrane zone strongly suggest that abnormal formation of type IV collagen is associated with focal dermal hypoplasia, and that this abnormal formation of collagen is correlated with the clinical sign of skin atro

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01177.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:The frequency and significance of minor clinical features for the diagnosis of atopic dermatitis (AD) were evaluated in different age‐related subgroups of 100 children (up to 12 yrs) with AD and an equal number of controls. Additional features studied were infraauricular fissuring, diffuse scaling of the scalp, and Hertoghe sign. Features found to be nonspecific were ichthyosis, nipple eczema, cheilitis, keratoconus, anterior subcapsular cataract, pityriasis alba, anterior neck folds, and food intolerance. Other minor features were of variable significance in different age groups. Seasonal variation of AD was observed in 44% of patients, with worsening during winter in 29% and in summer in 15%. Of the additional features, diffuse scaling of the scalp and infraauricular fissuring were significant, but Hertoghe sign was not. It was therefore concluded that minor clinical features for the diagnosis of AD need modification, with deletion of some of the present features and addition of newer one

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01178.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:We recently made a double‐blind safety‐in‐use comparison of four different brands of baby wipes using a panel of 302 infants over a period of 10 weeks. For the first two weeks, only soap and water was used to clean the babies’ skin after each diaper change, and then for the eight‐week test phase each baby was allocated one or other of the products for normal home use. The wipes differed in cleansing lotion formulation (emollients, preservative, pH) and fibrous composition. There were no clinically detectable differences in the effects of the wipes in terms of erythema, frequency of rashes, edema, and desquamation, but we recorded significant changes in the pH of pubic and buttock skin inside the diaper area. In particular, the brand of wipes with the lowest pH (2.8) in the lotion reduced the mean skin pH from 5.6 to 5.0 (p<0.01), and those with a pH of 5.5 had no significant effect. Wipes of intermediate pH (3.7) gave a final skin pH of 5.4‐but the downward trend was not statistically significant. These data indicate that skin pH can be depressed by such topical application, although the trial lasted only a fraction of the total time wipes might be used on each infant. Further research is necessary to evaluate the implications of the

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01179.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:A number of craniofacial malformation syndromes are characterized by prominent abnormalities of the skin, hair, nails, and mucous membranes. Crouzon disease has been reported in association with severe acanthosis nigricans in 12 patients. We report a new case of this association in a 16‐year‐old woman with multiple melanocytic nevi. Melanocytic nevi have been already reported in the medical literature in association with several congenital syndromes, although, to our knowledge, not with Crouzon dise

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01180.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:A 9‐year‐old girl was diagnosed as having a linear sebaceous nevus syndrome (LSNS). The nevus sebaceus was located on the face, and the girl also had nevoid hypertrichosis on the neck, sensorineural deafness, partial anodontia, blocked tear ducts, labiopalatoschisis, and an area of micropolygyria in the left encephalic (cerebral) hemisphere. Electroencephalographic alterations were detected, but they were not accompanied by a history of seizures; furthermore, the child was not mentally retarded. This phenotypic pattern of LSNS is unusual for the rarity of associated abnormalit

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01181.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Metastatic Crohn's disease (MCD) is a rare extraintestinal manifestation of Crohn's disease characterized by the histologic finding of granulomatous dermatitis at a site noncontiguous to the gastrointestinal tract. An adolescent had MCD of the face that was initially mistaken for severe, treatment‐resistant acne. Histopathologic and microbiologic evaluation, combined with clinical examination and response to therapy, ultimately led to the correct diagnosis.Mycobacterium paratuberculosisis a suspected cause of Crohn's disease. Polymerase chain reaction was used to detect genomic DNA specific for the organism in biopsy specimens from the patient's cutaneous lesions. This study failed to demonstrateM. paratuberculosisin the specimen

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01182.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:We report a 4 year‐old boy affected with hypohidrotic ectodermal dysplasia in whom loose anagen hair syndrome (LAHS) was suspected clinically. The diagnosis was confirmed by examination of hair by optic and scanning electron microscopy. Loose anagen hairs have not been previously described in the ectodermal dysplasias. It is possible that our patient had real LAHS or an ectodermal dysplasia with loose anagen hair as an epiphenomeno

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01183.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:We report a patient with phakomatosis pigmentovascularis lib and numerous iris hamartomas. Phakomatosis pigmentovascularis lib is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01184.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Abstract:Phakomatosis pigmentovascularis is a rare cutaneous malformation characterized by a combination of melanocytic nevi and nevus flammeus. We report a 16‐year‐old boy with extensive and predominantly unilateral skin involvement combining features of speckled melanocytic nevi over a background of nevus flammeus. A brief review of the literature and classification of this phakomatosis is presen

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1996.tb01185.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY