摘要:

AbstractAn 8–year–old boy with reticular erythematous mucinosis syndrome had erythematous plaques on his chest, face, and arms for three years. Sun exposure resulted in pruritus and increased lesions. Histologic examination revealed a perivascuiar mononuciear celi infiltrate with hematoxylln and eosin staining, positive staining materiai between the dermal coilagen bundles with alcian blue (pH 2.5) staining, and granular basement membrane deposits of IgM with direct immunofluorescence staining. Results of all lupus erythematosus serologies and porphyrin studies were negative. Minimai erythema dose determinations to ultraviolet A and B were normai, and the lesions could not be induced with high doses of irradiation. Topical sunscreens, corticosteroid cream, and systemic beta–carotene produced no therapeutic be

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01065.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractCat–scratch disease (CSD) is a common cause of chronic lymphadenopathy (especially regional) that primarily affects children and adolescents. The clinical diagnosis of CSD is based on the presence of three of four criteria, which may inciude a positive CSD skin test. Usuaily a benign, self–limiting disease, CSD may sometimes have atypical manifestations and serious complications, particuiarly in tmmunocompromised hosts. Cat–scratch disease is now known to be caused by a smail, gram–negative, pleomorphic bacterium. Antibiotics are dramatically effective against CSD in immunocompromised patients, and are of proven benefit in typical cases. Most patients recover with only symptomatic treatment. This article reviews the history of CSD research, oiinicai features of typical and atypical CSD, and current topics of interest in CSD research, especiaily in the areas of diagnosis and tr

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01066.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractOne hundred and twelve cases of chitdhood psoriasis, constituting 5% of all psoriasis patients registered in our ciinic, were studied over a period of nine years (January 1980 to December 1988). There were 57 male and 55 female patients. Age of onset ranged from 1 month to 13 years, with a mean and median age of 7 years. A positive famiiy history of psoriasis was elicited from 9.8% of patients. The commonest form of psoriasis found was plaque (89.6%), followed by guttate (25.9%). Infection was the most common triggering factor, occurring in 15.2% of cases. Many of these findings are at variance with observations reported elsewhere for this age group.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01067.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractTwenty–six children with a symmetric papular or papulovesicular acrolocated eruption were studied to evaluate a possible link with viral infection. A history of a recent immunization was present in four patients, two of whom had a polio vaccine virus isolated from their stool samples. There was serologic evidence of a recent Epstein–Barr virus infection in one patient, adenovirus infection in another, a concurrent Epstein–Barr virus and adenovirus Infection in a third, and cytomegalovirus infection in a fourth. Viral cultures were positive in two patients for adenovirus and in two others for rotavirus. Although some of the virologlc findings could have been coincidentai, this study confirms that multiple virai infections can be reiated to most papular and papulovesicular acrolocated eruptions of chiidhood. Virologic investigations in the eariier phases of the eruption are recommended to identify the etiologic factors of these le

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01068.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractThe hair of patients with three ectodermal dysplasias–ectrodactyly ectodermal dysplasia clefting syndrome (EEC); orofacial–digital syndrome (OFD) type I; and anhidrotic ectodermal dysplasia syndrome (AED)– were studied by scanning electron microscopy. While no pathognomonic abnormalities were noted for each condition, hair shaft structural defects were evident in all patients studied. The EEC clefting syndrome and OFD I shared the most deforming defects, while AED had

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01069.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractTwelve cases of pachyonychia congenita were reviewed. The mode of inheritance was autosomal dominant The ciinical features of these patients included thickened nails, hyperiteratosis of the palms and soles, thinning of hair or alopecia, painful bullae or ulcerations of the palms and soles, leukokeratosis oris, verrucous lesions of the extremities, hyperhidrosis, premature eruption of teeth, paronychial infections, epidermal cysts with miiia, and corneai dyskeratosis at times associated with cataracts. Biopsy from the plantar lesions usually revealed marked hyperkeratosis, acanthosis, moderate hypergranulosis, and minimal dermal infiammatory infiltration. Treatment with keratolytic agents and lubricants is indicated to areas of palmar and plantar hyperkeratosis but usually produces only transient benefit. Squamous cell carcinoma developed in one of the patients over the site of chronic plantar ulcerations. Areas of chronic bullous formation or ulceration should be observed for possible skin malignancy.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01070.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Abstractidiopathic atrophy of the naiis has been described as an acquired condition of childhood wherein the changes may iead to permanent ioss with scarring of the nait bed. Three patients with idiopathic atrophy are described here, two of whom are siblings, ages 9 and 11 years. The authors suggest that ail family members of patients with this condition be examined for possible involvement.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01071.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractLymphomatoid papulosis (LyP) is rarely seen in children. We report a case of LyP in a 4‐year‐old boy in whom immunopathologic studies demonstrated T cell antigen deletions. In contrast to all but two previous reports, a T suppressor (CD‐8) phenotype was predominant. Southern blot analysis of DNA isolated from a typical skin lesion indicated a clonal rearrangement of the T cell receptor β gene. Because of a 10% frequency of malignant lymphomas in patients with LyP, long‐term observation is cruciai, especially in chiidren. We recommend routine clonal rearrangement studies for aid in diagnosis and follow‐up, and as possible prognostic indicators in children with this

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01072.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractBannayan‐Zonana syndrome is an autosomal dominant condition that has not been weii described in the dermatoiogy literature. The typical case is characterized by macrocephaly, multipie angiomas, and muitiple encapsulated or infiltrating iipomas. As in other autosomai dominant hamartoneopiastic syndromes, the degree of expression within one family frequently varies wideiy. Our patient had macrocephaly and angiomas as well as iipomas with peculiar histoiogic features simiiar to lymphangiomyomas. Her father had a iarge nevus flameus on his leg, and Iipomas with normal histoiogic appearance. The paternai grandfather had multiple encapsulated Iipomas with normal histoiogic appearance. Neither father nor grandfather had macrocephal

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01073.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

AbstractWe describe a patient with the keratitis, ichthyosis, and deafness (KID) syndrome who also had a generalized cytomegalovirus infection. Patients with the KID syndrome are susceptible to not only bacterial and fungal infections, but also to viral infections.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1990.tb01074.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY