摘要:

Abstract:The typical appearance of cutaneous hemangiomas of infancy is well known. We studied unusual manifestations of this common tumor. We reviewed over 500 hemangiomas in the registry of the Vascular Anomalies Program at Boston Children's Hospital. We found four uncommon morphologic variations: deep hemangiomas with normal overlying skin (n = 12); macular hemangiomas with a port‐wine stainlike appearance (n = 6); bossed hemangiomas with telangiectasia and peripheral patlor (n = 5); and hemangiomas with persistent fast flow (n = 3). Deep and superficial (macular) varieties regressed at a normal rate. Telangiectatic (bossed) hemangiomas, however, invotuted rapidly, usualiy before 1 year of age. Hemangiomas with persistent fast flow required either resection or sclerotherapy for complications in early childhoo

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00114.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:We present a case of r4etherton disease, where the hairs lacked the characteristic microscopic feature of Uichorrhexis invaginata. In its piace were certain hairs with a goif tee tnorphoiogy. These represent the proximal half of the invaginate node seen in typicai Netherton disease. Scanning eiectron microscopy demonstrated the three‐dimensiona) quality of this abnormaiity, which is subtie when assessed by iight microscopy aione. We describe this sign so that it might be recognized when seen in isolation, as here, and aiiow tire diagnosis of Netherton disease to be confirme

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00115.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:A trend toward increasing resistance of Staphylococcus aureus to Standard antibiotic therapy has been reported. Specimens were taken from 98 patients in our outpatient staff clinic who had clinical signs of superficial skin infections. Patients with erythromycin‐resistant 5. aureus were contacted by telephone or seen in clinic. The organism was found in 87% of patients. Twenty‐two (26%) of the 85 cultures that grew 5. aureus were resistant to erythromycin. Treatment failure occurred in one of these patients. We conclude that S. aureus is the most common causative organism in secondary skin infections and impetigo in our patient popuiation. Despite significant erythromycin resistance, there was a low frequency of treatment faiture in this group, Erythromycin may still be a reasonable agent in the treatment of uncomplicated superficial skin infections in our community at this t

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00116.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:We present the ninth case of epidermolysis bullosa acquisita (EBA) reported in children. As in most of the other childhood cases, the 7‐year‐old boy described herein had an acute, widespread, inflammatory vesiculobuilous eruption with oral involvement. Indirect immunofluorescence on salt‐split skin as well as Western imrnunobtot confirmed the diagnosis of EBA. The patient responded to combined prednisone and dap‐sone, and was maintained with dapson

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00117.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:A 5‐month‐old boy with erythrokeratoderma variabilis is presented. The parents noted that the asymptomatic erythematous plaques changed over the course of hours to days, Topical therapy with retinoic acid proved ineffectlve. A brief review of the literature is presen

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00118.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:We report a patiënt affected by the rare branchio‐oculo‐facial syndrome. Histologic study of the branchial clefts showed thymic remnants. A scanning electron microscopic study of the hair was also performed. The clinical features of the patiënt were compared with those of other patients reported in the liter

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00119.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:An infant suffered from psoriasiform dermatitis complicated by severe constitutional symptoms. During his 12 months of hospitalization, the symptoms exacerbated periodically despite numerous therapeutic trials. Histologie findings revealed lichenoid psoriasiform dermatitis with striking eosinophilic necrosis of epidermal cells and satellite cell necrosis. Immunohisfochemically, CD1 + Langerhans cells had almost disappeared, and CD8+ cytotoxic‐suppressor T cells were predominant over CD4+ helper‐inducer T cells in the epidermis. These findings in the skin biopsy specimens suggested some similarity to graft‐versus‐host disease but no known cause of that disorder was proved. Finally, methotrexate was effective. The patient became afebrile, and his skin lesions Improved, leaving almost no scarring. This patient seems to have had specific clinical features that do not correspond with any of the known dermatitites. Clinical, histoiogic, and laboratory findings did not uncover any etiologic

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00120.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:Eosinophilic pannicuiitis is a pooriy defined entity with variabie ciinicai features. We report a case of rapidiy eniarging, asymptomatic subcutaneous scalp nodules in a 6‐year‐old biack boy with atopic dermatitis. The noduies resolved spontaneously over two to three days. Biopsy specimens were remarkable for eosinophilic pannicuiitis without evidence of epidermal change or vasculltis. We beiieve that this is the youngest reported patient with this disor

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00121.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:A 9‐month‐old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approxitnately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then maiitly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00122.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Abstract:We present two patients with subcutaneous fat necroses (SCFN) in whom endocrinologie studies revealed an association with elevated prostaglandin E (PGE) levels. A boy born after prolonged labor complicated by meconium aspiration developed erythematous, indurated plaques over the back, arrns, buttocks, and cheeks at 4 days of age. A biopsy specimen of involved skin showed panniculitis with foei of necrotic adipocytes containing radially arranged, needle‐shaped clefts and a granulomatous infiltrate in the septae. Laboratory studies revealed hypercalcemia of 13.6 mg/dl (normal 8.8–10,1 mg/dl), eievated 1,251,25(OH)2D3, and increased urinary excretion of PGE2. The child was hospitalized and treated with systemic steroids and diuretics, with resolution of SCFN and hypercalcemia. The second patient was a girl born with cyanotic heart disease. A diagnosis of Ebstein anomaly was made, and intravenous PGE, was started to keep patent the ductus arteriosus. Four days later erythematous, indurated plaques were noted on the knee, back, and anterior chest. A skin biopsy specimen revealed SCFN. There was no associated laboratory abnormality. On discontinuing PGE, no new tesions formed and the existing panniculltis resolved. These two cases demonstrate the association between SCFN and eievated PGE levels (endogenous in patiënt 1, exogenous in patiënt 2). No previous reports of SCFN after the administration of PGE, have appeared in the liter

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1995.tb00123.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY