摘要:

Abstract:Congenital dermatofibrosarcoma protuberans occurred in a 16‐year‐old girl. The lesion was a hard cutaneous plaque on the abdomen. It was present at birth and slowly enlarged during the patient's lifetime. Histologic examination was essential to establish the diagnosis and to differentiate it from other congenital fibrohistiocytic proliferati

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00011.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:A 16‐year‐old girl had a three‐year history of many cafe au lait spots and freckles. In a dermatomal distribution. The diagnosis of segmental neurofibromatosis (NF‐5) was made on the basis of the clinical features, distribution of the lesions, and absence of systemic involve‐ment. Only a few cases in the literature describe NF‐5 with only macular lesions. An accurate clinical examination is important to identity this unusual p

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00012.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:Annular erythema of Infancy is an uncommon, nonpruritic, figurate erythema that begins in the first year of life. Biopsy specimens reveal a perivascular and interstitial lymphocytic infiltrate with numerous eoslnophils. The cause of the disorder is unknown, but a hypersensitivity response to unrecognized antigens is suspected. The disorder is selflimited, but may last for many months. In our patient the eruption continued 19 months after its onset.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00013.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:The fibromatoses are a group of benign proliferations of fibrous tissue with clinical behavior ranging from that of truly malignant tumors to that of benign reactive fibrous proliferations. Some of the superficial fIbromatoses are fairly common, but the deep ones, also known as desmoid tumors or musculoaponeurotic flbromatoses, are rare idiopathic multicentric osteolysis is a rare skeletal disorder of childhood that causes progressive destruction of bones and renal failure. We recently saw a young girt with both extraabdominal musculoaponeurotic fibromatosis and idiopathic multicentric osteolysis. The classification, diagnosis, and treatment of the deep fibromatoses are reviewed, and the possible association between the minor bone changes occasionally reported In musculoaponeurotic fibromatosis and idiopathic multicentric osteolysis is discussed.

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00014.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:A 20‐year‐old woman and her 12‐year‐old brother had hypohidrotic ectodermal dysplasia, cleft lip and palate, midfacial hypoplasia with narrow nose from the nasal bridge to the tip, narrow dysplastic nails, and conical teeth and hypodontia, and hypospadias and hypoplastic uvula in the boy. The woman had major underdevelopment of intellectual capacity. The most important hair anomalies in both siblings were sparse eyebrows, pili torti, and pili canaliculi. Some of the pili canaliculi had two canals (pili bicanaliculi), and the cross section for scanning electron microscopy had a quadrangular aspect. This is the seventh family reported with Rapp‐Hodgkin ectodermal

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00015.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:A 38‐day‐old prematurely born Infant developed rapidly progressive facial cellulitis In association with ipsilateral submandibular lymphadenopathy and pulmonary consolidation. Group B streptococci (GBS) were isolated from blood, endotracheal, and lesion cultures. Prompt recognition of GBS cellulitis‐adenftis end Institution of parenteral, synergistic antibiotic therapy are impo

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00016.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:A 4‐month‐old boy had numerous nodular lesions. Microscopic findings revealed a histtocytic proliferation without foam cells and giant cells. Immunohistochemical studies showed that the histiocytes belonged to the monocyte‐macrophage system and not to the Langerhans cell system. This case is an unusual variant of juvenile xanthogranutoma that, when congenital, may be difficult to differentiate from HashimotoPrtizker di

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00017.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:An 18‐year‐old men has been followed in our department since age 10 years when he began to develop numerous yellow, papular lesions on the scalp, face, neck, trunk, and upper extremities. The lesions enlarged slowly to form yellow‐brown nodules measuring up to 6 cm. During this period five of the nodules and two small papules were excised. Histopathology was consistent with juvenlle xanthograuloma (JXG). Recently, the patient complained of significant loss of memory, and a computed tomography scan was performed showing several cerebral and cerebellar lesions. The cutaneous lesions In our patient were almost identical to those described elsewhere as being characteristic of progressive nodular histiocytoma. The many clinical and histopathologic similarities between lesions of progressive nodular histiocytoma and JXG suggest that they may represent a continuum rather than two distinct disease processes. Given the fact that there are no histopathologic differences with JXG, as well as the wide range of clinical lesions that JXG may adopt, there Is no reason to separate the entities. Although JXG lesions are usually believed to be benign and self‐healing, targe nodular forms can be associated with visceral lesions. Only four previous cases of cutaneous JXG with central nervous system involvement were found in a review of the lit

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00018.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:An infant had a 3 × 1.5‐cm congenital, slow‐growing, lobulated tumor below the right inferior eyelid. The mass transilluminated, and needle aspiration yielded tears. Dacryocystography showed a large cystic area connected with the lower lacrimal canailcuium. Histoiogicaily, the tumor proved to be a dermoid cyst. This association has not been reported previoualy. Surgical excision and suture of the pedicuie resulted in permanent

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00019.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Abstract:We cared for an Infant girl with the clinical constellation of supraumbllical midabdomlnal raphe, sternal atresia, and cutaneous facial and upper trunk hemangioma. This is the first report of this clinical association in the dermatologic literature. The vascular component of the disorder responded to fiashlamp‐pumped pulsed dye laser therapy and to systemic Interferon alfa‐2a (Roferon

ISSN:0736-8046    

DOI:10.1111/j.1525-1470.1993.tb00020.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY