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Biomedical subjects

A A Hebert

Publications and source records attributed to A A Hebert.

At least 55 records · Page 3Linked to original sources
PubMed1989

Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children.

A retrospective analysis of 21 consecutive patients hospitalized with either Stevens-Johnson syndrome or toxic epidermal necrolysis was carried out to assess morbidity and mortality rates and to establish the value of a specific management practice. Fourteen children with Stevens-Johnson syndrome and seven with toxic epidermal necrolysis were cared for at the Children's Memorial Hospital, Chicago, between 1978 and 1988. All were managed in a well-staffed medical ward or, when necessary, in the pediatric intensive care unit. Supportive measures included reverse barrier isolation, intravenous fluids and nutritional support, meticulous skin care, early detection and treatment of infection, and daily ophthalmologic examination. No patient was treated with systemic steroids. The mortality rate was zero. Eye complications, consisting of dry eyes or mild chronic symblepharon, were the most significant long-term sequelae.

Adolescent↗
PubMed1989

Gianotti-Crosti syndrome associated with Epstein-Barr virus infection.

Gianotti-Crosti syndrome, a distinctive eruption occurring after hepatitis B infection, is characterized by symmetric, nonpruritic lichenoid papules usually localized to the face, limbs, and buttocks. In North America, hepatitis B antigenemia is rarely associated with Gianotti-Crosti syndrome in infants. Recent reports indicate there are a variety of infectious agents associated with Gianotti-Crosti syndrome. We report a case of an 11-month-old white female infant with Gianotti-Crosti syndrome and concurrent primary Epstein-Barr virus infection without evidence of hepatitis B infection.

Acrodermatitis↗
PubMed1989

Dermal hematopoiesis in neonates: report of five cases.

We report five cases of dermal hematopoiesis in newborn infants. Dermal hematopoiesis, manifested clinically as a blueberry muffin eruption, has been associated with both intrauterine viral infections and congenital hematologic dyscrasias. Of our five patients, four of whom were boys, the cutaneous findings could be attributed to congenital infections in two instances. The infectious agents were cytomegalovirus and coxsackie virus B-2. Two infants had hematologic abnormalities, including twin transfusion syndrome and ABO blood group incompatibility. In our fifth patient a cause was not identified.

Coxsackievirus Infections↗
PubMed1989

Kindler syndrome: report of two cases and review of the literature.

We evaluated two patients with hereditary bullous poikiloderma. Both had acral bullae, generalized poikiloderma with prominent atrophy, and acral keratoses. One patient, with sporadic disease, had, in addition, urethral and subglottic stenoses, webbing of digits, and poor dentition. The other patient, whose disease was dominantly inherited, had koilonychia. The results of cutaneous histopathology, electron microscopy, and immunofluorescence mapping studies are presented. It is possible that Kindler syndrome and Weary's hereditary acrokeratotic poikiloderma are variants of the same disease.

Age Factors↗
PubMed1989

Bullous amyloidosis.

Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.

Amyloidosis↗
PubMed1988

Treating tinea capitis: should ketoconazole replace griseofulvin?

We undertook a randomized double-blind comparison of griseofulvin and ketoconazole for the treatment of tinea capitis. The outcome was based on fungal culture results and changes in clinical signs and symptoms determined by an assessment system that yielded a severity score. Patients were evaluated at 4-week intervals for 12 weeks. Seventy-nine patients were enrolled; 46 received griseofulvin, and 33 received ketoconazole. Forty-eight patients (26 griseofulvin, 22 ketoconazole) were considered evaluable. Pathogenic fungi were isolated from 78% of the enrolled and 87% of the evaluable patients. Trichophyton tonsurans was the predominant dermatophyte isolated. Potassium hydroxide preparation correlated poorly with culture results (sensitivity 59%). Of 22 evaluable ketoconazole patients, 16 (73%) were considered to have been treated successfully; 25 of 26 (96%) patients who received griseofulvin were successfully treated (chi-square = 3.54, p less than 0.10). The proportion of culture-positive patients at each follow-up visit appeared somewhat greater for the ketoconazole-treated group than for the griseofulvin-treated group, but the differences were not statistically significant. Analysis of severity scores revealed no significant differences between the groups (t test and Mann-Whitney U test). No significant hepatotoxicity or other adverse reactions were observed. We conclude that griseofulvin should remain the drug of choice for treating tinea capitis.

Adolescent↗
PubMed1987

Uncombable hair (pili trianguli et canaliculi): evidence for dominant inheritance with complete penetrance based on scanning electron microscopy.

Uncombable or spun-glass hair (pili trianguli et canaliculi) is an uncommon condition in which the hair is "unmanageable" and has a distinct appearance on scanning electron microscopy. The hair is usually grossly abnormal in infancy and childhood, but may become normal later in life. Although dominant inheritance has been observed, most cases have been sporadic. Both recessive and dominant transmission with incomplete penetrance have been suggested as modes of inheritance. We report the occurrence of this condition in a young girl, her brother, and her father. Although the proposita and her brother had characteristically uncombable hair, their father appeared normal and denied any history of hair abnormality. However, the characteristic hair morphology was observed on scanning electron microscopy in all 3 relatives, documenting dominant transmission and complete penetrance of the gene in this family.

Child, Preschool↗
PubMed1987

Scarring alopecia.

The scarring alopecias are a diverse group of diseases characterized by the combination of follicular destruction and dermal scarring. In this article we divide scarring alopecias into three broad categories, pediatric diseases, perifollicular lymphocytic diseases, and folliculopustular diseases, and discuss selected entities from each category.

Adult↗
PubMed1987

The CHILD syndrome. Histologic and ultrastructural studies.

A 4 1/2-month-old female infant had cutaneous and musculoskeletal changes consistent with the diagnosis of congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome. This syndrome encompasses abnormalities of several organ systems, and its origins and pathogenesis are poorly understood. This report describes an additional patient with this uncommon syndrome and includes light and electron microscopic studies of her skin lesions. Our patient's clinical features of meningocele, Shone's syndrome, and the electron microscopic findings have not previously been described in the American literature (to our knowledge) in association with this syndrome.

Abnormalities, Multiple↗
PubMed1986

Knuckle pads in children.

Knuckle pads occur from benign thickening of the skin, principally the epidermis, and are occasionally seen in children. Despite the name, knuckle pads most commonly overlie the proximal interphalangeal joints. They must be differentiated from a variety of other cutaneous abnormalities. There is no consistently effective treatment, but knuckle pads have been reported to resolve spontaneously in children.

Child↗
PubMed1986

Annular erythema of infancy.

An urticarial annular erythema began in our patient at 6 weeks of age and resolved completely by 14 months of age. Individual lesions lasted 2 to several days and tended to erupt in cycles, with each cycle lasting 5 to 6 weeks. The lesions were totally asymptomatic and left no residual scaling or hyperpigmentation. Histologically, a dense perivascular infiltrate of lymphocytes, histiocytes, eosinophils, and a few plasma cells was evident. The clinical appearance, course, and histologic characteristics resemble closely the case reported by Peterson and Jarratt in 1981.

Biopsy↗
PubMed1986

Nevoid basal cell carcinoma syndrome: bilateral ovarian fibromas in a 3 1/2-year-old girl.

A 3 1/2-year-old black girl with bilateral ovarian fibromas was subsequently diagnosed as having nevoid basal cell carcinoma syndrome at the age of 4 years, when basal cell tumors and pits of the hands and feet were noted on examination. This case is unusual because of the development of bilateral ovarian fibromas at an extremely early age. Prepubertal patients with this ovarian tumor should be thoroughly investigated for the possibility of nevoid basal cell carcinoma syndrome.

Basal Cell Nevus Syndrome↗
PubMed1986

Pityriasis lichenoides in children: therapeutic response to erythromycin.

Fifteen of twenty-two children with pityriasis lichenoides were treated with oral erythromycin. Eleven (73%) had a remission, usually within 2 months. Two others showed partial improvement, and two were unimproved. Seven of the children who experienced a remission were off erythromycin and free of lesions after 2 to 5 months of therapy. A trial of erythromycin as described herein should be considered in children with pityriasis lichenoides before other, possibly more toxic, measures are instituted.

Adolescent↗
PubMed1986

Anterior abdominal wall angiosarcoma in a morbidly obese woman.

A review of the literature showed that angiosarcomas of the anterior abdominal wall are rare neoplasms and that all published cases have been preceded by irradiation and/or prior surgery. We believe that our case of a primary angiosarcoma of the anterior abdominal wall is unique and that chronic lymphedema secondary to morbid obesity may be of etiologic importance.

Abdominal Muscles↗