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Biomedical subjects

A A Hebert

Publications and source records attributed to A A Hebert.

At least 19 recordsLinked to original sources

Crusted scabies in a patient with chronic graft-versus-host disease.

We describe a 20-year-old man with chronic graft-versus-host disease and progressive cutaneous changes. His skin became more lichenified despite therapy with azathioprine, prednisone, and cyclosporine. Although it was initially thought that lichenoid graft-versus-host disease had developed, it was subsequently discovered that the patient had crusted (Norwegian) scabies.

Adult

The relationship between skin maturation and electrical skin impedance.

When performing electrophysiological testing, high electrical impedance values are sometimes found in neonates. Since excessive impedance can invalidate test results, a study was conducted to delineate the relationship between skin maturation and electrical skin impedance. This study investigated the skin impedance in 72 infants ranging from 196 to 640 days of age from conception. Regression analyses demonstrated a significant relationship between impedance and age, with the highest impedance centered around full-term gestation with values falling precipitously at time points on either side. Clinically, impedance values fall to normal levels at approximately four months following full-term gestation. Skin impedance values are low in premature infants, but rapidly increase as the age approaches that of full-term neonates. Low impedance values in premature infants are attributed to greater skin hydration which results from immature skin conditions such as 1) thinner epidermal layers particularly at the transitional and cornified layers; 2) more blood flow to the skin; and 3) higher percentage of water composition. These factors facilitate the diffusion of water vapor through the skin. As the physical barrier to skin water loss matures with gestational age, the skin impedance reaches a maximum value at full term neonatal age. After this peak, a statistically significant inverse relationship exists between electrical skin impedance and age in the first year of life. This drop in skin impedance is attributed to an increase in skin hydration as a result of the greater functional maturity of eccrine sweat glands.

Age Factors

Serum sickness-like reactions from cefaclor in children.

Cefaclor is an oral semisynthetic cephalosporin that is popular in the treatment of infections in children. This drug has been associated with a serum sickness--like reaction characterized by an urticarial eruption, pruritus, arthritis, and/or arthralgias. We report 12 additional cases of cefaclor-related serum sickness--like reactions in children. All patients improved after discontinuation of the drug and no long-term complications resulted.

Cefaclor

Massive labial edema in pregnancy.

We have presented two cases of massive labial edema in pregnant women. The differential diagnosis included infectious diseases, neoplasms, congenital anomalies, trauma, inflammatory conditions, and metabolic disorders. Appropriate tests in the evaluation of this condition were discussed.

Adolescent

Neurocristopathy syndrome: review of four cases.

Aberrant embryologic development of the neural crest may clinically result in one of several patterns of malformation. Four examples of the neurocristopathy syndrome are described that collectively feature a very broad range of expression in these infants. Because of the many expressions, the syndrome may not be recognized. We believe that greater familiarity with the disorder will aid in the discovery of unsuspected anomalies and provide clues about predisposing etiologies.

Abnormalities, Multiple

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

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

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

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

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