Biomedical subjects
Publications and source records attributed to A A Hebert.
Symmetric areas of erythema and induration developed on the cheeks of a 17-day-old black girl after ice packs were applied to her face for treatment of supraventricular tachycardia. To our knowledge, this is the first report of cold panniculitis caused by treatment of a cardiac arrhythmia with ice packs.
Neonatal lupus erythematosus (NLE) is an antibody-mediated disorder most commonly associated with autoantibodies to Ro and/or La antigens. There have been five previous reports describing eight NLE patients with anti-U1RNP antibody in the absence of anti-Ro and anti-La autoantibodies. We report two cases of anti-U1RNP antibody-positive NLE, and briefly review the five previous reports. The diagnosis of NLE was based on physical examination and serological studies by ELISA, immunodiffusion, and immunoblotting. By conventional immunodiffusion and ELISA, our cases were negative for anti-Ro and anti-La antibodies, and positive for anti-U1RNP antibody. However, one of the mothers had anti-La antibody detected by immunoblot assay only. All anti-U1RNP antibody-positive infants had classic cutaneous lesions of NLE, but it is of interest that none had congenital heart block (CHB). Although these infants were negative for anti-Ro and anti-La antibodies with immunodiffusion and ELISA techniques, these antibodies might be detectable by immunoblotting, as was the case in one of the mothers.
BACKGROUND: Pemphigus refers to a group of autoimmune blistering diseases of the skin. Of the two major types of pemphigus, pemphigus vulgaris and pemphigus foliaceus, only pemphigus vulgaris has been known to affect newborn infants via passive transfer of maternal IgG antibodies across the placenta. Although pemphigus foliaceus antibodies have also been shown to cross the placenta, never before has a newborn been clinically affected. We report the first of neonatal pemphigus foliaceus confirmed by both clinical presentation and immunofluorescence studies. OBSERVATIONS: The distinguishing factors in this case were high antibody titers by indirect immunofluorescence present in both the mother and her fetus (1:640 and 1:80, respectively). CONCLUSIONS: A threshold of fetal antibody titer ( > 1:40) may need to be surpassed before neonatal disease can occur in pemphigus foliaceus. The likelihood of reaching this threshold has been shown to be increased with higher maternal antibody titers. Thus, strict control of maternal pemphigus foliaceus should lower the incidence of placental antibody transfer and improve neonatal outcome.
Peutz-Jeghers syndrome is inherited as an autosomal dominant trait with variable incomplete penetrance. Patients with Peutz-Jeghers syndrome characteristically have hamartomatous polyps throughout their entire gastrointestinal tract, particularly in the small bowel, and mucocutaneous hyperpigmentation involving the lips, oral cavity, and skin. Although the intestinal hamartomatous polyps have a lower incidence of malignant change in the gastrointestinal tract than do adenomatous polyps, recent information suggests that the overall neoplastic transformation from Peutz-Jeghers syndrome is not a rare event.
OBJECTIVE: To catalog the paraspinal skin lesions of early childhood that are associated with occult spinal dysraphism. RESEARCH DESIGN: Retrospective review of a series of patients. SETTING: Tertiary care referral center. PATIENTS: Fifteen patients who had significant paraspinal skin lesions were identified from the personal files of the authors who saw them. RESULTS: The skin lesions included various combinations of hyperpigmentation, hypopigmentation, hypertrichosis, acrochordons, dimples, lipomas, hemangiomas, or teratomas. Not all lesions were evaluated with the same tests, which included plain roentgenography, ultrasonography, myelography, computed tomography, and magnetic resonance imaging. Of the 15 patients, six had spinal anomalies, eight had no apparent spinal dysraphism, and one had insufficient results of the evaluation to assess the spinal column. CONCLUSIONS: Early recognition of paraspinal skin lesions is essential to prevent neurologic damage. Urinary or fecal incontinence, recurrent urinary infections, muscle atrophy, foot deformities, weakness, pain, or decreased sensation in the lower extremities may eventually develop in these patients. Magnetic resonance imaging appears to be the single best screening test for dysraphism.
Osteochondromas are among the most common cartilaginous neoplasms, but are rarely seen on the fingers and toes. We report a 10-year-old girl who had a dystrophic fingernail since infancy that was identified as a subungual osteochondroma radiologically and histopathologically. The clinical, radiographic, and histopathologic features, as well as the pertinent differential diagnoses and treatment options are discussed.
This article discusses many of the more common diagnoses that should be considered when managing a child with nonresponsive excema. Some of these possible diagnoses are infectious and parasitic disorders, psoriasis, heritable disorders, Langerhans cell histiocytosis, and syndromes with altered immunity and eczematous skin changes. This overview of diagnostic and laboratory evaluations of the child with eczema who fails standard therapy is presented as a useful guide in evaluating these patients.
A prospective, randomized, single (investigator) blind multicenter study was performed to compare the safety and efficacy of clarithromycin and cefadroxil oral suspensions in the treatment of mild to moderate skin and skin structure infections in children. Male and female patients ages 6 months to 12 years were enrolled at 24 study centers in the United States. Patients had signs and symptoms consistent with mild to moderate skin or skin structure infections judged suitable for oral antimicrobial therapy. Clarithromycin oral suspension was given to 118 children in a dose of 7.5 mg/kg (maximum of 500 mg) twice daily; cefadroxil oral suspension was given to 113 children in a dose of 15 mg/kg (maximum of 1000 mg) twice daily. Among clinically evaluable patients clinical success rates (cure plus improvement) were 96% (71 of 74) for clarithromycin and 98% (83 of 85) for cefadroxil (P = 0.664). Bacteriologic cure rates in evaluable clarithromycin and cefadroxil patients were 96% (72 of 75) and 99% (89 of 90), respectively (P = 0.331). Pathogen eradication rates based on 204 evaluable pathogens were 97% in the clarithromycin group and 99% in the cefadroxil group (P = 0.326). Adverse events were mild or moderate and were reported in 25% of clarithromycin and 35% of cefadroxil patients (P = 0.085). In both groups adverse events involved primarily the digestive tract. No significant laboratory changes were noted. Clarithromycin oral suspension appears to be a safe and effective alternative to cefadroxil for the treatment of pediatric skin and skin structure infections.
Two sisters of Mexican ancestry had focal epithelial hyperplasia (FEH). The lesions on the oral mucosa of the older child were initially misinterpreted as representing sexual abuse. Microscopic evaluation of a hematoxylin and eosin-stained section from a lower lip papule demonstrated the histologic features of FEH. Although human papillomavirus (HPV) type 13 and HPV32 have been most consistently present in FEH lesions, types 6, 11, 13, and 32 were not detected in the paraffin-embedded tissue specimen of our patient using an in situ hybridization technique. The lesions persisted or recurred during management using destructive modalities; subsequently, they completely resolved spontaneously.
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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.
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.