Lymphomatoid papulosis in children.

BACKGROUND: Although lymphomatoid papulosis is well described in adults, the clinical course, prognosis, risk for lymphoma, and recommendations for follow-up have not been established in children. OBJECTIVE: Our aim was to analyze our data on six children with lymphomatoid papulosis and to analyze available information on reported cases from the literature to characterize better lymphomatoid papulosis in childhood and to compare it with adult-onset lymphomatoid papulosis. METHODS: Clinical records, laboratory studies, and histopathologic evaluation of skin biopsy specimens from six children with lymphomatoid papulosis were reviewed. A literature search was also performed and disclosed detailed information on 17 childhood cases. RESULTS: In most cases childhood lymphomatoid papulosis is clinically and histologically similar to lymphomatoid papulosis in adults, but three unusual patterns were identified in our children: first, after initial outbreak, dwindling outbreaks (both in frequency and number of lesions) until the eruption ceased completely; second, lymphomatoid papulosis localized to one area for years before generalizing, and third, presentation of lymphomatoid papulosis with hundreds of lesions. In our children and in those previously reported, response to systemic antibiotics and potent topical steroids was variable, as in adults. All our children to date have remained healthy; the longest period of follow-up is 9 years. However, in previously reported cases two patients with childhood-onset lymphomatoid papulosis had lymphoma as adults. CONCLUSION: Childhood lymphomatoid papulosis may be more likely to resolve spontaneously than adult lymphomatoid papulosis; nevertheless these children may still be at risk for lymphoma and thus need lifelong follow-up.

Odonto-onycho-dermal dysplasia.

Odonto-onycho-dermal dysplasia is an ectodermal dysplasia (ED) described once previously in two families who exhibited atrophic malar patches, sparse hair, conical teeth, dystrophic nails and hyperkeratosis of the palms and soles. We describe a boy who developed a blistering malar rash soon after birth. When examined at 11 months, and then at 27 months of age, he had persistent atrophic malar plaques that reddened with heat. He also showed nail dystrophy, sparse hair, lingual concavity of the incisors, a bifid maxillary incisor, a five-cusped molar, and hyperhidrosis of the palms and soles. In addition he had chronic tearing, photophobia, blepharitis, and a mild keratitis. After reviewing EDs with atrophic or scar-like skin changes, we believe this child most resembles the patients with odonto-onycho-dermal dysplasia, although his eye findings are unique.

Hematologic derangements of cardiopulmonary bypass: a comparison of two perfusion systems.

A major goal of new perfusion equipment is minimal trauma to blood elements. This study compares two perfusion systems, quantifies the change in blood components and generation of microemboli, as well as compares the hospital courses of each perfusion system. Forty-four coronary patients were assigned to either Group S, a silicone rubber membrane (SciMed) and centrifugal pump (Bio-Medicus) (N=19) or Group C (our routine equipment), a microporous polypropylene membrane (COBE CML) and roller pump (COBE) (N=25). The rise in plasma hemoglobin (26+/-14mg* in Group S and 26+/-17mg* in Group C), the drop in hematocrit (-15.0+/-3.9* in Group S and -14.7+/-3.8* in Group C at the second post-op day), and the decrease in platelet count (-152,000+/-78,000* in Group S and -129,000+/-52,000* in Group C) were similar in both groups. There was no difference in rise in post-op alveolar-arterial oxygen gradients or debris generated by each system. 27.7% in Group S required red cell transfusions and only 8% required red cell transfusions in Group C. There was no significant difference in clinical endpoints such as ICU stay, hospital stay and complication rate. We found no advantage to more expensive perfusion devices and no improvement upon the extensive CPB damage to formed blood elements. * p less than .001