Search

Background: Discoid lupus erythematosus (DLE) is uncommon in childhood. Less than 2% of patients with DLE develop the disease before 10 years of age., Observations: We present eight cases of childhood DLE with onset before age 10 years: four black boys with cutaneous DLE, three black girls ages 7, 2, and 6 years at onset, who developed systemic lupus erythematosus at ages 12, 9, and 8 years, respectively, and a 10-year-old Hispanic boy who had a systemic flare at the age of 20 years., Conclusions: A review of the 16 published cases of childhood DLE reveals that it is similar to its adult counterpart in its presentation and chronic course. However, several important differences are noted: a lack of female predominance, a low incidence of photosensitivity, and frequent progression to systemic lupus erythematosus at an early age. A discussion of the management of DLE in children is also presented.

Background: Neonatal lupus erythematosus (NLE) is a distinct subset of lupus characterized by cutaneous findings (50%), cardiac conduction defects (50%), and autoantibodies to Ro (SS-A) antigen. HLA typing studies of Ro (SS-A) antibody-positive mothers of infants with NLE have shown an association with the HLA-DR3 phenotype. We report the clinical and serologic features of two infant-mother pairs who are U1RNP antibody positive and Ro (SS-A) antibody negative. HLA typing is reported on these infants, their mothers, and two additional infant-mother pairs with U1RNP antibody-positive lupus whose clinical features have been reported previously., Observations: Cutaneous findings included malar erythema, annular and polycyclic plaques, and scales that resolved with residual telangiectasia and hyperpigmentation 6 months after birth. Systemic abnormalities, including complete heart block, were absent. HLA typing revealed HLA-DR3 in two of four mothers, HLA-DR4 and HLA-DRw53 in two of four mothers, and either HLA-DQ1 or HLA-DQ3 in four of four mothers. No distinct HLA associations were seen in the three infants examined., Conclusions: The spectrum of cutaneous disease in U1RNP antibody-positive infants is similar to Ro (SS-A) antibody-positive infants with NLE. Complete heart block was not a feature of U1RNP antibody-positive NLE. HLA typing studies show a more diverse immunogenetic pattern in U1RNP antibody-positive mothers of infants with NLE compared with Ro (SS-A) antibody-positive mothers.

Background: Three percent to 13% of patients with cystic fibrosis present with protein-energy malnutrition that is characterized by hypoproteinemia, edema, and anemia and is associated with high morbidity and mortality. Cutaneous manifestations of malnutrition are rare in patients with cystic fibrosis and have been attributed to deficiencies of protein, zinc, and essential fatty acids., Observations: We describe five patients who presented with failure to thrive, hypoproteinemia, edema, and a cutaneous eruption before the onset of pulmonary symptoms and before the diagnosis of cystic fibrosis was made. The rash had a predilection for the extremities (lower > upper), perineum, and periorificial surfaces. In most cases, erythematous, scaling papules developed by 4 months of age and progressed within 1 to 3 months to extensive, desquamating plaques. Alopecia was variable, and mucous membrane or nail involvement was not observed. The rash was associated with malnutrition and resolved in all survivors within 10 days of providing pancreatic enzyme and nutritional supplementation. The pathogenesis of the rash is unclear, but it appears to stem from deficiencies of zinc, protein, and essential fatty acids and may be mediated by alterations in prostaglandin metabolism., Conclusions: Cystic fibrosis should be included in the differential diagnosis of the red, scaly infant, particularly when failure to thrive, hypoproteinemia, and edema are also present. Recognition of rash as a sign of cystic fibrosis complicated by protein-energy malnutrition will allow earlier diagnosis and treatment of these patients and may improve their outcome.

Eosinophilic pustular folliculitis is a cutaneous disorder that consists of recurrent crops of pruritic, sterile, papulopustules in a follicular distribution. In pediatric patients, EPF presents primarily in the scalp and is confused with several other more common dermatoses in children. The diagnosis of EPF rests on its inclusion in the differential diagnosis of papulopustular disorders, the recognition of the clinical presentation, and the presence of an eosinophilic infiltrate on biopsy. Treatment with midpotency topical corticosteroids has thus far met with modest success. This is the first report on EPF in the pediatric literature. As pediatricians become more aware of EPF as a distinct clinical entity and as our clinical experience and insight into the pathogenesis of EPF grows, perhaps more effective treatment modalities will be devised.

A survey of 220 adolescents attending a multiphysician pediatric office in Virginia was conducted to determine the frequency with which they used sunscreens. Eighty-one percent of the teenagers in the study stated that they spent most weekends in the sun; however, only 9% always used sunscreen, while 33% never did. Factors found to be associated with increased sunscreen use included female sex (odds ratio = 4.5, P less than .0001), having a best friend who routinely used sunscreen (odds ratio = 3.0, P less than .001), having parents who insisted on sunscreen use when the teenagers were children (odds ratio = 3.0, P = .006), and knowing that the maximum time for safe exposure to the sun is short (odds ratio = 6.2, P less than .0001). Adolescents with a history of skin cancer in the family were not more likely to use sunscreens than other teenagers. Thirty-three percent of the girls and 16% of the boys older than 15 years of age reported that they had visited a tanning salon at least once. This survey substantiates poor compliance with sunscreen use by teenagers despite increasing evidence of the dangers of excessive sun exposure.

Discrete areas of cutaneous hyperpigmentation were seen in two children with metastatic sarcoma who received chemotherapeutic bone marrow ablation with cyclophosphamide, etoposide, and carboplatin prior to autologous bone marrow transplantation. The hyperpigmented patches occurred only in areas of skin occluded by tape, electrocardiogram pads, or elastic bandages. Identical skin findings were reported in five adult women who received intravenous thiotepa and cyclophosphamide. Measurable levels of thiotepa were detected in these patients' serum, skin, sweat, and occluded gauze, suggesting that the chemical was excreted onto the skin surface in sweat and accumulated under occlusive dressing, thus producing some toxic effect on the epidermis or melanocytes resulting in abnormal pigmentation. We suspect that a similar mechanism was operative in our patients to produce the unusual patterned hyperpigmentation, and suggest that this complication may be prevented by minimizing sweat accumulation in areas occluded by adhesive materials.

We present two cases of asymptomatic, widespread keratotic eruptions in young female patients. Clinically, the lesions are well-demarcated, small, hypopigmented, flat-topped papules occurring on the trunk and extremities in a uniform distribution. Skin biopsy specimens from one patient revealed hyperorthokeratosis, papillomatosis, and a normal amount of melanin. We suggest that this is a newly recognized dermatologic entity that may be descriptively termed disseminated hypopigmented keratoses. Disseminated hypopigmented keratoses may be distinguished by clinical and histologic criteria from similar keratotic eruptions. Since the lesions of disseminated hypopigmented keratoses are both inconspicuous and asymptomatic, it is likely that the disorder is more prevalent than our two cases would suggest.

Nevoid basal cell carcinoma syndrome is a multisystem disease with a wide range of initial symptoms that can be seen at any age. The most characteristic features are vertebral or rib anomalies, intracranial falx calcification, multiple basal cell carcinomas, odontogenic keratocysts of the jaw, and palmar and/or plantar pits. Pediatricians need to be aware that if any one of these major anomalies is seen, this diagnosis should be considered. There now appears to be an established association between cardiac tumors and nevoid basal cell carcinoma syndrome. Primary cardiac tumors have been associated with cerebral tuberous sclerosis and neurofibromatosis, and evaluation of cardiac status is recommended when these genetically determined syndromes are diagnosed. This communication should serve to alert pediatricians to the need for complete cardiac evaluation and genetic counseling when a diagnosis of nevoid basal cell carcinoma is made.