Neurologic manifestations of the organoid nevus syndrome.

Prominent neurologic abnormalities were observed in six patients with epidermal or linear sebaceous nevi (organoid nevi). These cases were remarkable for unilateral facial nevi, cognitive impairment, seizures, and focal or lateralized epileptic EEG abnormalities. Additional manifestations included the onset of seizures in the neonatal period, unilateral hypsarrhythmia or Lennox-Gastaut EEG pattern, hemiparesis, asymmetric macrocephaly, and somatic growth disturbances. The full expression of this disorder was not apparent at birth, but emerged gradually during infancy. The neurologic abnormalities in these patients were attributed to unilateral or asymmetric malformations of the CNS as demonstrated by computed tomography. A lateralized disorder of neuroectodermal proliferation, differentiation, and migration could account for both the cutaneous and neurologic abnormalities in this disorder. The striking clinical similarities in these patients suggest a close link between epidermal and linear sebaceous nevi.

Forehead lipoblastoma mimicking a hemangioma.

A case of forehead lipoblastoma simulating a hemangioma in a male infant is reported, to alert pediatricians to this rare tumor and to increase the index of suspicion in atypical hemangiomas. A 2-month-old male infant developed a protruding forehead mass with increased vascularity. It demonstrated progressive and accelerated growth over the subsequent 6 months, unresponsive to steroid therapy. A magnetic resonance imaging scan supported the diagnosis of hemangioma because of the hypervascular nature of the lesion. Surgical excision was performed because of visual obstruction. Pathologic examination of the specimen was consistent with a very primitive lipoblastoma. This tumor is a rare, benign lesion of immature fat cells that is found almost exclusively in the pediatric population. Lipoblastomas are more common in males than females and frequently present as asymptomatic, rapidly enlarging, soft lobular masses on the extremities. Complete surgical excision is the definitive treatment. In the vast majority of reported cases, however, the preoperative diagnosis was incorrect, underscoring the diagnostic dilemma presented by these rare tumors.

Semiquantitative study of tinea capitis and the asymptomatic carrier state in inner-city school children.

OBJECTIVE: To quantify and characterize the asymptomatic carrier state of tinea capitis in school children from the inner city. METHODS: All students attending a parochial school (kindergarten through seventh grade) in the city of Philadelphia were cultured for tinea capitis periodically over 16 months (1404 hemi-scalp cultures from 224 children). RESULTS: Our initial prevalence study of this all-black population (ages 5 to 13 years) found a 3% rate of index cases (symptomatic) and a 14% rate of asymptomatic carriers (without black-dot lesions, obvious hair loss, scaling, crusts, pustules, or erythema). Trichophyton tonsurans was the predominant dermatophyte (96% of 125 positive cultures; Microsporum canis was the only other isolate). Fifty percent of all positive cultures came from children in kindergarten and first grade; first grade had the highest rate of index cases. The overall prevalence of asymptomatic carriers was not higher in the classes containing index cases. Fifty-nine percent of asymptomatic carriers had a 1+ spore load (1 to 10 colonies isolated per scalp), while 74% of index cases had a 4+ spore load (> 150 total colonies). Forty-five untreated asymptomatic carriers were followed for 2 to 5 months: 19 (42%) became culture-negative; of these, 17 (90%) had a 1+ spore load. CONCLUSIONS: We found that inner-city black school children who are asymptomatic carriers of T tonsurans had lower spore loads than index cases. Index cases did not appear to be the primary mode of transmission within a classroom. More than half of untreated asymptomatic carriers remained culture-positive after 2 months and probably play a role in the transmission of tinea capitis within this population.

Selenium sulfide: adjunctive therapy for tinea capitis.

Selenium sulfide lotion used as a shampoo has been shown to be an effective adjunctive agent to griseofulvin in the treatment of tinea capitis. Of 16 children with Trichophyton tonsurans infections 15 had negative fungal cultures at two weeks following a regimen of daily oral griseofulvin and selenium sulfide shampooing twice weekly. All patients treated with griseofulvin alone or in combination with either a bland shampoo or topical clotrimazole had positive cultures not only at the two-week interval but also as long as eight weeks later. In vitro analysis shows selenium sulfide to be sporicidal, correlating well with the in vivo observations. It is postulated that selenium sulfide usage may lessen the chances for spreading of infectious spores to other individuals.

Purpuric phototherapy-induced eruption in transfused neonates: relation to transient porphyrinemia.

OBJECTIVE: Blue light phototherapy is commonly administered to neonates as treatment of indirect hyperbilirubinemia, often in conjunction with blood transfusions to treat hemolytic anemia. We observed a distinctive cutaneous complication of phototherapy in six neonates with hyperbilirubinemia. METHODOLOGY: We studied the clinical and histologic characteristics of the eruption, as well as the porphyrin levels in affected neonates. Five of the patients had erythroblastosis fetalis; the other had profound anemia from twin-twin transfusion. All of the neonates developed purpuric patches at sites of maximal exposure to the phototherapy lights, with dramatic sparing at shielded sites within 24 hours after initiation of the phototherapy. On discontinuation of phototherapy, all eruptions cleared within 1 week. Examination of skin biopsy sections showed purpura without significant inflammation or keratinocyte necrosis. Plasma porphyrins (copro- and proto-) were elevated in the two patients in which they were assessed. CONCLUSIONS: The distribution of the eruption in areas exposed to light and presence of circulating porphyrins suggest that porphyrinemia may underlie the light-induced purpuric eruption. Additional studies will be required to determine definitively the mechanisms of both the purpuric phototherapy-induced eruption and the development of increased blood porphyrin levels in these transfused neonates.

Congenital herpes simplex virus infections. Report of three cases and review of the literature.

Three infants with congenital herpes simplex virus infection were examined and treated. One demonstrated findings of individual vesicles on an erythematous base; another had pustules. The children were treated with vidarabine intravenously. Two of the three survived. The literature suggests a potential increase in the occurrence of this infection in neonates. Since the dermatologist is often asked to aid in the diagnosis of this entity and early recognition is vital, it is important to be familiar with the possible causes of vesicopustular lesions, as well as the epidemiology, clinical manifestations, pathogenesis, and current forms of treatment of congenital herpes simplex virus infections.

Etretinate therapy for generalized pustular psoriasis in children.

Two children, 19 months of age, were independently treated with a compassionate protocol of etretinate therapy for recalcitrant, debilitating pustular psoriasis. Laboratory test results, roentgenograms of the spine, and bone age were periodically monitored. Over a 31/2-year period of intermittent treatment with a maximum dosage of 1.5 mg/kg/d, both children showed remarkable improvement with no apparent drug effect on growth and development. Side effects included xerosis, skin fragility and transient, minimal elevations of aspartate aminotransferase, lactic dehydrogenase, and triglyceride levels. Etretinate therapy may prove to be a viable treatment option for the child with intractable pustular psoriasis that seriously impairs quality of life.

U1RNP antibody-positive neonatal lupus. A report of two cases with immunogenetic studies.

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.

Erythroderma, hypogammaglobulinemia, and T-cell lymphocytosis. Occurrence following therapy with phenytoin.

Erythroderma and exfoliative dermatitis developed in a 17-year-old boy following therapy with phenytoin sodium. Immunologic studies performed early in the course of the dermatitis disclosed panhypogammaglobulinemia and a marked increase in T lymphocytes that responded poorly in vitro to T-cell mitogens. After therapy with prednisone, the dermatitis improved somewhat, and the patient's lymphocytes proliferated in vitro when exposed to phenytoin. We speculate that our patient's acute hypersensitivity reaction may have been mediated by an excessive number of phenytoin-sensitized suppressor-cytotoxic T lymphocytes and may represent a disorder of immunoregulatory T cells.

Blistering disorders in childhood.

Many conditions present in childhood with vesicles and bullae, ranging from benign conditions to life-threatening diseases. This article reviews selected blistering disorders, with emphasis on new information that is helpful to the practicing physician. Entities that are discussed in this article include childhood zoster and fetal varicella syndrome, urticaria pigmentosa, scabies, bullous bug bites, lichen sclerosus et atrophicus, epidermolysis bullosa, and erythema multiforme.

Lymphangitis of the anterior chest wall.

Lymphangitis of the anterior chest wall in children is an unusual finding and may be a diagnostic enigma. The following case report describes a five year old boy who presented with this condition. The diagnosis was somewhat elusive until a history of an antecedent insect bite was obtained. Lymphangitis should therefore be considered in the differential diagnosis when perplexing erythematous chest wall lesions are present in children.

An approach to psychiatric referrals in pediatric patients. Psychosomatic complaints.

Psychiatric referral is an important aspect of clinical pediatric practice. This paper discusses referral to a family-oriented psychiatrist of difficult families with children who have an acute or chronic psychosomatic complaint. It is the responsibility of primary care physicians to develop facility in making psychiatric referrals. In this way the physician will be equipped to deal effectively with cases requiring attention beyond his time and expertise, as well as cases that he will manage alone. The paper considers five decision-points relevant for pediatricians making effective referrals of children with somatic complaints. (1) The pediatrician decides whether to conduct the evaluation on an outpatient or inpatient basis. In either case the work-up should be completed rapidly. A diagnostic hospitalization, whereby the parents agree to accept the results of a clearly defined evaluation as definitive, may be used with inpatient evaluations. The proposal of such a diagnostic hospitalization is considered. (2) After the medical evaluation is completed, the pediatrician decides whether to manage the case alone or to make a psychiatric referral. (3) A family conference can be used to initiate pediatric management or to make a psychiatric referral. A joint meeting, with both pediatrician and psychiatrist present, may be a crucial part of the management of difficult psychosomatic cases. The four stages of a typical joint meeting are described. (4) The utility of giving indirect rather than direct explanations of the meaning of a somatic symptom is described. By scapegoating the symptom rather than either the child or the parents, indirect explanations are often less threatening and more acceptable to the family. (5) Similarly, psychiatric involvement is often best proposed by recommending evaluation rather than treatment, since evaluation is less binding and takes into account the natural reluctance of many families to see the psychiatrist. After the referral has been made, ongoing contact between psychiatrist and pediatrician is important.

Scaling scalp diseases in children.

Scaling scalp diseases in 142 children were evaluated. Included were seborrheic dermatitis, atopic dermatitis, tinea capitis, and psoriasis. Clinical signs were noted, and various treatments were then undertaken. Patients with seborrheic dermatitis responded well to treatment with antiseborrheic shampoo and topical corticosteroids. Atopic disease was more resistant to treatment, but did improve with less frequent shampooing and twice daily topical corticosteroid application. Selenium shampoo was a useful adjunct in the treatment of tinea capitis (cultures became negative at two weeks compared to persistent positivity in other treatment groups).

Hemothorax in a child. An unusual cause of chest pain.

The pediatrician should search carefully in a child with chest pain for evidence in the history or physical examination of an organic cause. Hemothorax is one of the pathologic processes that can present with this symptom. The differential diagnosis of hemothorax in children is reviewed.

Congenital ingrown toenails: clinical significance.

The great toenails of 302 newborn infants were evaluated to identify a specific conformation which might predispose these children to chronic paronychia. Forty-one infants who appeared to have great toenails impeded by tissue distally were followed for 12 months. All had essentially normal appearing nails by six months of age. None developed chronic paronychia. The results suggest that the changes originally noted were variations in the normal development of the great toenail.

Diaper dermatitis. Factors to consider in diagnosis and treatment.

Diaper dermatitis is a common problem in infants and young children. Although the pathogenesis of diaper dermatitis is not well defined, many associated factors have been identified, including individual predisposition to atopic or seborrheic dermatitis, occlusion and friction caused by the diaper, and overgrowth of bacteria and yeast. General treatment goals involve keeping the diaper area as dry as possible, washing the area as infrequently as possible, and avoiding tight-fitting diapers. Petrolatum, talc, baking soda, and fluorinated topical steroidal medications should be avoided. If all else fails, toilet training provides the final answer.