Biomedical subjects
Publications and source records attributed to P J Honig.
Types of lesions and patterns of distribution aid in the recognition of viral exanthems. Varicella and hand-foot-and-mouth disease can appear atypically in areas of inflammation. We describe 5 cases of morphologically altered exanthems that localized early and preferentially to areas of inflammation.
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Atopic dermatitis is the most common skin disease of childhood, and its prevalence has steadily increased over the last three decades. A chronic, relapsing condition, atopic dermatitis has a significant impact on affected children, their families, and the community at large. Although the fundamental pathogenesis has remained elusive, intensive research has greatly contributed to our understanding of this disease. As the specific immunobiologic pathways become deciphered, we have seen the propagation of several new therapeutic options that rationally attack specific underlying immune system abnormalities. This article highlights the specific contributions made to the literature over the past year, with particular attention to the immunopathogenesis of atopic dermatitis, as well as some new targeted therapies currently and soon to be available.
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.
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The clinical presentation of tinea capitis may be varied and confusing. This results in delays in diagnosis, loss to follow-up, and spread of the infection. This article describes the use of calcofluor white (CFW) as a method for rapid diagnosis of tinea capitis. One hundred children with scaling scalps were evaluated. Specimens were obtained for fungal culture from each patient. Scales and hair were tested using the CFW test. The CFW test was positive in 58 (76%) of those with positive fungal cultures and 10 (42%) with negative fungal cultures. The sensitivity and specificity of the test were 76 and 58%, respectively. The relative ease and rapidity of the CFW test allows for rapid diagnosis of tinea capitis, immediate treatment of patients, and decreased loss to follow-up.
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We observed a newborn infant of a previously reported kindred with absent dermal ridge pattern, syndactyly, and facial milia. The infant's features were consistent with three other kindreds, suggesting that this entity is a single disorder with variable expression. Furthermore, this entity should be considered in the differential diagnosis of excessive congenital facial milia and erosions.
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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.
Therapy for kerions was evaluated by randomly assigning 30 patients to one of four treatment groups: group A griseofulvin, group B griseofulvin plus erythromycin, group C griseofulvin plus prednisone, and group D griseofulvin, erythromycin, and prednisone. Data indicate that antibiotic and steroid therapy, in addition to griseofulvin, may reduce scaling and pruritus, but does not reduce the time it takes for kerions to flatten.
BACKGROUND: Atopic dermatitis is an important cause of morbidity in children of all ages. Despite its high prevalence, there has been no examination of ways in which care for atopic dermatitis is delivered. OBJECTIVE: This study reviewed the costs for care of childhood atopic dermatitis in an urban setting and estimated the national cost for treatment of the disease. METHODS: We used data from one children's hospital to study the use of the emergency room for atopic dermatitis and used national data sets to estimate the cost of care in the United States. RESULTS: A large proportion of visits occur in the emergency department, during daytime office hours, and mostly by patients who have public insurance. The total national cost for treatment of childhood atopic dermatitis is $364 million annually, which is a conservative estimate. CONCLUSION: Given its high prevalence, associated morbidity, and cost, resources must be better allocated to improve the organization of care for patients with atopic dermatitis.
Qualitative and semiquantitative bacterial culture specimens were obtained from 44 patients with kerions. Staphylococcus aureus was isolated from the scalp surface overlying the kerion in 29% of patients and from the pus within the kerion in 48% of patients. Gram-negative bacteria were found in the same locations in 11.3% and 18% of patients, respectively. The data indicate that bacteria are frequently cultured from kerions.
We describe a 2 1/2-year-old boy who has hirsutism and ringed creases of the extremities associated with an underlying smooth muscle hamartoma. Cutaneous findings in this child resemble those in other reports of the "Michelin tire syndrome." Histologic examination showed numerous well-demarcated fascicles of smooth muscle cells randomly distributed at all levels of the reticular dermis with haphazard orientation. These cells were immunoreactive with desmin, which confirmed their smooth muscle nature. In addition to the skin changes, this child has multiple unusual phenotypic anomalies, some of which have not previously been associated with the Michelin tire syndrome. These include distinctive facial dysmorphia, submucous cleft palate, lateral clefting of the mouth, genital, and dental anomalies. He also developed seizures at age 2 1/2 years and has moderate developmental delay. The patient and his mother have apparently identical paracentric inversions of the long arm of chromosome 7 (46,XY,inv(7)(q22q31.3) with no detectable loss or gain of either chromosomal material or DNA markers from the cystic fibrosis (CFTR) region. The relevance, if any, of the karyotypic abnormality to the phenotype in this child is discussed.
Cutaneous fragility at age 2 years with blistering, scarring, milia, and hypertrichosis at age 4 years were noted in an otherwise healthy girl who had no family history of porphyria. Results of porphyrin analyses of urine, serum, and red blood cells revealed a pattern consistent with porphyria cutanea tarda. Red blood cell uroporphyrinogen decarboxylase activity was diminished to approximately 50% of normal in the child and in her mother and maternal grandmother, who were without symptoms; activity was normal in her sister, father, and maternal grandfather. Therapeutic phlebotomies were followed by a biochemical and clinical remission.
BACKGROUND: A neonate with epidermolysis bullosa herpetiformis (EBH) (Dowling-Meara) had an undescribed ultrastructural and immunohistochemical abnormality. OBJECTIVE: The objective was to clarify the ultrastructural and immunohistochemical abnormalities in EBH to gain further insight into the pathogenesis of this disorder. METHODS: Tissue from the patient was studied with routine histochemistry, electron microscopy, and immunohistochemistry. RESULTS: Excessive clumping of tonofilaments on electron microscopic examination, anomalous hemidesmosomes, and immunohistochemical evidence of aberrant keratin expression by basal epidermal cells was found. CONCLUSION: This case of EBH provides further evidence for primary abnormalities involving cytoskeletal-membrane attachment plaque formation in this rare disorder.