Pediatric trichodysplasia spinulosa: A report of 2 cases and review of the literature.

Trichodysplasia spinulosa is a rare disorder caused by the ubiquitous trichodysplasia spinulosa-associated polyomavirus (TSPyV) and characterized clinically by predominately centrofacial, but often generalized, folliculocentric papules with protuberant keratinaceous spines. Although seroprevalence reaches up to 70% in adult populations, TSPyV causes clinical manifestations in a small percentage of patients who are immunosuppressed. Diagnosis can be made using typical clinical and histologic features, SV40T antibody immunostaining, and PCR of various tissues including the keratinaceous spine, skin, serum, urine, and CSF. Various topical and systemic medications have demonstrated variable success. Decreasing or discontinuing immunosuppression has also been shown to improve or alleviate clinical manifestations.

Hemorrhagic, bullous Henoch Schonlein purpura in a 16-year-old girl with previously undiagnosed dominant dystrophic epidermolysis bullosa.

A 16-year-old girl presenting with systemic and cutaneous symptoms of hemorrhagic Henoch-Schonlein purpura continued to develop bullae on top of old scars. Past history was significant for bullae on the feet and legs after trauma. Based on history, physical examination, disease course, and biopsy, the patient, her mother, and other family members were diagnosed with dominant dystrophic epidermolysis bullosa, explaining the severe course and complications of her Henoch-Schonlein purpura.

Intrinsically defective skin barrier function in children with atopic dermatitis correlates with disease severity.

BACKGROUND: Recent genetic evidence supports that an underlying defect in skin barrier function contributes to the pathogenesis of atopic dermatitis (AD). The integrity of the skin barrier can be assessed objectively by measuring transepidermal water loss (TEWL). Previous investigations of TEWL as a biomarker of skin barrier function have been limited by small sample size, and studies including African American subjects are lacking. OBJECTIVE: We sought to determine whether children with AD have inherently altered skin barrier function by comparing TEWL as a measure of skin barrier function in African American and white children with AD with that in control subjects without AD. METHODS: TEWL was measured on nonlesional normal-appearing skin at 4 sites (the volar forearm, dorsal arm, lower leg, and cheek) in (1) children with AD (cases), (2) children with asthma or allergic rhinitis but without AD (allergic control subjects), and (3) nonatopic control subjects. AD severity was assessed by using the objective SCORAD index. RESULTS: TEWL was increased in children with AD compared with that seen in both control groups at most of the anatomic sites tested (P < .05). TEWL also correlated with objective SCORAD score. The presence of allergic sensitization or other allergic conditions did not affect TEWL among children with AD. TEWL was higher in white than in African American children. CONCLUSION: Skin barrier function as assessed by TEWL is intrinsically compromised in children with AD but not in children with other allergic conditions. The magnitude of skin barrier dysfunction correlates with AD disease severity.

The Greater Cincinnati Pediatric Clinic Repository: A Novel Framework for Childhood Asthma and Allergy Research.

BACKGROUND: Allergic disorders, including asthma, allergic rhinitis, atopic dermatitis, eosinophilic esophagitis, and food allergy, are a major global health burden. The study and management of allergic disorders is complicated by the considerable heterogeneity in both the presentation and natural history of these disorders. Biorepositories serve as an excellent source of data and biospecimens for delineating subphenotypes of allergic disorders, but such resources are lacking. METHODS: In order to define subphenotypes of allergic disease accurately, we established an infrastructure to link and efficiently utilize clinical and epidemiologic data with biospecimens into a single biorepository called the Greater Cincinnati Pediatric Clinic Repository (GCPCR). Children with allergic disorders as well as healthy controls are followed longitudinally at hospital clinic, emergency department, and inpatient visits. Subjects' asthma, allergy, and skin symptoms; past medical, family, social, diet, and environmental histories; physical activity; medication adherence; perceived quality of life; and demographics are ascertained. DNA is collected from all participants, and other biospecimens such as blood, hair, and nasal epithelial cells are collected on a subset. RESULTS: To date, the GCPCR has 6,317 predominantly Caucasian and African American participants, and 93% have banked DNA. This large sample size supports adequately powered genetic, epidemiologic, environmental, and health disparities studies of childhood allergic diseases. CONCLUSIONS: The GCPCR is a unique biorepository that is continuously evaluated and refined to achieve and maintain rigorous clinical phenotype and biological data. Development of similar disease-specific repositories using common data elements is necessary to enable studies across multiple populations of comprehensively phenotyped patients.

Multiple juvenile xanthogranulomas in a 13-year-old.

A 13-year-old Caucasian girl presented with a 1(1/2) month history of multiple, asymptomatic, discrete, orange-yellow to skin-colored, dome-shaped, smooth, 3 to 6 mm papules on the arms, legs, trunk, and buttock. Pathology showed a dermal infiltrate of Touton-type giant cells, scattered lymphocytes, and macrophages. The patient was diagnosed with juvenile xanthogranuloma. We present this patient because of the uncommon presentation of multiple juvenile xanthogranulomas in a 13-year-old. Although juvenile xanthogranuloma generally occurs in infancy, it must be included in the differential diagnosis for an older child. This entity can less frequently occur in adults and typically a solitary lesion is found. Multiple juvenile xanthogranuloma is rare in older children and adults. Work-up should include a thorough review of systems, physical examination, and ophthalmology examination. Pediatricians and dermatologists should be aware that juvenile xanthogranulomas might occur in older children.

Tinea capitis mimicking dissecting cellulitis: a distinct variant.

BACKGROUND: Tinea capitis is a common scalp dermatosis with several clinical patterns. Only two patients with a presentation of tinea capitis mimicking dissecting cellulitis have been described in the English literature. OBSERVATION: We report a patient with tinea capitis mimicking dissecting cellulitis who did not respond to griseofulvin therapy at 16 mg/kg/day but eventually cleared after a protracted course of higher dose griseofulvin. CONCLUSION: recognition of a dissecting cellulitis-like pattern of tinea capitis will increase clinical suspicion and avoid inappropriate management of a recalcitrant "dissecting cellulitis" in favor of prompt antifungal therapy of appropriate dosage and duration for patients with this unusual variant of tinea capitis.

Complications of subcutaneous fat necrosis of the newborn: a case report and review of the literature.

Subcutaneous fat necrosis of the newborn is an uncommon, self-limited panniculitis of neonates. Rare complications such as hypercalcemia, thrombocytopenia, hypertriglyceridemia, and hypoglycemia have been reported. We describe the first case where all of the above complications were encountered in the same infant. Physicians caring for infants with subcutaneous fat necrosis of the newborn should be aware of the above associations in order to provide prompt and appropriate treatment to prevent associated, undesirable sequelae.

Cutaneous arteritis presenting with hyperpigmented macules: macular arteritis.

Macular arteritis is a novel form of cutaneous arteritis in which the primary lesion is a hyperpigmented macule. Traditional stigmata of cutaneous vasculitis such as palpable purpura and erythematous nodules are not present. The disease is asymptomatic and appears to follow an indolent course. Systemic involvement has not been observed.