Joseph G. Morelli

Does Sensitization to Contact Allergens Begin in Infancy

Objective. Because previous studies have found allergic contact sensitization common in children by 5 years of age, our aim was to determine the prevalence of positive epicutaneous test results in children <5 years of age and to determine whether sensitization to contact allergens was as common in infancy. Methods. We recruited 95 asymptomatic children 6 months to 5 years of age from well-child visits at Denver area pediatric practices for epicutaneous patch testing using the T.R.U.E. Test system. Allergens were placed on the skin for 48 hours, and at a later follow-up visit, positive reactions were evaluated. Results. A total of 85 patients completed the study. Of these, 20 (24.5%) had 1 or more positive reactions to the tested allergens. Positive reactors ranged from 6 to 65.5 months of age, with an average of 30.4 months of age. Of the children, 16 reacted to 1 allergen, and 4 reacted to 2. Eleven positive reactions were observed to nickel, followed by 8 to thimerosal. Other positive reactions were to neomycin, cobalt, and kathon CG. Conclusions. Children as young as 6 months of age may be sensitized to contact allergens. Within this pediatric population, the prevalence of sensitization is 24.5%. Sensitization to contact allergens may occur in infants.

Block the sun, not the fun: evaluation of a skin cancer prevention program for child care centers.

INTRODUCTIONnThis paper describes the evaluation of a skin cancer prevention program for preschools and daycare centers. The intervention was targeted primarily at staff of child care centers, with the aim of increasing use of sun protection practices for young children while attending these centers. Secondary target groups included parents and the children themselves. The intervention, which adopted the slogan, Block the Sun, Not the Fun, included workshops for child care center staff, and information/activity packets for parents.nnnMETHODSnTwenty-seven preschools and daycare centers were randomly assigned to an intervention or wait-list control group. The intervention group received the intervention during the spring of 1994; the wait-list control group received the intervention during the spring of 1995. Evaluation consisted of interviews with center directors, observations of practices, and review of written policies before the intervention (in summer, 1993) and after the intervention (in summer, 1994). A survey of 201 parents was conducted during late summer 1994.nnnRESULTSnWhile the intervention did not appear to change the sun protection attitudes or practices of parents, or use of clothing and shade at child care centers, results suggested significant changes in the sun protection knowledge/attitudes of center directors and the use of sunscreen at child care centers. Additionally, parents with children attending centers in the intervention group were more likely to be satisfied with sun protection practices at their centers.nnnCONCLUSIONnThis low-intensity intervention appears to be effective at changing sun protection attitudes and sunscreen use at child care centers, and can be easily replicated. However, high staff turnover at child care centers would suggest that boosters will be necessary to sustain the impact. More intensive efforts directed at social norms are likely to be necessary to change clothing and outdoor play practices.

Cutaneous pustular leukemoid reactions in trisomy 21

Abstract: We report two neonates with Down syndrome and postnatal leukemoid reactions who developed acute widespread pustular eruptions. The white blood cell (WBC) counts on the first day of life were markedly elevated, with blasts seen on examination of the peripheral blood smear. The skin eruptions progressed and became pustular. Viral and bacterial cultures were negative. Skin examination revealed pustules on an erythematous base on the cheeks, shoulders, trunk, and proximal extremities. Skin biopsy specimens showed an intraepidermal pustule with an inflammatory infiltrate including neutrophils, eosinophils, and mononuclear cells. The mononuclear cells had atypical, immature‐appearing nuclei. In patient 1, these cells were strongly myeloperoxidase positive on immunohistochemistry, indicating myeloid lineage. In patient 2, these cells were CD3‐positive T cells. Patient 1 received a 5‐day infusion of continuous cytarabine (ara‐C) secondary to high WBC counts and symptomatic hyperviscosity. During therapy, the high WBC count and the pustules resolved. The lesions of patient 2 improved with topical mometasone furoate and resolved as her WBC count decreased. Recently, similar cases have been reported. Transient myeloproliferative disorders, or leukemoid reactions, should always be considered when newborns with Down syndrome or trisomy 21 mosaicism develop a pustular eruption.

Inflammatory nevus comedonicus in children.

More than 100 years has passed since the first report of a nevus comedonicus. The earliest reports emphasized the inflammatory aspect of the nevus comedonicus as being the most significant problem. In the past 30 years, publications have ignored the inflammatory aspect of nevus comedonicus while emphasizing a variety of associated malformations. In this review, we describe five prepubertal children with prominent and persistent inflammatory changes limited to areas within a nevus comedonicus. In our experience, inflammation can be severe and resistant to treatment. Ultimately, surgical removal of the involved skin was required in two children.

Identification and pharmacological characterization of platelet-activating factor and related 1-palmitoyl species in human inflammatory blistering diseases.

Through its pro-inflammatory effects on leukocytes, endothelial cells, and keratinocytes, the lipid mediator platelet-activating factor (PAF) has been implicated in cutaneous inflammation. Although the 1-alkyl PAF species has been considered historically the most abundant and important ligand for the PAF receptor (PAF-R), other putative ligands for this receptor have been described including 1-acyl analogs of sn-2 acetyl glycerophosphocholines. Previous bioassays have demonstrated a PAF-like activity in lesions of the autoimmune blistering disease bullous pemphigoid. To assess the actual sn-2 acetyl glycerophosphocholine species that result in this PAF agonistic activity, we measured PAF and related sn-2 acetyl GPCs in fresh blister fluid samples from bullous pemphigoid and noninflammatory (suction-induced) bullae by mass spectrometry. We report the presence of 1-hexadecyl as well as the 1-acyl PAF analog 1-palmitoyl-2-acetyl glycerophosphocholine (PAPC) in inflammatory blister fluid samples. Because PAPC is the most abundant sn-2 acetyl glycerophosphocholine species found in all samples examined, the pharmacological effects of this species with respect to the PAF-R were determined using a model system created by transduction of a PAF-R-negative epidermoid cell line with the PAF-R. Radioligand binding and intracellular calcium mobilization studies indicated that PAPC is approximately 100x less potent than PAF. Though a weak agonist, PAPC could induce PAF biosynthesis and PAF-R desensitization. Finally, intradermal injections of PAF and PAPC into the ventral ears of rats demonstrated that PAPC was 100x less potent in vivo. These studies suggest possible involvement of PAF and related species in inflammatory bullous diseases.

Inflammatory nuchal-occipital port-wine stains

A port-wine stain is a congenital vascular anomaly caused by a malformation of the papillary dermal capillaries. We observed three children with an unusual appearance of their port-wine stains. All had a prominent inflammatory component consisting of scaling, excoriations, oozing, and crusting, resembling a dermatitis. In two patients, the port-wine stain occurred on the nape of the neck; in the third, it occurred over the occipital area of the scalp. Treatment with topical steroids helped to decrease the scaling and crusting, but when the steroids were stopped the inflammatory component returned. In tow patients, the lesions were treated with the flashlamp-pumped pulsed dye laser with almost total clearing of the port-wine stains and complete absence of the inflammatory component after one treatment. The recognition of this inflammatory manifestation of port-wine stains will enable earlier diagnosis and allow for effective treatment with the pulsed dye laser.

Self-healing juvenile cutaneous mucinosis

Abstract: A healthy 14‐month‐old black girl presented with a 3‐week complaint of “knots” on the face and hands. The lesions were acute in onset and asymptomatic. Multiple, firm, nontender, skin‐colored to erythematous nodules were noted on the scalp, forehead, axillae, lower legs, abdomen, and hands. A skin biopsy specimen revealed a well‐circumscribed accumulation of mucin in the reticular dermis. Colloidal iron stain was positive. Radiographs showed soft tissue prominence only. Serum protein electrophoresis, thyroid function tests, complete blood count, sedimentation rate, and antinuclear antibody were normal, except for lymphocytosis. Findings were consistent with self‐healing juvenile cutaneous mucinosis (SHJCM). SHJCM is a condition of unknown etiology characterized by rapid onset of asymptomatic, indurated papules or nodules. Affected children may have arthralgias, but are otherwise well. Spontaneous resolution is the rule. Most skin lesions in our patient had resolved within 6 months of onset. This patient is unique because of the young age of onset.

Interactions between Ultraviolet Light and MC1R and OCA2 Variants Are Determinants of Childhood Nevus and Freckle Phenotypes

Background: Melanocytic nevi (moles) and freckles are well known biomarkers of melanoma risk, and they are influenced by similar UV light exposures and genetic susceptibilities to those that increase melanoma risk. Nevertheless, the selective interactions between UV exposures and nevus and freckling genes remain largely undescribed. Methods: We conducted a longitudinal study from ages 6 through 10 years in 477 Colorado children who had annual information collected for sun exposure, sun protection behaviors, and full body skin exams. MC1R and HERC2/OCA2 rs12913832 were genotyped and linear mixed models were used to identify main and interaction effects. Results: All measures of sun exposure (chronic, sunburns, and waterside vacations) contributed to total nevus counts, and cumulative chronic exposure acted as the major driver of nevus development. Waterside vacations strongly increased total nevus counts in children with rs12913832 blue eye color alleles and facial freckling scores in those with MC1R red hair color variants. Sunburns increased the numbers of larger nevi (≥2 mm) in subjects with certain MC1R and rs12913832 genotypes. Conclusions: Complex interactions between different UV exposure profiles and genotype combinations determine nevus numbers and size, and the degree of facial freckling. Impact: Our findings emphasize the importance of implementing sun-protective behavior in childhood regardless of genetic make-up, although children with particular genetic variants may benefit from specifically targeted preventive measures to counteract their inherent risk of melanoma. Moreover, we demonstrate, for the first time, that longitudinal studies are a highly powered tool to uncover new gene–environment interactions that increase cancer risk. Cancer Epidemiol Biomarkers Prev; 23(12); 2829–39. ©2014 AACR.

Sun damage in ultraviolet photographs correlates with phenotypic melanoma risk factors in 12-year-old children

BACKGROUNDnUltraviolet (UV) photography has been used to motivate sun safety in behavioral interventions. The relationship between sun damage shown in UV photographs and melanoma risk has not been systematically investigated.nnnOBJECTIVEnTo examine the relationship between severity of sun damage in UV photographs and phenotypic melanoma risk factors in children.nnnMETHODSnUV, standard visible and cross-polarized photographs were recorded for 585 children. Computer software quantified sun damage. Full-body nevus counts, skin color by colorimetry, facial freckling, hair and eye color were collected in skin examinations. Demographic data were collected in telephone interviews of parents.nnnRESULTSnAmong 12-year-old children, sun damage shown in UV photographs correlated with phenotypic melanoma risk factors. Sun damage was greatest for children who were non-Hispanic white and those who had red hair, blue eyes, increased facial freckling, light skin and greater number of nevi (all P values < .001). Results were similar for standard visible and cross-polarized photographs. Freckling was the strongest predictor of sun damage in visible and UV photographs. All other phenotypic melanoma risk factors were also predictors for the UV photographs.nnnLIMITATIONSnDifferences in software algorithms used to score the photographs could produce different results.nnnCONCLUSIONnUV photographs portray more sun damage in children with higher risk for melanoma based on phenotype. Therefore sun protection interventions targeting those with greater sun damage on UV photographs will target those at higher melanoma risk. This study establishes reference ranges dermatologists can use to assess sun damage in their pediatric patients.

“Painful and Disabling Granuloma Annulare”: A Case of Munchausen by Proxy

Abstract: We report the unusual case of a child who from age 6 received fourteen excisions and four grafting procedures in an attempt to control “painful and disabling granuloma annulare.” Although the lesions were considered disabling by the mother, they were nontender on repeated examinations. A careful investigation revealed that the mother was falsely describing symptoms necessitating surgery so she could receive public housing and other benefits for parents with a disabled child. We believe this case should alert the clinician as to the extreme measures imposed on children in Munchausen syndrome by proxy.