Perceptions of skincare ingredients and product labels in pediatric eczema care.

Educating pediatric eczema patients and caregivers on appropriate product selection and avoidance of common irritants or allergens is a crucial aspect of eczema management. This study surveyed 80 pediatric caregivers in an academic pediatric dermatology clinic to assess influential factors in caregivers' selection of pediatric eczema-care products and identify ways to improve patient counseling on appropriate product selection and avoidance of common irritants or allergens. Caregivers frequently reported positive perceptions of commonly recommended ingredients for eczema but had inconsistent perceptions of fragrant plant oils and extracts, regardless of previous counseling on fragrance avoidance. These findings demonstrate uncertainty and misperceptions perpetuated by product labeling and a need for improved counseling strategies for avoiding fragrance and excessive product costs.

Oral candidiasis and seborrheic dermatitis in an 8-year-old female with a heterozygous variant of the IL-17RC gene.

An 8-year-old female with chronic oral candidiasis and severe seborrheic dermatitis was found to have a heterozygous mutation (p.R14X c.40 C>T) of the IL-17RC gene, which was predicted to possibly represent a new pathogenic variant via truncation or nonsense-mediated mRNA decay. Given previously reported IL-17RC-related disorders are autosomal recessive, we would expect an affected individual to have two mutated alleles whereas our patient was heterozygous. Given the overlapping clinical picture, this variant could be responsible for altered immunity against both Candida and Malassezia species. This is the first report to our knowledge of chronic oral candidiasis and severe seborrheic dermatitis in a patient with a heterozygous variant (p.R14X c.40 C>T) for the IL-17RC gene.

Distinguishing annular pustular psoriasis from subcorneal pustular dermatosis-A diagnostic dilemma in a 10-year-old boy.

Subcorneal pustular dermatosis (SPD) and annular pustular psoriasis (APP) are very rare in childhood and difficult to differentiate both clinically and histopathologically. We report the case of a 10-year-old male with a 9-year history of erythematous scaly annular plaques with scattered pustules on the trunk. Although initially diagnosed as SPD, a lack of response to dapsone, presence of spongiosis on histology, and early age of disease onset led to consideration of APP. The patient was subsequently treated with adalimumab 80 mg weekly and completely cleared. This case illustrates the overlapping features of SPD and APP and suggests that the two disorders may represent a spectrum of the same disease.

The role of nutrition in acne vulgaris and hidradenitis suppurativa.

Acne vulgaris and hidradenitis suppurativa (HS) are chronic inflammatory, multifactorial skin disorders that often develop in adolescence and young adulthood. Both acne vulgaris and HS can cause significant morbidity and psychologic distress, with a negative effect on the quality of life. The relationship among diet, acne, and HS remains somewhat controversial; however, there is increasing evidence that high-glycemic diets and consumption of milk and dairy products promote acne. Studies suggest that weight loss through dietary interventions or bariatric surgery and brewer's yeast exclusion diets have the potential to ameliorate the signs of HS. We review the role of diet in the pathogenesis, prevention, and treatment of HS and acne vulgaris.

Simultaneous occurrence of idiopathic trachyonychia in dizygotic twins.

Trachyonychia (or twenty-nail dystrophy) is an uncommon chronic disorder manifesting as thin, flattened, brittle nails with excessive longitudinal ridging and loss of luster creating a "sandpaper-like" texture that most commonly presents spontaneously in childhood as an isolated phenomenon; however, it has been historically associated with numerous dermatoses. Rarely, trachyonychia has been reported to occur in families, suggesting a potential hereditary predisposition. We report trachyonychia occurring simultaneously in dizygotic twins, further supporting a possible underlying genetic basis of this idiopathic nail disorder.

Acne: Kids are not just little people.

Acne vulgaris is a common inflammatory disease of the pilosebaceous follicles that affects patients of all ages, from neonates to adults. We have compared and contrasted the clinical presentation of acne in neonates, infants, children, teenagers, and young adults and review the scenarios in which further systemic endocrine or hormonal tests are indicated. We also discuss age-dependent treatment considerations, including appropriate oral antimicrobial regimens and the proper dosing of isotretinoin in young children versus teenagers and adults.

Psoriasis in adults and children: Kids are not just little people.

Pediatric psoriasis is a common skin disorder. Although pediatric psoriasis has many similarities to adult psoriasis, there are differences in presentation, particularly in infants. In addition, a more limited therapeutic armamentarium exists for children due to different inherent risks. Nearly all therapies are unapproved in the pediatric population, with far fewer pre- and postmarketing studies having been conducted. Despite these challenges, appropriate treatment should never be denied to a young patient, as physical and psychosocial ramifications of psoriasis in the school age child and adolescent are significant. A partnership with the patient, caregivers, and primary care providers is necessary and rewarding in meeting the challenges of this chronic, incurable disease. We address the presentation, treatment options, and counseling necessary when treating psoriasis in the pediatric population.

Six children with allergic contact dermatitis to methylisothiazolinone in wet wipes (baby wipes).

Methylchloroisothiazolinone/methylisothiazolinone (MCI/MI) is a combination preservative used in personal care and household products and is a common cause of allergic contact dermatitis (ACD). Recently, MI alone, without MCI, has been increasingly used in consumer products in attempts to minimize allergic reactions. Wet wipes are extensively tested and traditionally believed to be innocuous. MI in wet wipes ("baby wipes") has not been previously reported to cause ACD in children in the United States. Only 1 previous report of ACD in a child in Belgium has been recently reported. We report 6 children with chronic, perianal/buttock, and facial eczematous dermatitis, refractory to multiple topical and oral antibiotics and corticosteroids. All tested positive to MCI/MI on patch testing. None wore diapers. All patients had been using wet wipes containing MI (without MCI) to affected areas. Discontinuation of wipes resulted in rapid and complete resolution. This is the first report of pediatric ACD to MI in wet wipes in the United States, and the largest series to date. ACD to MI in wet wipes is frequently misdiagnosed as eczema, impetigo, or psoriasis. Wet wipes are increasingly marketed in personal care products for all ages, and MI exposure and sensitization will likely increase. Dermatitis of the perianal, buttock, facial, and hand areas with a history of wet wipe use should raise suspicion of ACD to MI and prompt appropriate patch testing. Rapid resolution occurs after the allergen exposure is eliminated. All isothiozolinones should be avoided in personal care and household products for these patients.

Congenital multiple clustered dermatofibroma in a 12-year-old girl.

Congenital multiple clustered dermatofibroma (MCDF) is a rare, idiopathic, benign tumor presenting at birth as an asymptomatic hyperpigmented patch that is stable until puberty, at which time it enlarges and develops papules. Ultimately, MCDF appears to follow a stable, benign course. We present a case of a 12-year-old girl with congenital MCDF. To our knowledge, this is only the third reported case of congenital presentation of MCDF and the only case featuring atrophoderma-like depression.

Molecular diagnosis of a benign proliferative nodule developing in a congenital melanocytic nevus in a 3-month-old infant.

Small and intermediate congenital melanocytic nevi have a lifetime risk of developing melanoma estimated to range from 0% to 5%. Secondary benign melanocytic proliferations commonly arise in congenital melanocytic nevi; however, some are difficult to definitively distinguish from malignant melanoma based on clinical features and conventional histology. Herein, we describe the use of comparative genomic hybridization in supporting the diagnosis of a deep penetrating nevus developing within a congenital melanocytic nevus of a 3-month-old infant.

Severe varicella caused by varicella-vaccine strain in a child with significant T-cell dysfunction.

In March 1995, the US Food and Drug Administration approved a live attenuated varicella vaccine for use in healthy children 12 months to 12 years old. We report here an 18-month-old girl with cell-mediated immunodeficiency who developed a severe vaccine-associated rash and clinical evidence of vaccine-associated pneumonia 1 month after inadvertent receipt of varicella vaccine.

Confluent and reticulated papillomatosis associated with tinea versicolor in three siblings.

We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and reticulated papillomatosis, and the only report describing family members having both confluent and reticulated papillomatosis and tinea versicolor. This report lends further evidence to the hypothesis that confluent and reticulated papillomatosis may be etiologically linked to tinea versicolor, and also suggests a genetic predisposition for it.

Genetic association of cutaneous neonatal lupus with HLA class II and tumor necrosis factor alpha: implications for pathogenesis.

OBJECTIVE: Cutaneous neonatal lupus resembles subacute cutaneous lupus erythematosus (SCLE), and photosensitivity is a common symptom. Tumor necrosis factor alpha (TNFalpha) release by ultraviolet light-exposed keratinocytes may be exaggerated in SCLE patients who have the haplotype TNFalpha -308A;DRB1*03. Accordingly, this study was undertaken to seek genetic and histologic evidence for a role of TNFalpha in the pathogenesis of cutaneous neonatal lupus. METHODS: DNA was isolated from 83 children (22 with rash, 35 with congenital heart block [CHB], 26 unaffected siblings) and 58 mothers from the Research Registry for Neonatal Lupus. RESULTS: The -308A allele (associated with higher TNFalpha production), HLA-DRQB1*02, and HLA-DRB1*03 were each present in the majority of children with rash (64%, 68%, and 64%, respectively). The frequency of all 3 6p alleles occurring together in 1 individual was greater in children with rash than in children who had either CHB or no manifestation of neonatal lupus (59% versus 30%; P = 0.02). This association with neonatal lupus rash was equivalent to published findings in a cohort of patients with SCLE, but significantly greater than the association in patients with discoid lupus erythematosus. Prominent TNFalpha staining in the epidermis was observed in lesional skin from 3 children with rash, but not in skin from a healthy neonate. CONCLUSION: Taken together, the finding of a genetic predisposition to generate increased levels of TNFalpha following tissue injury and the histologic demonstration of TNFalpha in the target organ support the notion that this inflammatory cytokine plays a role in the pathogenesis of cutaneous neonatal lupus. Furthermore, the results of these studies provide evidence of a biologic link between neonatal lupus and the rash of SCLE.

Infantile granular parakeratosis: recognition of two clinical patterns.

Granular parakeratosis is an acquired, idiopathic disorder of keratinization typified by retention hyperkeratosis. It usually occurs in women at intertriginous sites. There have been only 2 reports of infants with granular parakeratosis to our knowledge. We describe 3 additional infants with granular parakeratosis. We demonstrate that infantile granular parakeratosis exhibits 2 clinical patterns: bilateral linear plaques in the inguinal folds; and erythematous geometric plaques underlying pressure points from the diaper. A thick, flakelike scale is present in both forms and is characteristic. Diaper wearing appears to play an important role in the genesis of infantile granular parakeratosis but the mechanisms are unclear. Therapeutic responsiveness to topical agents is ambiguous, however, spontaneous clearance after months to 1 year appears to be the rule.

Infantile systemic hyalinosis.

Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.

Congenital primary cutaneous rhabdomyosarcoma in a neonate.

We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2-week-old infant boy. Rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of "small round cell tumors." Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle-specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long-term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6-month follow-up.

Updated classification of hemangiomas and other vascular anomalies.

Vascular anomalies comprise a widely heterogenous group of tumors and malformations. Great confusion has arisen because of the term hemangioma has been and is continued to be used to represent a multitude of vascular entities. This review presents the updated classification of vascular anomalies with the goal of clarifying the term hemangioma. In addition, newer clinical concepts in hemangiomas and other vascular tumors is presented. Hemangioma subtypes and hemangioma variants are also discussed, and a brief review of pyogenic granuloma and Kaposiform hemangioendothelioma is provided. Finally, the immunohistochemical marker GLUT1 is reviewed, a marker that heralds a new era in vascular anomalies research.