Cutaneous findings in Fanconi anemia.

BACKGROUND: Fanconi anemia (FA) is a genetic disorder that results in bone marrow failure, physical abnormalities, and solid organ malignancies. The diagnosis of FA is often delayed because the early disease characteristics have not been well established. OBJECTIVE: To outline the spectrum of cutaneous findings seen in patients with FA. METHODS: A cross-sectional study in which patients with FA received a full-body skin examination. Patient characteristics are summarized with mean (SD) for continuous and count (%) for categorical variables. Poisson regression and logistic regression models were used to examine the relationships between pigmentary changes and patient characteristics. RESULTS: At least 1 cutaneous pigmentary alteration was present in 96.8% of patients, most arising before the teenage years. The most common finding was café-au-lait macules. Other findings included hypopigmented macules, skin-fold freckle-like macules, extensive sun-exposed freckling, and both hypopigmented and hyperpigmented pigment macules. LIMITATIONS: Patients received a single assessment, so the number of pigmentary changes could not be assessed over time. CONCLUSIONS: Characteristic morphology of FA includes faint and ill-defined café-au-lait macules, hypopigmented skin-fold freckle-like macules and the concurrence of hypopigmented and hyperpigmented macules. The recognition of these findings could aid clinicians in making earlier diagnoses.

Facial hypopigmentation in skin of color: An atypical presentation of lichen nitidus.

Lichen nitidus is a benign skin condition of unknown etiology that is classically described on the trunk, extremities, and genitalia as pinpoint flat-topped papules. In dark-skinned persons, the lesions may appear shiny or even hypopigmented. Lichen nitidus is less often described on the face. We describe a series of pediatric patients with skin of color who presented with the chief complaint of facial skin lightening and had associated clinical findings consistent with lichen nitidus.

Examination of use and barriers for five sun protection strategies in parents and their children.

BACKGROUND/OBJECTIVES: Sun protection starting in childhood is an important means of skin cancer prevention. Factors associated with sunscreen use have been previously described. However, less is known about factors associated with children's utilization of non-sunscreen sun protection strategies. We sought to examine parent and child characteristics, parental use of sun protection, and barriers associated with children's use of five sun protection strategies: sunscreen, shirts with sleeves, wide-brimmed hats, sunglasses, and shade. METHODS: A cross-sectional survey of parents of children entering kindergarten through grade 8. Survey participants were recruited at the 2016 Minnesota State Fair. RESULTS: A total of 409 parents were surveyed. The most common sun protection strategies parents reported for their child were wearing a shirt with sleeves (69.9%) and using sunscreen (61.9%), while protection via shade, wide-brimmed hats, and sunglasses were reported by less than one-quarter of parents. For each individual strategy, parents' own use of that strategy was strongly associated with that strategy in their children. Lack of child cooperation was associated with decreased use of sunscreen, shirts with sleeves, and wide-brimmed hats. CONCLUSION: Significant room for improvement exists in childhood sun protection. Parents should especially be encouraged to use shade, wide-brimmed hats, and sunglasses as methods of protection for their children. Advising parents to model seeking shade, applying sunscreen, and choosing appropriate clothing and hats may improve sun safety practices.

Methotrexate treatment in a case of juvenile pityriasis rubra pilaris.

An 8-year-old boy who was initially diagnosed with plaque psoriasis failed management with topical therapies and skin biopsy confirmed the suspected diagnosis of juvenile pityriasis rubra pilaris (PRP). Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma. Treatment modalities include topical and systemic therapies, although previous studies have not shown much benefit with methotrexate in children. We present a case in which methotrexate led to significant improvement of the skin findings in a child with type IV juvenile pityriasis rubra pilaris.

Perceptions and Practices Regarding Atopic Dermatitis: A Survey.

Parents (N = 392) completed a survey at the Minnesota State Fair in August and September 2015 addressing knowledge regarding the pathogenesis of atopic dermatitis (AD), basic skin care practices, and the perceived role of infection and food allergies in AD. Of participating parents, 82% identified food allergy or sensitivity in AD pathogenesis, whereas only 42% identified bacterial infection as an important factor in AD severity. Participants most often received education on skin health from their pediatrician (49%), followed by Internet-based sources (23%), rather than a dermatologist or pediatric dermatologist. Parents of children with AD overestimate the role of food allergies in this condition and are not as aware of the role of bacterial infection. Future educational directions from our specialty should focus on resources for pediatricians and families that emphasize these factors.

Transplacental Transmission of Pemphigus Herpetiformis in the Setting of Maternal Lymphoma.

Pemphigus herpetiformis (PH) is characterized by grouped vesicular or papular pruritic lesions with histologic and immunopathologic features of pemphigus. PH can manifest at any age, and paraneoplastic cases have been reported. We describe a healthy boy born with acral crateriform erosions of the hands and feet whose 35-year-old mother had similar lesions. Biopsies from both patients were most consistent with PH. The mother was diagnosed with high-grade B-cell non-Hodgkin lymphoma and began chemotherapy with dexamethasone, and her lesions quickly improved. The infant had no additional lesions after 3 weeks of age and his acral erosions healed. To our knowledge, this is the first report of a diagnosis of PH in an infant and the first case of transplacental transmission of PH. This is also the first report of paraneoplastic PH in lymphoma. This case adds to the growing differential diagnosis of skin disease in postpartum women and their neonates.

Local vaccine site reactions and contact allergy to aluminum.

Childhood vaccines are a routine part of pediatric care in the United States; clinicians must be able to recognize and interpret associated localized adverse reactions. Redness and induration at the site of injection are commonly reported and are considered to be the result of local inflammation or hematoma formation, although other atypical reactions can occur. We report the case of a 6-month-old infant who developed subcutaneous nodules at the sites of his 4- and 6-month Pentacel (DTaP/Hib/IPV, diphtheria, tetanus, acellular pertussis, Haemophilus b conjugate, and inactivated poliovirus vaccine) and 6-month Prevnar (heptavalent pneumococcal vaccine) injections. Infectious disease and immunodeficiency examinations were unremarkable. Aluminum contact allergy was considered, and contact allergy testing confirmed sensitivity to aluminum. Although rare, aluminum contact allergy after routine immunization can occur and should be considered in the differential diagnosis of persistent subcutaneous nodules after vaccination.

Panniculitis in childhood.

Panniculitis refers to disorders with inflammation of the subcutaneous fat. Such inflammation can be primary or can be a reaction pattern induced by a systemic process. Some types of panniculitis are seen more commonly or exclusively in children. These include erythema nodosum, subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, and cold panniculitis. The most typical clinical finding is tender, erythematous subcutaneous nodules. Clinical clues can aid in the diagnosis of the panniculitides, but pathology is often necessary to confirm the diagnosis. In general, the pediatric panniculitides are treated with supportive care and management of any underlying disorders, but certain types such as infectious panniculitis and malignancy-related panniculitis require more specific therapies.

Morphology-Based Diagnosis of Acneiform Eruptions.

Acne is the most common skin disease. Distinguishing between true acne vulgaris and the various acneiform eruptions is important yet sometimes challenging. Given the common nature of acne and acneiform eruptions, the pediatrician must be aware of these lesion patterns and possess the skills to effectively evaluate the pediatric presentation of these eruptions. This article discusses several of the most common acneiform eruptions, including neonatal acne and cephalic pustulosis, periorificial dermatitis (perioral dermatitis), facial angiofibromas, iatrogenic acneiform drug eruptions, and childhood rosacea.