Phylloid terminal hair nevus: A unique clinical entity.

We present a case of an otherwise healthy infant with a localized patch of phylloid hypopigmentation bordered by terminal hairs on the back. We believe that this is a unique clinical entity and propose the term "phylloid terminal hair nevus." We believe that this is a localized form of phylloid hypomelanosis that is not associated with extracutaneous abnormalities.

Feed and Wrap MRI Technique in Infants.

The feed and wrap technique refers to the use of feeding and swaddling to induce natural sleep in infants. It can be used prior to an magnetic resonance imaging (MRI) scan, avoiding sedation or anesthesia. We performed a retrospective review of feed and wrap MRI scans in infants 3 months or younger over a 2-year period at our center (279 scans) to evaluate the efficacy of this technique. Of scan results reviewed, 79% addressed the clinical question, 20% partially addressed the clinical question, and 1% were technically inadequate. History of preterm birth (odds ratio [OR] = 2.368; P = .032) and spine MRI (OR = 2.821; P = .001) were associated with a less-successful scan outcome. The feed and wrap technique can be used successfully in infants undergoing MRI; however, it may be less successful in preterm infants and those requiring spinal MRI. A standardized technique performed by experienced personnel may avoid anesthesia and sedation in infants who require MRI.

Malignant Melanoma With Rhabdomyosarcomatous Differentiation: A Case Report.

Malignant melanoma may exhibit morphologic characteristics of nonmelanocytic cell or tissue components, a phenomenon termed divergent differentiation. Melanoma with rhabdomyosarcomatous differentiation is rare, with 6 definite cases in adults reported in the literature. The authors describe a 75-year-old man with a cutaneous lesion of the right ear initially diagnosed as malignant melanoma. Three months later, biopsy of a right cervical lymph node showed changes suggestive of rhabdomyosarcoma. Reexamination of the initial skin biopsy with muscle markers confirmed a diagnosis of malignant melanoma with rhabdomyosarcomatous differentiation. This case serves to highlight the diagnostic challenges associated with this rare subtype of melanoma.

Orbital Hemangioma with Intracranial Vascular Anomalies and Hemangiomas: A New Presentation of PHACE Syndrome?

We present two cases of infants with a similar constellation of clinical findings: retro-orbital infantile hemangioma (IH), internal carotid artery (ICA) arteriopathy, and intracranial IH. In both cases, intracranial vascular anomalies and hemangiomas were found incidentally during evaluation of unilateral proptosis. Neither infant had evidence of cutaneous segmental IH of the face or neck, which might have provided a clue to the diagnosis of PHACE syndrome or of intracranial hemangiomas. In one case, intracranial involvement was particularly extensive and function threatening, with mass effect on the brain parenchyma. These cases serve to highlight the fact that clinical findings of proptosis, globe deviation, and strabismus should prompt immediate imaging to confirm the presence of orbital IHs and to exclude other diagnoses. Moreover, based on our cases and the embryologic origin of the orbit as a unique developmental unit, patients with confirmed retro-orbital IHs should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IHs and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.

High prevalence of mupirocin resistance in Staphylococcus aureus isolates from a pediatric population.

Topical mupirocin is used widely to treat skin and soft tissue infections and to eradicate nasal carriage of methicillin-resistant Staphylococcus aureus (MRSA). Few studies to date have characterized the rates of S. aureus mupirocin resistance in pediatric populations. We retrospectively studied 358 unique S. aureus isolates obtained from 249 children seen in a predominantly outpatient setting by the Division of Pediatric Dermatology at a major academic center in New York City between 1 May 2012 and 17 September 2013. Mupirocin resistance rates and the associated risk factors were determined using a logistic regression analysis. In our patient population, 19.3% of patients had mupirocin-resistant S. aureus isolates at the time of their first culture, and 22.1% of patients with S. aureus infection had a mupirocin-resistant isolate at some time during the study period. Overall, 31.3% of all S. aureus isolates collected during the study period were resistant to mupirocin. Prior mupirocin use was strongly correlated (odds ratio [OR] = 26.5; P = <0.001) with mupirocin resistance. Additional risk factors for mupirocin resistance included methicillin resistance, atopic dermatitis (AD), epidermolysis bullosa (EB), immunosuppression, and residence in northern Manhattan and the Bronx. Resistance to mupirocin is widespread in children with dermatologic complaints in the New York City area, and given the strong association with mupirocin exposure, it is likely that mupirocin use contributes to the increased resistance. Routine mupirocin testing may be important for MRSA decolonization strategies or the treatment of minor skin infections in children.

Pityriasis Lichenoides in Childhood: Review of Clinical Presentation and Treatment Options.

Pityriasis lichenoides (PL) is a skin condition of unclear etiology that occurs not uncommonly in childhood. It is often classified into the acute form, pityriasis lichenoides et varioliformis acuta (PLEVA), and the chronic form, pityriasis lichenoides chronica (PLC). We performed a comprehensive review of the English-language literature using the PubMed database of all cases of childhood PL reported from 1962 to 2014 and summarized the epidemiology, clinical features, treatment options, and prognosis of this condition in children. The proposed etiologies are discussed, including its association with infectious agents, medications, and immunizations and evidence for PL as a lymphoproliferative disorder. We found an average age of PL onset of 6.5 years, with a slight (61%) male predominance. We also found that PLEVA and PLC tend to occur with equal frequency and that, in many cases, there is clinical and histopathologic overlap between the two phenotypes. When systemic therapy is indicated, we propose that oral erythromycin and narrowband ultraviolet B phototherapy should be first-line treatment options for children with PL since they have been shown to be effective and well tolerated. In most cases, PL follows a benign course with no greater risk of cutaneous T-cell lymphoma, although given the rare case reports of transformation, long-term follow-up of these patients is recommended.

Early-onset heart failure, alopecia, and cutaneous abnormalities associated with a novel compound heterozygous mutation in desmoplakin.

Mutations in the desmosomal protein desmoplakin have been associated with various conditions affecting the skin and heart. The prototype is Carvajal syndrome, characterized by cardiomyopathy, woolly hair, palmoplantar keratoderma (PPK), and skin fragility. We report the case of a 3-year-old boy presenting with severe left-sided heart failure with a preceding history of cutaneous abnormalities including congenital alopecia, PPK, nail dystrophy, and follicular hyperkeratosis on the extensor surfaces. Genetic testing revealed a novel combination of two heterozygous mutations in the DSP gene encoding desmoplakin: R1400X and R2284X. Both are predicted to be deleterious to protein function. This case adds to our understanding of the spectrum of clinical presentations of syndromes associated with desmoplakin mutations and highlights the need for cardiac examination in patients with characteristic cutaneous findings.