Pediatric Primary Cutaneous Marginal Zone Lymphoma Treated With Doxycycline.

Primary cutaneous marginal zone lymphoma (PCMZL) is a cutaneous B-cell lymphoma that rarely occurs in children. We present a 13-year-old boy with multiple asymptomatic erythematous papules and nodules on the trunk and arms that were confirmed on biopsy to be PCMZL. He was treated with doxycycline and intralesional triamcinolone with improvement of lesions. This case supports the use of doxycycline for the treatment of pediatric PCMZL in patients with widespread involvement despite negative Borrelia serology. Multiple low-risk treatment modalities may be used in conjunction to clear disease in pediatric patients.

Treatment Options for Juvenile Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris represents a group of familial and acquired disorders of cornification that affect both adult and pediatric patients. Treatment options are difficult to assess through clinical trials, given the rarity of the disorder and its tendency for spontaneous remission. Case reports and case series are therefore the primary means of assessment. Because of the heterogeneity of the disease, there is no universal approach to treatment, and multiple agents may need to be trialed to achieve disease control. At present, topicals are used for most pediatric patients, though monotherapy with topicals is only effective for less severe disease. Despite concerns over their side-effect profiles, oral retinoids are generally accepted as a first-line systemic therapy. However, interleukin-17 inhibitors and ustekinumab, an interleukin-12 and interleukin-23 inhibitor, may soon become first-line systemic treatment as well, given their efficacy and relative safety in trials thus far. Ustekinumab, in particular, is emerging as a first-line agent for patients with pityriasis rubra pilaris with CARD14 gene variations. When these therapies fail, second-line and adjunctive therapies to consider include tumor necrosis factor-alpha inhibitors, methotrexate, and phototherapy. However, further investigation is necessary to assess the safety and efficacy of many of these agents in juvenile pityriasis rubra pilaris.

Infestations, Bites, and Insect Repellents.

Infestations and arthropod bite reactions in children are common reasons for presentation to pediatric health care providers. Infestations in children include head lice, scabies, and other mites. Fleas and bed bugs are common causes of bite reactions in children, and papular urticaria is a chronic, recurrent eruption resulting from delayed hypersensitivity to a variety of insect bites. Both infestations and bite reactions may result in severe pruritus with associated sleep disturbance and can be a source of significant distress for patients and families. In this review, we discuss infestations and bite reactions affecting pediatric patients, along with the approach to treatment and prevention of these conditions. [Pediatr Ann. 2020;49(3):e124-e131.].

Complementary and alternative therapy for pediatric acne: A review of botanical extracts, dietary interventions, and oral supplements.

Many supplements and products containing botanical extracts are marketed to patients for the treatment of acne vulgaris. Additionally, increasing attention has been paid to the role of diet in acne vulgaris. Studies on this topic including pediatric patients are limited, with variable efficacy data. Despite these limitations, knowledge of alternative therapies in pediatric acne vulgaris is often expected from pediatric dermatologists. Here we review available data on the efficacy of complementary and alternative medicines for treatment of acne in pediatric patients, focusing on topical, oral, and dietary modifications.

Use of antiviral medications in drug reaction with eosinophilia and systemic symptoms (DRESS): A case of infantile DRESS.

A 3-month-old girl with Sturge-Weber syndrome presented with a morbilliform rash, eosinophilia, and fulminant liver failure to our tertiary pediatric hospital. She was diagnosed with drug reaction with eosinophilia and systemic symptoms complicated by viremia and evidence of viral hepatitis on liver biopsy. We discuss the role of viral reactivation in drug reaction with eosinophilia and systemic symptoms and the relevance of antiviral therapy in management.

Restricted Range of Motion and a Cold Upper Extremity in a Two-Year-Old Boy: Kaposiform Hemangioendothelioma of the Bone and the Brachial Plexus: A Case Report.

CASE: We report a case of kaposiform hemangioendothelioma (KHE) of the scapula in a 2-year-old boy with motor and sensory abnormalities of the left upper extremity, suggesting brachial plexus involvement. The locally invasive nature prevented resection; sirolimus therapy resulted in improvement of the motor and sensory impairment, as well as decreased tumor size on imaging. CONCLUSION: Osseous infiltration of KHE is known to occur, but its primary presentation in bone without skin involvement is rare and diagnostically challenging. Awareness of rare presentations of KHE, along with accurate histopathologic interpretation, is important to achieve a diagnosis and to differentiate KHE from more common vascular lesions (e.g., infantile hemangioma). Sirolimus therapy is emerging as a promising treatment for unresectable KHE.

Current Data on Risk Factor Estimates Does Not Explain the Difference in Rates of Melanoma between Hispanics and Non-Hispanic Whites.

United States Hispanics have seven times lower melanoma incidence rates than non-Hispanic whites (NHW). It is unclear whether this difference can be explained solely by phenotypic risk factors, like darker skin, or whether modifiable risk factors, like sun exposure, also play a role. The purpose of this paper is to summarize what is currently known about melanoma risk factors among Hispanics and NHWs, and whether or not those differences could explain the difference in melanoma incidence. Through literature review, relative risks and prevalence of melanoma risk factors in Hispanics and NHWs were identified and used to calculate the expected rate in Hispanics and rate ratio compared to NHWs. We found that melanoma risk factors either have similar frequency in Hispanics and NHWs (e.g., many large nevi) or are less frequent in Hispanics but do not explain a high proportion of disease variation (e.g., red hair). Considering current knowledge of risk factor prevalence, we found that melanoma incidence rates in the two groups should actually be similar. Sun exposure behavior among Hispanics may contribute to the explanation for the 7-fold difference in melanoma rates. Currently, limited data exist on sun exposure behavior among Hispanics, but possibilities for improving primary prevention by further studying these practices are substantial.

Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder that ranges from single-system to disseminated multisystem disease. Patients younger than 24 months of age more commonly present with risk organ (liver, spleen, hematopoietic system, or lung) involvement at diagnosis and have a poor prognosis. Treatment approaches have changed over the last 25 years. Our goal was to describe the course and outcomes of patients younger than 24 months of age at diagnosis and identify the role of bone involvement in outcomes. METHODS: We conducted a retrospective chart review of patients diagnosed with LCH at Children's Hospital Los Angeles from 1984 to 2010, focusing on 71 patients younger than 24 months of age at diagnosis. RESULTS: Ten patients had single bone lesions at diagnosis and did well irrespective of therapy. The majority of patients (40/71 or 56%) had multiple bone lesions. Of the 37 patients with multisystem disease, 27 children (73%) had risk organ involvement. Fourteen patients with risk organ involvement received ≤ 6 months of initial chemotherapy with prednisone and vinblastine. Six of these patients had reactivation of the disease, and bone was the most frequent site of reactivation. Seven patients with risk organ involvement were treated with at least 12 months of chemotherapy. Only one of these patients had reactivation of the disease, and none died. The majority (7/10) of patients with risk organ involvement who progressed on therapy died despite multiple treatment regimens. CONCLUSIONS: Patients younger than 24 months of age at diagnosis are more likely to have multiple bone lesions than older patients, supporting that a radiographic skeletal survey at the time of LCH diagnosis is important to evaluate the extent of bone involvement. Bones were the most common site for reactivation for all patients. As expected, subjects with risk organ involvement had better outcomes when treated with systemic chemotherapy for at least 1 year. LEVEL OF EVIDENCE: As a retrospective review of all cases at our institution over a 26-year period, this article represents level IV evidence.

Recognizing and managing the immunologic reactions in leprosy.

Immunologic reactions are an important aspect of leprosy that significantly impacts the course of the disease and the associated disability. Reversal reaction (type 1), erythema nodosum leprosum (type 2), and Lucio phenomenon are the 3 leprosy reactions, and they are most commonly seen in patients with the lepromatous and borderline categories of the disease. Because these forms of leprosy are the most common types seen in the United States, it is particularly important for physicians to be able to recognize and treat them. The reactions may occur before, during, or after treatment with multidrug therapy. Reversal reactions are the most common cause of nerve damage in leprosy, and erythema nodosum leprosum may also lead to neuritis. Although there have not been enough studies to confirm the most effective management regimens, treatment of reversal reaction and Lucio phenomenon with prednisone and of erythema nodosum leprosum with thalidomide and/or prednisone may help improve symptoms and prevent further disability.