Evolving Landscape of Biologic Therapy for Pediatric Psoriasis.

Pediatric psoriasis is a chronic inflammatory skin condition. Current treatment modalities include topical medications, phototherapy, and systemic drugs, including biological agents. In cases of moderate-to-severe psoriasis recalcitrant to other therapies, biological therapies are often an attractive option given their dosing schedules, safety profiles, and need for less frequent laboratory monitoring, when compared with traditional systemic therapies. This article reviews biological treatment options approved for pediatric psoriasis and identifies others actively under investigation.

Acral Fibrochondromyxoid Tumor Presenting as Enlarging Nodule Involving the Distal Fingertip and Hyponychium: A Case Report.

An acral fibrochondromyxoid tumor is a newly described type of benign soft tissue neoplasm that presents as a single nodular lesion on a finger or toe. There has only been one previous report on this tumor, a case series that described the initial pathologic and clinical findings; however, details on clinical history, physical examination, and outcome are unknown. In this report, we describe a case of a 39-year-old male who presented with a painful enlarging mass involving the distal right 3rd finger and hyponychium. Punch biopsy was performed and the lesion was identified as an acral fibrochondromyxoid tumor on microscopic examination. X-ray showed no bony involvement. The tumor was successfully excised with complete resolution of pain symptoms. We discuss the clinical features and immunohistochemistry findings of our case in the context of the current limited knowledge about this very rare tumor.

Head Lice.

Head lice infestation is associated with limited morbidity but causes a high level of anxiety among caregivers of school-aged children and adolescents. Since the 2015 clinical report on head lice was published by the American Academy of Pediatrics, new medications have been approved, and an algorithm for management of affected patients is included. This revised clinical report clarifies current diagnosis and treatment protocols.

A dermatologic perspective on multisystem inflammatory syndrome in children.

As of May 2020, an emerging immune-mediated syndrome primarily affecting children has been detected primarily in Europe and the United States. The incidence of this syndrome appears to mirror the initial infectious assault with a delay of several weeks. This syndrome has been termed "multisystem inflammatory syndrome in children" (MIS-C) and is observed in association with the coronavirus disease 2019. The phenotypes of presentation include several characteristic features, including prolonged fever, skin eruptions, neck stiffness, and gastrointestinal manifestations with pronounced abdominal pain. Shock and organ dysfunction on presentation are frequent but inconsistent, whereas respiratory distress is typically, and notably, absent. We have reviewed the recent published data aiming to better understand MIS-C, with a focus on its mucocutaneous manifestations.

A dermatologic perspective on multisystem inflammatory syndrome in children.

As of May 2020, an emerging immune-mediated syndrome mainly affecting children has been detected primarily in Europe and the United States. The incidence of this syndrome appears to mirror the initial infectious assault, with a delay of several weeks. This syndrome has been termed "multisystem inflammatory syndrome in children" (MIS-C) and is observed in association with the coronavirus disease 2019 (COVID-19). The phenotypes of presentation include several characteristic features, including prolonged fever, skin eruption, neck stiffness, and gastrointestinal manifestations with pronounced abdominal pain. Shock and organ dysfunction on presentation are frequent but inconsistent, whereas respiratory distress is typically and notably absent. We have reviewed recently published data aiming to better understand MIS-C, with a focus on its mucocutaneous manifestations.

Topical corticosteroid use for atopic dermatitis in the pediatric emergency department.

BACKGROUND/OBJECTIVES: To investigate the evaluation and management of atopic dermatitis (AD) in the pediatric emergency department (PED). METHODS: This retrospective chart review was performed at the PED of a single institution and examined data from 2012 to 2017. Of 335 visits from patients 18 years and younger coded for AD, 167 visits with documented findings that supported a diagnosis of AD according to guidelines from the American Academy of Dermatology were included. RESULTS: The mean age of presentation was 6.3 years (standard deviation [SD]: 5.9). Of 11 patients with multiple visits, the mean between-visit interval was 31 days (SD: 41). Topical corticosteroids (TCSs) were not prescribed or recommended in 63/167 visits. In an additional 46/167 visits, over-the-counter topical hydrocortisone was recommended. Of prescribed TCS, the mean TCS class was 5.5 (SD: 1.9). 61/104 recommended or prescribed TCSs were weak (Class 7), the most likely used class (P < .001). Dermatology consultation was requested in 14/167 visits and was associated with higher rates of TCS prescriptions (13/14 vs 91/153, P = .018), a higher mean class of TCS prescribed (3.1 vs 5.9, P < .001), higher prescription rates of systemic antibiotics (8/14 vs 10/153, P < .001), and higher recommendation rates for emollient usage (10/14 vs 46/153, P = .005). CONCLUSIONS: Most patients presenting to the PED for AD were either not prescribed a TCS or were prescribed a weak TCS, often one that is over-the-counter. While there may be a variety of explanations for these findings, it is possible they reveal a practice gap regarding AD management in the PED.

The use of respiratory pathogen panel nasal polymerase chain reaction testing in predicting cutaneous enteroviral infections in the pediatric population.

BACKGROUND/OBJECTIVE: To characterize the relationship between the presence of enteroviral skin infection, defined as a positive skin polymerase chain reaction (PCR) test, and the nasopharyngeal (NP) respiratory pathogen panel (RPP) PCR test which includes enterovirus/rhinovirus as an analyte. METHODS: A retrospective chart review was performed on 543 subjects, age 18 years or younger, who had enterovirus (EV) skin swabs performed at an academic medical center in New York City between September 2014 and November 2019. Those patients with positive EV skin PCR were considered to have an enteroviral skin infection, and those with a negative EV skin PCR were considered not to have an enteroviral skin infection. Of those 543 children who had EV skin PCR, 170 also had an NP swab RPP performed. These NP swab RPP results were characterized as positive or negative, and if positive, it was noted if the patient was positive or negative for enterovirus/rhinovirus. The positive predictive value (PPV), negative predictive value (NPV), specificity, and sensitivity of a NP swab RPP for enteroviral skin infection were then calculated. RESULTS: An enterovirus/rhinovirus NP swab RPP had a NPV of 95%, PPV of 43%, sensitivity of 90%, and specificity of 62% for cutaneous enterovirus infection. CONCLUSION: The enteroviral skin PCR test is an assay that was validated at this institution. In clinically suspicious cases of EV, a positive NP swab RPP for enterovirus/rhinovirus is a sensitive test. A negative test is highly predictive of not having EV on the skin. Although further data are needed, given that NP swab RPP is readily available, these data may suggest that an NP swab RPP, when appropriately utilized, can support or exclude a clinical diagnosis of cutaneous enterovirus in the pediatric population.

Lichen sclerosus among women in the United States.

BACKGROUND: Vulvar lichen sclerosus (LS) is a chronic dermatosis for which the true prevalence is not well established. LS remains an underrecognized and undertreated disease, and treatment patterns and differences among various specialties that care for patients with LS are largely unknown. OBJECTIVE: This study sought to determine the prevalence of diagnosed vulvar LS, demographic characteristics, and diagnostic and treatment patterns in the United States. METHODS: A database comprising >21.7 million women with commercial insurance in the United States was analyzed, and the largest cohort of patients with LS in the United States to date was identified. RESULTS: We found that obstetricians/gynecologists are diagnosing and managing approximately half of women with LS, and regardless of specialty, the majority of providers are appropriately prescribing high-potency topical steroids. CONCLUSION: Our results suggest that LS may truly be underrecognized and underdiagnosed, especially in younger women.

Mycoplasma pneumoniae, more than a lung disease.

Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a recently described clinical entity and should be considered in children who present with oral (94% of patients), ocular (82% of patients), and urogenital lesions (63% of patients). MIRM was first described as a distinct clinical entity from Stevens Johnson syndrome/Toxic epidermal necrolysis (SJS)/(TEN) in 2015 [1]. As a new, uncommon diagnosis it frequently poses a diagnostic and therapeutic challenge for pediatricians and dermatologists. We report a case of MIRM in a previously healthy 15-year-old boy.

Cutaneous Crohn disease without gastrointestinal involvement in a 9-year-old boy.

Cutaneous Crohn disease (CCD) is a rare dermatologic manifestation of Crohn disease and is defined as noncaseating, granulomatous skin lesions noncontiguous with the gastrointestinal tract. It most commonly affects the skin of the legs, although genital CCD is the most common presentation in children. Diagnosis of CCD is made by a combination of clinical and histopathological findings. Therapeutic options include topical, intralesional, and systemic corticosteroids as well as topical and systemic immunosuppressants and immunomodulators. Surgical excision may be considered for refractory cases. We report CCD in a 9-year old boy with penile swelling, granulomatous cheilitis-like lesions, and perianal plaques.

Paraneoplastic Pemphigus in a 34-year-old.

Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Diagnosis of PNP requires direct immunofluorescence of perilesional skin and indirect immunofluorescence. Treatment of PNP is difficult and largely limited to glucocorticoids, steroid-sparing immunomodulators, rituximab and intravenous immunoglobulin (IVIG). Despite therapies, prognosis is poor. We report a case of paraneoplastic pemphigus in a 34-year old male with severe stomatitis and lichenplanus-like cutaneous lesions.

Fibrous Hamartoma of Infancy: A Firm Plaque Presenting with Hypertrichosis and Hyperhidrosis.

Fibrous hamartoma of infancy (FHI) is a rare, benign, soft tissue tumor. The lesion's clinical resemblance to more aggressive soft tissue tumors of childhood makes histologic diagnosis paramount. We report a unique presentation of a FHI associated with localized hypertrichosis and hyperhidrosis.

PHACE syndrome: a retrospective review of 23 patients.

We present 23 patients with PHACE syndrome showing similarities in our population with data that already exist while highlighting neurodevelopmental occurrences arising in a subset of these patients.

Effectiveness of transition from intravenous epoprostenol to oral/inhaled targeted pulmonary arterial hypertension therapy in pediatric idiopathic and familial pulmonary arterial hypertension.

Before 2001, intravenous epoprostenol was the only approved drug for patients with idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH) who were nonresponsive to high-dose calcium channel blockade. The investigators report transitioning select pediatric patients with IPAH or FPAH from intravenous epoprostenol to oral and/or inhaled agents for pulmonary arterial hypertension. A retrospective review was performed on all pediatric patients with IPAH or FPAH treated at Columbia University (1987 to 2008) who transitioned off epoprostenol to oral or inhaled drugs. Criteria for transition included functional class I or II, mean pulmonary arterial pressure <35 mm Hg, normal cardiac index, and age >6 years. Hemodynamic and clinical data were obtained at baseline (before epoprostenol), at peak epoprostenol dose, and after epoprostenol discontinuation. Fourteen of 104 pediatric patients with IPAH or FPAH transitioned off epoprostenol to oral or inhaled drugs from April 2003 to July 2008. Of the 14 subjects, 13 transitioned off epoprostenol successfully to oral or inhaled drugs. No significant changes in functional class, hemodynamics, or exercise data were seen after epoprostenol (mean follow-up duration 7.0 +/- 5.8 months) compared to peak epoprostenol, but further improvement was reported in World Health Organization functional class (p <0.005) after epoprostenol discontinuation. After successful epoprostenol discontinuation, 77% of patients were treated with endothelin receptor antagonists, 69% with phosphodiesterase-5 inhibitors, 38% with calcium channel blockers, and 8% inhaled iloprost. At the cut-off date (May 2009), there was 100% survival and 93% transition success. In conclusion, in carefully selected children with IPAH or FPAH initiated on intravenous epoprostenol before the availability of nonparenteral therapy, transition to oral or inhaled therapy for pulmonary arterial hypertension appears safe, with efficacy maintained when performed with close follow-up at a pulmonary hypertension specialty center.