Chiu, Yvonne E

AbstractA 4‐month‐old male presented for a large, hypertrichotic brown patch on the upper back with several scattered 0.5–1.5 cm, round to oval, brown macules and patches on the trunk and extremities. The lesion was initially diagnosed as a giant congenital melanocytic nevus based on clinical exam and histopathology with immunohistochemical stains. The patient was later diagnosed with neurofibromatosis type 1, and the lesion on the back developed a “bag of worms” texture consistent with a plexiform neurofibroma and found to harbor a pathogenic variant in the NF1 gene. This case highlights the diagnostic challenge of differentiating these lesions and their overlapping clinical and histopathological features.

AbstractSolid organ and hematopoietic stem cell transplantation may be complicated by the development of post‐transplant lymphoproliferative disorders (PTLDs). The World Health Organization categorizes PTLDs into four entities including non‐destructive, monomorphic, polymorphic, and classical Hodgkin lymphoma types. The most common PTLDs are B‐cell lymphomas, with T‐cell lymphomas accounting for only a few cases. Cutaneous T‐cell lymphomas are rarer still in post‐transplant patients with primary cutaneous peripheral T‐cell lymphoma being an extraordinarily rare subtype in this population. PTLDs may be aggressive and are often associated with high morbidity and mortality. Advances in medicine have led to increased awareness of PTLDs and improved diagnostic tools which assist in the diagnosis of these conditions. However, the clinical and histopathologic heterogeneity of PTLDs may make diagnosis a challenge. In the transplant patient population, the cutaneous manifestations of the lymphoproliferative disease may mimic other conditions, such as eczematous dermatitis and graft‐vs‐host disease. Herein, we report a case of post‐transplant primary cutaneous peripheral T‐cell lymphoma not otherwise specified (PTCL‐NOS) in a pediatric heart transplant patient and describe the clinical presentation and diagnostic histopathologic features.

AbstractBackground/ObjectivesThe COVID‐19 pandemic has raised questions about the approach to management of systemic immunosuppressive therapies for dermatologic indications in children. Change to: Given the absence of data to address concerns related to SARS‐CoV‐2 infection and systemic immunosuppressive therapies in an evidence‐based manner, a Pediatric Dermatology COVID‐19 Response Task Force (PDCRTF) was assembled to offer time‐sensitive guidance for clinicians.MethodsA survey was distributed to an expert panel of 37 pediatric dermatologists on the PDCRTF to assess expert opinion and current practice related to three primary domains of systemic therapy: initiation, continuation, and laboratory monitoring.ResultsNearly all respondents (97%) reported that the COVID‐19 pandemic had impacted their decision to initiate immunosuppressive medications. The majority of pediatric dermatologists (87%) reported that they were pausing or reducing the frequency of laboratory monitoring for certain immunosuppressive medications. In asymptomatic patients, continuing therapy was the most popular choice across all medications queried. The majority agreed that patients on immunosuppressive medications who have a household exposure to COVID‐19 or test positive for new infection should temporarily discontinue systemic and biologic medications, with the exception of systemic steroids, which may require tapering.ConclusionsThe ultimate decision regarding initiation, continuation, and laboratory monitoring of immunosuppressive therapy during the pandemic requires careful deliberation, consideration of the little evidence available, and discussion with families. Consideration of an individual's adherence to COVID‐19 preventive measures, risk of exposure, and the potential severity if infected must be weighed against the dermatological disease, medication, and risks to the patient of tapering or discontinuing therapies.

AbstractPHACE(S) syndrome is a condition characterized by posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe. We present four children with PHACE(S) syndrome who have absence of or severe malformation of the roots of their permanent first molars (PFMs). Root abnormalities in the children's molars were bilateral and not restricted to the segments affected by cutaneous hemangioma. The reason for root abnormalities is unknown, but given the rarity of these findings in healthy children, it is likely an additional dental manifestation of PHACE syndrome. The absence of functional roots in the PFMs can result in significant consequences. Therefore, we recommend a panoramic dental radiograph during transitional dentition for children with PHACE syndrome to screen for dental root abnormalities.

AbstractBackgroundErythema multiforme (EM) is an acute condition characterized by distinctive target lesions of the skin often accompanied by mucosal ulcers. A subset of individuals experience frequent episodes of recurrent EM, which is rare and poorly understood, especially in children.ObjectiveTo characterize clinical features, laboratory findings, and treatment responses of pediatric recurrent EM.MethodsA retrospective chart review was conducted at the Children's Hospital of Wisconsin in Milwaukee, Wisconsin (2000‐2015) and the Mayo Clinic in Rochester, Minnesota (1990‐2015). Inclusion criterion was a diagnosis before age 18 years with recurrent EM, defined as a symmetrically distributed, fixed eruption, including target lesions, with or without mucous membrane involvement, occurring on at least three occasions. A literature review was conducted to include individuals who met the inclusion criterion.ResultsTwenty‐six patients were included, of whom 16 (62%) were male. The median age of onset was 9.1 years (range 0‐15.7 years). Nine patients (35%) required hospitalization. Herpes simplex virus testing was positive in 9 of 17 (65%) patients. Remission was achieved in 5 of 16 (31%) patients while taking suppressive antivirals. Eight patients received continuous anti‐inflammatory treatment, two (25%) of whom experienced remission.ConclusionThis study of pediatric recurrent EM found a greater male predominance, more hospitalizations, fewer cases caused by herpes simplex virus, and a lower response to immunosuppression in children than in the general population.

AbstractBackground/ObjectivesStudies describing treatment efficacy in pediatric morphea are lacking. Subspecialists have reached no consensus on how to optimally treat pediatric morphea. The objective of the current study was to describe the most common treatment practices of pediatric dermatologists worldwide who care for children with morphea.MethodsA survey regarding topical treatment practices of pediatric morphea, with representative case‐based scenarios, was distributed to pediatric dermatologists and results were tallied.ResultsThe survey response rate was 13.4%, with 110 respondents in the final analysis. The majority of respondents agreed on red violaceous rim (99%), increased local warmth (75%), raised borders (69%), and dermal thickening (64%) as signs of disease activity. Respondents had less agreement on sclerotic lesions (41%), scaling (43%), dyspigmentation (19%), and atrophy (13%) as signs of disease activity. Ninety‐two percent of respondents used primary therapy or monotherapy with topical medications, including 45% in linear morphea of the limbs and 37% in linear morphea of the head or neck. High‐potency topical corticosteroids were most commonly used (80%), although respondents did not agree on specific regimens. Sixteen different treatment regimens were selected as first‐line therapy for one case scenario of active disease.ConclusionThe survey found large variation in how pediatric dermatologists treat pediatric morphea. Consensus treatment guidelines developed by pediatric dermatologists and pediatric rheumatologists are urgently needed regarding the efficacy of therapies for pediatric morphea. Prospective studies of treatment efficacy in pediatric morphea are urgently needed as well.

AbstractBackground/ObjectivesUrticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life.MethodsBetween 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI). Research visits were conducted every 3 years and telephone interviews yearly. The Children's Dermatology Life Quality Index was administered to subjects 4 years of age and older at enrollment. Laboratory test results were collected for subjects younger than 4 years at enrollment. Subjects were followed until UP resolution or study end in August 2015.ResultsThe final cohort size was 43 subjects followed for a median of 8.1 years. Twenty‐six subjects were followed through study completion. At age 12 years, 6 patients had disease resolution and 14 remained active. Patients who had disease resolution before age 12 years were more likely to be male and had fewer years of age and smaller lesions, fewer affected areas, and earlier onset. Common medications and anesthetics resulted in no serious reactions. Hymenoptera stings occurred in 51%, with no reports of anaphylaxis. No patient reported a severe effect on quality of life, with most indicating mild to no effect.ConclusionSevere complications are not common with historically identified triggers. Disease does not resolve before adolescence in most children. UP has a minimal effect on quality of life for most children.