Publications by authors named "Yvonne E Chiu"

41 Publications

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

J Am Acad Dermatol 2021 Mar 6. Epub 2021 Mar 6.

Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Background: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized.

Objective: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations.

Methods: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software.

Results: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4).

Limitations: Retrospective nature and the inclusion of only patients with clinical photographs.

Conclusion: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.
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http://dx.doi.org/10.1016/j.jaad.2021.03.017DOI Listing
March 2021

Hidradenitis Suppurativa in the Pediatric Population: An International, Multicenter, Retrospective, Cross-sectional Study of 481 Pediatric Patients.

JAMA Dermatol 2021 Feb 24. Epub 2021 Feb 24.

Section of Dermatology, Division of Pediatric Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Canada.

Importance: Hidradenitis suppurativa (HS) in pediatric patients has been understudied. Increased awareness and recognition of HS prevalence in children demand efforts to better understand this condition.

Objective: To describe the demographics, clinical features, treatment, associated comorbidities, and outcomes in a large cohort of pediatric patients with HS.

Design, Setting, And Participants: International, multicenter, retrospective medical record review of pediatric patients (aged 1-18 years) with a clinical diagnosis of HS carried out in 10 dermatology clinics across the US, Canada, Israel, Australia, and Italy from January 1996 to January 2017.

Main Outcomes And Measures: Patient demographics, clinical features, severity, associated comorbidities, and treatments in pediatric patients with HS.

Results: This cross-sectional study included 481 patients diagnosed with HS. Overall, 386 (80%) were girls. The mean (SD) age of disease onset was 12.5 (2.9) years, and the mean (SD) age at diagnosis was 14.4 (3.5) years. Family history of HS was present in 111 of 271 (41%) patients. First signs/symptoms reported at disease onset were cyst/abscess in 229 of 481 (48%), pain/tenderness in 118 of 481 (25%), and papules/pustules in 117 of 481 (24%). At initial dermatologic assessment, 233 of 481 (48%) patients already had evidence of skin scarring. Disease severity (Hurley staging) was documented in 288 of 481 (60%) patients (47% stage 1, 45% stage 2 and 8% stage 3). Comorbid conditions were reported in 406 of 481 (85%) patients, the most common being obesity (263/406 [65%]) and acne vulgaris (118/406 [29%]). Complications occurred in 378 of 481 (79%) patients, the most common of which were scars or contractures (301/378 [80%]).

Conclusions And Relevance: The findings of this study indicate that there is a gap in recognizing and diagnosing pediatric HS. Pediatric patients with HS are likely to present with other comorbidities. Prospective observational and interventional studies are needed to better understand clinical course and optimal treatments for pediatric HS.
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http://dx.doi.org/10.1001/jamadermatol.2020.5435DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7905696PMC
February 2021

Post-transplant primary cutaneous peripheral T-cell lymphoma not otherwise specified in a pediatric patient.

J Cutan Pathol 2021 May 9;48(5):706-712. Epub 2021 Mar 9.

Department of Dermatology, Mayo Clinic, Jacksonville, Florida, USA.

Solid 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.
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http://dx.doi.org/10.1111/cup.13967DOI Listing
May 2021

Histopathologic Spectrum of Morphea.

Am J Dermatopathol 2021 Jan;43(1):1-8

Assistant Professor, Department of Pathology, Section of Pediatric Pathology, Medical College of Wisconsin, Milwaukee, WI and.

Abstract: Morphea is an autoimmune skin disease with protean clinical manifestations. Histologic features are similarly variable, and skin biopsies may be nondiagnostic. A single-institution retrospective cohort study was conducted. Morphea patients who had a biopsy in 2005-2015 were included, and a histopathological review was conducted by 2 pathologists. There were 51 biopsy specimens from 40 subjects. The most common histologic features were dermal sclerosis (90%), dermal thickening (78%), collagen homogenization (86%), a superficial and deep infiltrate (76%), a moderate-abundant inflammatory infiltrate (73%), and periadnexal fat loss/decreased skin appendages (71%). Twenty-four specimens were not diagnostic of morphea. In these specimens, the main clues to diagnosis included the presence of dermal sclerosis (79%), subtle collagen homogenization (75%), dermal thickening (58%), moderate-to-abundant plasma cells (50%), and perineural inflammation (50%). There were no statistically significant differences between active and inactive lesions, nor untreated and treated lesions. The histopathologic features of morphea are variable and a high proportion of biopsies are not diagnostic. Clinicians and pathologists should have a high degree of suspicion to correctly make the diagnosis of morphea.
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http://dx.doi.org/10.1097/DAD.0000000000001662DOI Listing
January 2021

Pediatric patients with orofacial granulomatosis likely to subsequently develop intestinal Crohn's disease: Brief Report.

Pediatr Dermatol 2020 Nov 28;37(6):1162-1164. Epub 2020 Sep 28.

Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition presenting as perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn's disease. Our retrospective review identified 7 patients diagnosed with OFG between 2000 and 2018 at a tertiary pediatric hospital. Four of the 7 patients subsequently developed Crohn's disease with a median delay of 3.1 years (range 0.4-6.9 years). This indicates that gastroenterology evaluation with long-term monitoring for intestinal Crohn's disease is warranted.
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http://dx.doi.org/10.1111/pde.14390DOI Listing
November 2020

Anti-Ku antibody-positive systemic sclerosis-polymyositis overlap syndrome in an adolescent.

Pediatr Dermatol 2020 Sep 10;37(5):960-961. Epub 2020 Jun 10.

Departments of Dermatology (Pediatric Dermatology) and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Systemic sclerosis-polymyositis overlap syndrome is rare in children. Anti-PM/Scl is the most common autoantibody associated with this syndrome. We present a case of systemic sclerosis-polymyositis overlap syndrome in a child with isolated anti-Ku antibodies, an uncommon antibody associated with this rare syndrome.
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http://dx.doi.org/10.1111/pde.14243DOI Listing
September 2020

Systemic immunosuppressive therapy for inflammatory skin diseases in children: Expert consensus-based guidance for clinical decision-making during the COVID-19 pandemic.

Pediatr Dermatol 2020 May 16;37(3):424-434. Epub 2020 May 16.

Department of Dermatology, University of California San Francisco School of Medicine, San Francisco, California, USA.

Background/objectives: The 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.

Methods: A 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.

Results: Nearly 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.

Conclusions: The 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.
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http://dx.doi.org/10.1111/pde.14202DOI Listing
May 2020

Clinical and histologic presentation of pediatric reactive granulomatous dermatitis.

Pediatr Dermatol 2020 May 1;37(3):498-503. Epub 2020 Mar 1.

Departments of Dermatology and Pediatrics, School of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin.

Objective: To characterize the clinical and histologic presentation of reactive granulomatous dermatitis (RGD) in the pediatric population.

Methods: In this multicenter retrospective chart review, 7 pediatric patients with biopsy-proven RGD were identified. Photographs, histology reports, and clinical course were reviewed to discover patterns in demographics, comorbid conditions, autoimmune sequelae, drug exposures, infections, morphology, and histologic features.

Results: Overall, 7 patients were included and analyzed. Most were female and Hispanic. All presented with a similar dermatologic phenotype previously described in the adult literature including macular erythema and annular, pink to violaceous, edematous papules and plaques, often involving proximal extremities and extensor joints. All biopsies demonstrated variable collagen alteration and a perivascular interstitial infiltrate of histiocytes with or without mucin. Neutrophils or karyorrhexic debris were present in 4/7 of the biopsies, and eosinophils were occasionally seen (2/7 cases). In all cases, RGD was associated with active SLE or led to a new diagnosis, and initiation of systemic treatment improved cutaneous disease.

Conclusions: Pediatric RGD was more common in female patients and ethnic minorities, and strongly associated with SLE. Clinical and histologic presentations were consistent across all cases with only minor variations, suggesting that recognition and confirmation might be expedited by familiarity with these dominant patterns. Diagnosis of RGD in pediatric patients should prompt screening for SLE.
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http://dx.doi.org/10.1111/pde.14137DOI Listing
May 2020

Hypergammaglobulinemic purpura of Waldenström in children.

Pediatr Dermatol 2020 May 27;37(3):467-475. Epub 2020 Feb 27.

Department of Dermatology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

Background: Hypergammaglobulinemic purpura of Waldenström (HGPW), a rare cutaneous eruption characterized by the triad of recurrent episodes of lower extremity petechiae, symptoms of stinging and burning, and lower extremity edema, is poorly described in children. Some children have been reported to follow a benign course, while others are eventually diagnosed with fulminant rheumatologic disease.

Objectives: To determine the distinguishing features of HGPW including the spectrum of disease manifestations and clinical outcomes.

Methods: This is a multicenter, retrospective case series of six children with HGPW combined with a literature review of 45 previously published pediatric cases.

Results: Most children were eventually diagnosed with systemic disease (63%) or developed autoantibody accumulation suggestive of evolving disease (71%). The most common diagnoses were Sjogren's syndrome and systemic lupus erythematosus. The mean duration between onset of cutaneous eruption and diagnosis of systemic disease was 5.6 years, underscoring that HPGW patients often present with a rash that precedes the development of systemic symptoms.

Conclusions: Diagnosis of HGPW should prompt initial screening for rheumatologic disease with long-term rheumatology follow-up, as the majority of patients present with evolving manifestations of systemic disease.
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http://dx.doi.org/10.1111/pde.14120DOI Listing
May 2020

Rare Vesiculopustular Eruptions of the Neonatal Period.

Clin Perinatol 2020 03 30;47(1):53-75. Epub 2019 Sep 30.

Department of Dermatology (Pediatric Dermatology), Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA; Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA.

Numerous disorders present with vesiculopustular eruptions in the neonatal period, ranging from benign to life-threatening. Accurate and prompt diagnosis is imperative to avoid unnecessary testing and treatment for benign eruptions, while allowing for adequate treatment of potentially fatal disorders. In this review, we highlight several rare blistering diseases of the newborn. A diagnostic approach is outlined to provide clinicians with a framework for approaching a neonate with vesicles, pustules, or ulcers.
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http://dx.doi.org/10.1016/j.clp.2019.09.005DOI Listing
March 2020

Progressive blistering and hypertrichosis in a young child.

Pediatr Dermatol 2019 Nov;36(6):951-952

Departments of Dermatology (Pediatric Dermatology) and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

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http://dx.doi.org/10.1111/pde.13977DOI Listing
November 2019

A 13-year-old girl with recurrent oral ulcers.

Pediatr Dermatol 2019 Nov;36(6):953-954

Departments of Dermatology (Pediatric Dermatology) and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

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http://dx.doi.org/10.1111/pde.13997DOI Listing
November 2019

Dental root abnormalities in four children with PHACE syndrome.

Pediatr Dermatol 2019 Jul 1;36(4):505-508. Epub 2019 Apr 1.

Department of Developmental Sciences, Marquette University School of Dentistry, Milwaukee, Wisconsin.

PHACE(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.
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http://dx.doi.org/10.1111/pde.13818DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6620126PMC
July 2019

Folliculotropic mycosis fungoides in a pediatric patient mimicking black dot tinea capitis.

Pediatr Dermatol 2019 May 21;36(3):386-387. Epub 2019 Feb 21.

Section of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

Immunosuppression following organ transplantation is a known risk factor for the development of lymphoproliferative disorders. Mycosis fungoides, a rare entity in pediatric patients, has seldom been reported as a post-transplant lymphoproliferative disorder. We report a case of folliculotropic mycosis fungoides in a pediatric patient following liver transplantation that was initially diagnosed as tinea capitis.
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http://dx.doi.org/10.1111/pde.13768DOI Listing
May 2019

New-Onset Blistering Eruption in a Young Child.

J Pediatr 2019 Feb 4;205:290-290.e1. Epub 2018 Oct 4.

Department of Dermatology Medical College of Wisconsin Milwaukee, Wisconsin.

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http://dx.doi.org/10.1016/j.jpeds.2018.09.022DOI Listing
February 2019

Characteristics of pediatric recurrent erythema multiforme.

Pediatr Dermatol 2018 Jan 12;35(1):97-103. Epub 2017 Dec 12.

Section of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA.

Background: Erythema 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.

Objective: To characterize clinical features, laboratory findings, and treatment responses of pediatric recurrent EM.

Methods: A 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.

Results: Twenty-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.

Conclusion: This 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.
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http://dx.doi.org/10.1111/pde.13357DOI Listing
January 2018

Discordance of pediatric morphea treatment by pediatric dermatologists.

Pediatr Dermatol 2018 Jan 22;35(1):47-54. Epub 2017 Nov 22.

Section of Dermatology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, USA.

Background/objectives: Studies 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.

Methods: A survey regarding topical treatment practices of pediatric morphea, with representative case-based scenarios, was distributed to pediatric dermatologists and results were tallied.

Results: The 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.

Conclusion: The 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.
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http://dx.doi.org/10.1111/pde.13281DOI Listing
January 2018

Longitudinal Study of Pediatric Urticaria Pigmentosa.

Pediatr Dermatol 2017 Mar 30;34(2):144-149. Epub 2017 Jan 30.

Department of Dermatology, Mayo Clinic Health System, Eau Claire, Wisconsin.

Background/objectives: Urticaria 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.

Methods: Between 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.

Results: The 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.

Conclusion: Severe 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.
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http://dx.doi.org/10.1111/pde.13066DOI Listing
March 2017

Successful Treatment of Vitiligo Associated with Vogt-Koyanagi-Harada Disease.

Pediatr Dermatol 2017 Mar 16;34(2):204-205. Epub 2016 Dec 16.

Division of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

Vogt-Koyanagi-Harada disease (VKH) is a rare multisystem disorder with cutaneous, ophthalmic, neurologic, and auditory manifestations. There is a paucity of published literature regarding the management of cutaneous features in VKH. We report a case of VKH-associated vitiligo responsive to topical corticosteroids and topical calcineurin inhibitors.
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http://dx.doi.org/10.1111/pde.13044DOI Listing
March 2017

Eczema and Urticaria as Manifestations of Undiagnosed and Rare Diseases.

Pediatr Clin North Am 2017 Feb;64(1):39-56

Section of Pediatric Dermatology, Department of Dermatology, Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, USA; Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address:

Eczema and urticaria are common disorders encountered in pediatric patients, but they may occasionally be the presenting complaint in a child with an underlying rare disease. Immunodeficiency syndromes should be suspected when eczema is associated with neonatal onset, recurrent infections, chronic lymphadenopathy, or failure to thrive. Nutritional deficiencies and mycosis fungoides are in the differential diagnosis for a child with a recalcitrant eczematous eruption. Autoinflammatory syndromes should be suspected in a child with chronic urticaria, fever, and other systemic signs of inflammation. Although these disorders are rare, early recognition allows for appropriate treatment and decreased morbidity for the child.
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http://dx.doi.org/10.1016/j.pcl.2016.08.004DOI Listing
February 2017

How Often are Pediatric Patients with Clinically Amyopathic Dermatomyositis Truly Amyopathic?

Pediatr Dermatol 2017 Jan 4;34(1):50-57. Epub 2016 Nov 4.

Department of Pediatrics, Section of Pediatric Rheumatology, Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation.

Objective: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution.

Methods: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin.

Results: Of 46 patients presenting with skin findings consistent with dermatomyositis, 10 patients (21.7%) did not have evidence of muscle involvement on history or exam, and these tended to be the younger patients. Of these 10, only 2 (4% of all the JDM patients) were truly amyopathic upon further evaluation (all five muscle enzymes [aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, creatine kinase and aldolase], magnetic resonance imaging [MRI], muscle biopsy). In our series, muscle biopsy was not helpful in identifying subclinical myositis. In contrast, MRI did uncover subclinical muscle disease.

Conclusion: These data suggest that truly amyopathic JDM is rare and that a thorough workup that includes all five muscle enzymes and MRI may uncover occult myositis.
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http://dx.doi.org/10.1111/pde.13013DOI Listing
January 2017

Toxic Epidermal Necrolysis-Like Cutaneous Lupus in Pediatric Patients: A Case Series and Review.

Pediatrics 2016 06;137(6)

Division of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin;

Bullous eruptions in patients with underlying systemic lupus erythematosus (LE) can mimic toxic-epidermal necrolysis (TEN), a rapidly progressive mucocutaneous reaction usually associated with medication use. Differentiating between classic drug-induced TEN and TEN-like cutaneous LE is important but difficult. We report a series of 3 patients with pediatric systemic LE who were admitted with severe worsening of skin disease resembling TEN. However, the initial photo-distribution of the eruption, subacute progression, limited mucosal involvement, mild systemic symptoms, supportive biopsy and laboratory results, and lack of culprit drugs was more suggestive of a TEN-like cutaneous LE. These patients recovered with various systemic immunosuppressive medications including methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Our cases are rare and demonstrate key clinical and histologic features of TEN-like cutaneous LE in young patients and the importance of differentiating this entity from drug-induced TEN.
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http://dx.doi.org/10.1542/peds.2015-4497DOI Listing
June 2016

New Pustular Lesions in an Infant with Fever.

Pediatr Dermatol 2015 Sep-Oct;32(5):737-8. Epub 2015 Jun 8.

Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin.

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http://dx.doi.org/10.1111/pde.12623DOI Listing
June 2016

Vaccine-Associated Herpes Zoster.

J Pediatr 2015 Aug 9;167(2):494. Epub 2015 May 9.

Department of Dermatology, Section of Pediatric Dermatology and Department of Pediatrics Medical College of Wisconsin, Milwaukee, Wisconsin.

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http://dx.doi.org/10.1016/j.jpeds.2015.04.034DOI Listing
August 2015

Neural tube dysraphism: review of cutaneous markers and imaging.

Pediatr Dermatol 2015 Mar-Apr;32(2):161-70. Epub 2014 Dec 29.

Department of Dermatology, Virginia Commonwealth University, Richmond, Virginia.

Neural tube dysraphisms are congenital anomalies resulting from impaired formation of structures along the craniospinal axis during central nervous system development. When these malformations are large or lack a skin covering, they are easily recognized, whereas smaller or skin-covered malformations may not be readily apparent. Due to the intimate embryologic origin of the skin and nervous system, these occult malformations are often heralded by associated cutaneous abnormalities. In this article, the common clinical presentations and cutaneous markers of craniospinal dysraphism are reviewed, along with the recommended imaging modalities.
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http://dx.doi.org/10.1111/pde.12485DOI Listing
December 2015

The natural history of skin-limited Langerhans cell histiocytosis: a single-institution experience.

J Pediatr Hematol Oncol 2014 Nov;36(8):613-6

*Pediatric Hematology and Oncology †Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI.

Introduction: Prior reports of Langerhans cell histiocytosis (LCH) suggest that isolated skin involvement is rare and often progresses to systemic disease. More rapid access to pediatric subspecialty care has likely led to more frequent representation of this condition. The purpose of this study is to characterize the natural history of skin-limited LCH in an era of increased access to pediatric subspecialty care.

Materials And Methods: A retrospective chart review was performed on all patients newly diagnosed with LCH between 2001 and 2012 at the Children's Hospital of Wisconsin. Extensive review of laboratory, physical examination, and imaging reports was performed and data collected for patients with biopsy-proven skin LCH.

Results: Sixteen individuals with skin-limited LCH were identified. The median age at onset of skin eruption was birth (range, birth to 6 mo), and median duration of follow-up was 19.5 months (range, 2 wk to 10 y) from diagnosis. One patient (6%) developed pituitary disease and 1 patient (6%) had refractory skin involvement. All others experienced complete resolution. For patients without progressive or refractory disease, resolution of skin findings occurred within 7 months from onset.

Discussion: Progression of skin-limited to multisystem LCH likely may be less frequent than previously described.
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http://dx.doi.org/10.1097/MPH.0000000000000248DOI Listing
November 2014

Tooth enamel hypoplasia in PHACE syndrome.

Pediatr Dermatol 2014 Jul-Aug;31(4):455-8. Epub 2014 Jun 11.

Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

Individuals with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe syndrome) have reported dental abnormalities to their healthcare providers and in online forums, but dental involvement has not been comprehensively studied. A study was conducted at the third PHACE Family Conference, held in Milwaukee, Wisconsin, in July 2012. A pediatric dentist examined subjects at enrollment. Eighteen subjects were enrolled. The median age was 4.2 years (range 9 mos-9 yrs; 14 girls, 4 boys). Eleven of 18 patients had intraoral hemangiomas and five of these (50%) had hypomature enamel hypoplasia. None of the seven patients without intraoral hemangiomas had enamel hypoplasia. No other dental abnormalities were seen. Enamel hypoplasia may be a feature of PHACE syndrome when an intraoral hemangioma is present. Enamel hypoplasia increases the risk of caries, and clinicians should refer children with PHACE syndrome to a pediatric dentist by 1 year of age.
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http://dx.doi.org/10.1111/pde.12361DOI Listing
May 2015

An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases.

Pediatr Dermatol 2014 Mar-Apr;31(2):138-45. Epub 2014 Jan 23.

Department of Dermatology, University of Missouri, Columbia, Missouri.

Circumscribed juvenile pityriasis rubra pilaris (PRP) is an uncommon dermatosis. We describe the unusual clustering of circumscribed juvenile PRP cases in our pediatric dermatology clinic in 2011. A retrospective chart review was done of patients presenting during the summer of 2011 with classic findings of circumscribed juvenile PRP. Clinical data including past medical and family history, presenting symptoms, infectious disease history and evaluation, biopsy results, and management were recorded. Seven patients, ages 5 to 19 years, all had strikingly similar skin findings of pink to hyperpigmented, well-defined, scaly papules and plaques on their elbows, knees, dorsal hands, ankles, and Achilles tendons. Four of the seven also had palmoplantar involvement. Four were sibling pairs and the other three were unrelated. Streptococcus pyogenes infection was suspected as a trigger in four of the patients. The unusual clustering of this uncommon disease, along with the occurrence in two sibling pairs, suggests that a genetic susceptibility unmasked by an infectious agent may play a role in its pathogenesis.
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http://dx.doi.org/10.1111/pde.12260DOI Listing
January 2015

Practice gaps: Evaluating the clinical utility of autoantibodies in morphea.

Authors:
Yvonne E Chiu

JAMA Dermatol 2013 Oct;149(10):1166

Division of Pediatric Dermatology, Department of Dermatology, Medical College of Wisconsin, Milwaukee.

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http://dx.doi.org/10.1001/jamadermatol.2013.4908DOI Listing
October 2013