About

Beth Drolet, MD, is the Professor and Chair of the Department of Dermatology at the University of Wisconsin School of Medicine and Public Health. She completed her undergraduate education at Michigan State University, earned her MD at Loyola University, and completed residency and fellowship training in Clinical Research and Pediatric Dermatology at the Medical College of Wisconsin. Dr. Drolet was promoted to professor, Vice Chair, and Chief of Pediatric Dermatology at the Medical College of Wisconsin, where she also served as Medical Director of Birthmarks and Vascular Anomalies, VP of Ambulatory Services, and Chief Experience Officer at the Children’s Hospital of Wisconsin. Since March 2019, she has served as the Chair of UW Dermatology, the fourth in the department's history since 1946. Her research has focused on birthmarks, vascular anomalies, and infantile hemangiomas, leading to over 150 peer-reviewed publications. She has established an infrastructure for multidisciplinary and inter-institutional research, and is a founding member and past president of the Hemangioma Investigator Group. Her work includes leading clinical trials and designing longitudinal studies to understand the incidence, demographics, complications, and outcomes of vascular anomalies. Leveraging next-generation sequencing technology, her team demonstrated that most vascular anomalies are caused by postzygotic mutations in highly conserved oncogenes, revealing potential pharmacologic targets for treatment. Dr. Drolet has served as past president of the Society for Pediatric Dermatology and is on the editorial board of Pediatric Dermatology. Her research has been supported by the NIH, Greater Milwaukee Foundation, Dermatology Foundation, and Pediatric Dermatology Research Alliance.

Research topics

• Medicine
• Internal medicine
• Biology
• Immunology
• Genetics
• Surgery
• Data Mining
• Pathology
• Artificial Intelligence
• Pediatrics
• Computer Science
• Virology
• Intensive care medicine
• Environmental health
• Family medicine
• Dermatology
• Simulation
• Cancer research
• Computational biology
• Anesthesia
• Medical emergency

Selected publications

• Genotype Analysis of Severe Blistering Mucocutaneous Reactions

  Journal of Investigative Dermatology · 2026-03-01

  articleSenior author
  PublisherDOI

• The Impact of Genetic Testing on Clinical Diagnosis, Treatment, and Surveillance in Patients with Vascular Anomalies

  Journal of Investigative Dermatology · 2026-03-01

  articleOpen access
  PublisherOA PDFDOI

• ReInspire: A Phase 2 Study of Mutant-Selective PI3Kα Inhibitor, RLY-2608, in Adults and Children with PIK3CA-Related Overgrowth Spectrum and Malformations Driven by PIK3CA Mutation

  Journal of Investigative Dermatology · 2026-03-01

  articleOpen access1st authorCorresponding
  PublisherOA PDFDOI

• Clinical Characterization of Reactive Infectious Mucocutaneous Eruption (RIME): A Pediatric Multicenter Retrospective Cohort

  Journal of Investigative Dermatology · 2026-03-01

  articleOpen access
  PublisherOA PDFDOI

• Reply to Expanding the Phenotypic Spectrum of <scp>SecVAUS</scp>: Report of Two Cases

  Pediatric Dermatology · 2025-05-01

  letterOpen access

  We declare the availability of data for Wiley.

  PublisherOA PDFDOI

• 60: Multi-Center Study of Reactive Infectious Mucocutaneous Eruption (RIME): Design and Methodology

  Journal of Investigative Dermatology · 2025-03-01

  articleOpen access
  PublisherOA PDFDOI

• Dermatologic Presentation of Pulmonary Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient

  American Journal of Respiratory and Critical Care Medicine · 2025-05-01

  article

  Abstract INTRODUCTION : Eosinophilic granulomatosis with polyangiitis (EGPA) is a chronic eosinophilic vasculitis that affects small and medium sized arteries. EGPA often presents as persistent asthma, rhinosinusitis with polyposis, peripheral eosinophilia, and other systemic involvement. Clinical presentations are heterogenous with variability in presentation of multisystem organ involvement; however, laboratory testing can increase clinical suspicion for underlying disease. The incidence ranges between 0.5-4.2 cases per year with a mean age of 50 years. Pediatric diagnoses are extremely rare. We present a case of biopsy-confirmed EGPA in a pediatric patient with an atypical presentation. DESCRIPTION OF CASE: A 12-year-old female with a history of epidermolysis bullosa acquisita (EBA; anti-COL7 Antibody positive) managed with systemic corticosteroids, dapsone, and mycophenolate, presented with a 2-year history of chronic, progressive, non-productive cough, progressive interstitial opacities on chest radiography, bronchial wall thickening with air trapping on computed tomography, and reversible obstruction with air trapping on spirometry. Trial of inhaled corticosteroids with long-acting beta-agonist did not improve obstruction. She was exposed to second-hand cigarette smoke, horses, dogs, chickens, and rabbits. Bronchoalveolar lavage was significant for eosinophils, hemosiderin laden macrophages, and Streptococcus pneumoniae and Haemophilus influenzae, thought to be chronic given symptom duration. Laboratory testing showed persistent eosinophilia (10-15%) despite intermittent corticosteroids, positive p-ANCA, positive anti-MPO, negative PR3 antibodies, elevated IgG, normal IgE, and negative testing for fungal infections. Immune evaluation revealed normal complement activity, robust response to vaccine titers, and normal immune deficiency panel flow cytometry. Transbronchial lung-cryobiopsy showed inflamed airways with numerous interstitial and peri-vascular eosinophils and pulmonary hemorrhage. Despite the absence of discrete granulomas, the pathologic findings, laboratory data, and clinical symptoms were consistent with a diagnosis of EGPA. DISCUSSION: This case highlights the variability in presentation of EGPA, particularly in pediatric patients. Vasculitis is classically seen in adults, but less often in children. The possibility of an underlying autoimmune disease complicated the timing of diagnosis in this case. The cough preceded the skin disease, and it is possible that ongoing lung injury and interstitial lung disease from EGPA may have contributed to the generation of autoantibodies and development of EBA (COL7 is a matrix protein found in the lung). Treatment of EGPA with steroid sparing agents such as mepolizumab or benralizumab offer the possibility of avoiding long term steroid side effects and were initiated for our patient. It is important in pediatric patients with eosinophilic lung disease that a comprehensive differential diagnosis includes EGPA.

  PublisherDOI

• Congenital Cutaneous Hamartomas With Skeletal Muscle Differentiation Associated With <scp>LUMBAR</scp> Syndrome

  Pediatric Dermatology · 2025-03-06 · 2 citations

  articleOpen access

  BACKGROUND/OBJECTIVES: Congenital skin anomalies have been observed in LUMBAR syndrome, but their clinicopathologic significance remains unclear. This study aimed to investigate the congenital, nonvascular skin anomalies in LUMBAR syndrome (lower body infantile hemangiomas, urogenital anomalies and hemangioma ulceration, spinal cord malformations, bony deformities, anorectal/arterial anomalies, and renal anomalies). We hypothesized that an association exists between complex skin hamartomas and LUMBAR syndrome, similar to recent findings in PHACE syndrome (posterior fossa anomalies, hemangioma, cerebrovascular arterial anomalies, cardiovascular anomalies, and eye anomalies). METHODS: This IRB-exempt, retrospective study utilized a database of 144 published LUMBAR cases, used to establish diagnostic criteria for the syndrome, to identify individuals with nonhemangioma skin anomalies. Data extracted from identified patients included lesion location and clinical description, lesional histopathology (when available), location of the segmental infantile hemangioma, and the presence of additional congenital anomalies. RESULTS: We discovered 57 individuals with LUMBAR syndrome and nonhemangioma skin anomalies. Of these, 5/57 (9%) presented with lumbosacral or pelvic soft tissue appendages accompanied by histopathological findings. All five exhibited complex skin hamartomas with skeletal muscle differentiation, including four with histopathologic features diagnostic or suggestive of rhabdomyomatous mesenchymal hamartoma and one with a fetal rhabdomyoma. CONCLUSIONS: This study reveals a novel association between complex skin hamartomas with skeletal muscle differentiation and LUMBAR syndrome. Our findings suggest that these complex skin hamartomas represent another common link between LUMBAR and PHACE syndromes. Furthermore, this association supports the potential role of abnormal mesodermal tissue migration during early embryogenesis in the shared pathogenesis of these syndromes.

  PublisherOA PDFDOI

• Deleterious variants in the autophagy-related gene RB1CC1/FIP200 impair immunity to SARS-CoV-2

  Nature Communications · 2025-11-27 · 2 citations

  articleOpen access

  The clinical outcome of SARS-CoV-2 infection spans from asymptomatic viral elimination to lethal COVID-19 pneumonia, which is due to type I interferon (IFN) deficiency in at least 15-20% of cases. We report two unrelated male patients with critical COVID-19 who are heterozygous for rare deleterious variants in RB1CC1, encoding the autophagy-related FIP200 protein. Airway epithelial cells genetically deprived of FIP200 or cell lines expressing the RB1CC1/FIP200 patient variants exhibit elevated SARS-CoV-2 replication and impaired autophagic flux. The antiviral function of FIP200 is independent of canonical autophagy and type I IFN, but involves the selective autophagy receptor NDP52. We identify a non-canonical function of FIP200 in a novel lysosomal degradation pathway, in which SARS-CoV-2 virions are targeted to single-membrane compartments for degradation of viral RNA in LC3B-positive acidified vesicles. This pathway is impaired in FIP200-deficient cells and in cells expressing FIP200 patient haplotypes. Collectively, we describe a cell-autonomous anti-SARS-CoV-2 restriction pathway, dependent on FIP200 and NDP52, and independent of canonical autophagy and type I IFN, which can underlie critical COVID-19 pneumonia.

  PublisherDOI

• The seven enigmas of SARS-CoV-2: From the past to the future

  Journal of Human Immunity · 2025-09-24 · 3 citations

  articleOpen access

  Five years ago, we launched the COVID Human Genetic Effort. Our goal was to explain the clinical variability among SARS-CoV-2-exposed individuals by searching for monogenic inborn errors of immunity (IEI) and their phenocopies. We deciphered the pathogenesis of critical COVID-19 pneumonia and multisystemic inflammatory syndrome in children (MIS-C) in ~15% and 2% of cases, respectively, thereby revealing general mechanisms of severe disease. We also defined neuro-COVID-19 genetically and immunologically in one child, while we delineated the immunological mechanisms of COVID-toes in healthy children and young adults, paving the way for their genetic study. Understanding the human genetic and immunological basis of resistance to SARS-CoV-2 infection, long COVID, and myocarditis post mRNA vaccination, has been challenging and investigations remain ongoing. This work highlights the power of patient-based basic research and large-scale international collaborative efforts to discover human genetic and immunological drivers of infectious disease phenotypes, with implications for the timely development of new medical strategies before the next pandemic arrives.

  PublisherDOI

Recent grants

• NIH Grant R34AR060881

  NIH · $175k · 2013

• NIH Grant R13AR061943

  NIH · $28k · 2012

Frequent coauthors

• Dawn H. Siegel

  Stanford University

  119 shared

• Ilona J. Frieden

  University of California, San Francisco

  119 shared

• Jean‐Laurent Casanova

  Université Paris Cité

  108 shared

• María C. Garzon

  97 shared

• Laurent Abel

  Université Paris Cité

  96 shared

• Denise W. Metry

  Texas Children's Hospital

  94 shared

• Elena Pope

  Oregon Dermatology & Research Center

  85 shared

• Kristen E. Holland

  Medical College of Wisconsin

  82 shared

Labs

• Our LabsPI

Education

• B.S.

  Michigan State University

• M.D.

  Loyola University

• Other

  Residency and fellowship training in Clinical Research & Pediatric Dermatology

Awards & honors

• Geneva F. and Sture Johnson Distinguished Chair