Pyoderma Gangrenosum (PG)

A man getting an ulcer cleaned in clinic by Dr. Alex Ortega

Specialized care for the management and treatment of pyoderma gangrenosum (PG)

Pyoderma gangrenosum (PG) is a rare skin condition characterized by open sores or wounds that develop on the skin (skin ulcers). These ulcers cause the outside layers of the skin to deteriorate, leading to intense redness, sensitivity and pain. Treatment of your skin ulcers is critical due to the possibility of severe infection (e.g. cellulitis), scarring, pain, mental concerns, and potential loss of mobility.

Clinic director Alex Ortega-Loayza, M.D., M.C.R., combines advanced wound care training with almost a decade of PG research to provide high quality, individualized care for patients. Furthermore, Dr. Ortega has dedicated his career to advancing PG diagnosis and treatment through research, assembling basic scientists, biostatisticians, and clinicians together to form the pyoderma gangrenosum study team (PYGAS) at OHSU.

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis — a group of conditions in which neutrophils (a type of white blood cells that protect us from infections) quickly react and infiltrate affected tissue often after some sort of abrasion or injury. PG usually begins as a small bump or sore before growing into a larger, painful open wound (skin ulcer). 
Skin ulcers associated with PG have elevated borders, with the skin surrounding the wound becoming red or purple. PG ulcers are often exposed and extremely sensitive, which can lead to concerns of infection, pain management, scarring, mental issues, and potential loss of mobility.
Key info:
  • The exact cause of pyoderma gangrenosum is unknown, but it is likely a disorder of the immune system
  • A common misconception due to its name, PG is neither infectious or related to gangrene
  • PG is not contagious
  • PG has an incidence of approximately 6 cases per million population per year.
  • The average age of onset is mid 40s
  • It is associated with other inflammatory conditions such as inflammatory bowel disease and rheumatoid arthritis; it is also sometimes associated with hematological malignancies.
  • Other variants exist: bullous, vegetative, pustular, peristomal, post-operative
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Diagnosing pyoderma gangrenosum (PG) can be challenging in clinical practice, which often leads to misdiagnosis. PG is rare, there are no specific laboratory or histopathologic markers to test for the disease, and currently there are no diagnostic frameworks that are widely accepted as gold standard. 
At OHSU, diagnosis is made by a thorough testing and exclusion process to rule out other similar conditions such as chronic venous ulcers (CVU) — the most common cause of lower extremity ulcers. This process may include skin biopsies, imaging, and analysis of individual patient's clinical presentation.
Dr. Ortega is leading efforts to identify specific markers that could be used to minimize the challenge of diagnosing PG worldwide, thus improve patient outcomes. Currently three clinical diagnostic frameworks have been proposed to diagnose PG. Dr. Ortega is the co-leader of UPGRADE (Understanding Pyoderma Gangrenosum: Review and Analysis of Disease Effects) an international initiative to study outcome measures in patients with PG. He has also been sponsored by the Medical Dermatology Society's Research Committee to develop consensus guidelines to treat PG patients.

A step-by-step treatment approach is utilized to reach control and remission of PG. The most immediate concern is to address any inflammation as well as wound care for any ulcers. This includes proper cleaning and dressing of the wound to create a proper environment for healing. It's common for a series of dressings and wraps to be used to treat the wound. In your appointment we will create a plan for treatment of your PG ulcer/ulcers which might include systemic medications (e.g. immunosuppressives), ordering wound care supplies and/or referral to a wound care center or pain/primary care clinic for proper pain management. Also, based on your concomitant signs and symptoms, you might need referral to a gastroenterologist, rheumatologist or oncologist.

Immunosuppressive medications (topical or systemic) are considered first-line treatments. These include topical corticosteroids (e.g. clobetasol), intralesional corticosteroids (e.g. triamcinolone) or systemic corticosteroids (e.g. prednisone). Other options include topical calcineurin inhibitors (e.g. tacrolimus) or cyclosporine. The presence of associated comorbidities should be considered when selecting medications for maintenance therapy such as biologics (e.g. infliximab in patients with PG and inflammatory bowel disease). Your provider will discuss what options may work best for you.

As part of an academic healthcare center, OHSU has faculty medical experts across the full spectrum of medicine. Because many conditions have crossover in their causes, symptoms, or treatments, it's common for OHSU providers to work collaboratively across medical specialties to achieve the best patient outcomes. We utilize this multidisciplinary care approach in our treatment and research of pyoderma gangrenosum (PG).

Along with clinic director Alex Ortega, M.D., M.C.R., OHSU dermatologists Teri Greiling, M.D., Ph.D., and Jesse Keller, M.D., M.C.R., have special interests in the treatment and research of complex conditions, including PG.

Our dermatologists frequently work with rheumatologists due to the inflammatory nature of PG which can manifest in inflammation of the joints. Rhuematologists Marcia Friedman, M.D., and James Rosenbaum, M.D., collaborate in PG treatment as necessary.

For our PG research program, we collaborate with dermatology basic scientist Yuangang Liu, Ph.D. (OHSU Dermatology Research Division), Suzanne Fei, Ph.D., Lina Gao, Ph.D., M.S., (Biostatistics and Bioinformatics Core at the Oregon National Primate Center) and Emile Latour, M.S., Jeong Lim, Ph.D (Biostatistics Shared Resource at OHSU).

Articles/Videos/Podcasts:
UCSD Grand Rounds Video Presentation, 2021: Pyoderma Gangrenosum
Dermasphere Podcast, 2021: How to diagnose PG with Dr. Alex Ortega – Antihistamines: Scary in the Elderly?
VuMedi Video, 2021: Pyoderma Gangrenosum: How Can We Overcome Challenges in Diagnosis? How Has COVID-19 Affected Current Practices?
MDedge Dermatology, 2020: Management of Classic Ulcerative Pyoderma Gangrenosum

Publications:
Dermatologic Therapy, 2021: Treatment of pyoderma gangrenosum: A multicenter survey‐based study assessing satisfaction and quality of life
Journal of Investigative Dermatology, 2021: Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum
British Journal of Dermatology, 2020: An assessment of the different tools used to assess treatments in pyoderma gangrenosum (plain language summary)
Journal of Trauma and Acute Care Surgery, 2019: Pyoderma gangrenosum misdiagnosis resulting in amputation: A review
Journal of the American Academy of Dermatology, 2015: Pathophysiology of pyoderma gangrenosum (PG): An updated review

Grants/Awards (Dr. Ortega):
Dr. Jesse Ettelson Fund for the Advancement of Dermatology Research, OHSU Dermatology, 2021
Cochrane Scholarship, American Academy of Dermatology, 2020
Victor Newcomer Research Award, Pacific Dermatologic Association, 2019
Gerlinger Endowment Award, 2018
New Investigator Award, Medical Research Foundation of Oregon, 2018

Presidential Citation Award, American Academy of Dermatology Diversity Task Force, 2017 

(Podcast) The Path of a Peruvian Dermatologist

Alex Ortega Loayza, M.D., M.C.R., is an Associate Professor of Dermatology at OHSU. Along with being a board-certified dermatologist, he is also board-certified in internal medicine, has completed a Masters of Clinical Research, and has undergone advanced training in wound care. He specializes in the care of patients with common skin inflammatory conditions such as psoriasis, and complex conditions such as hidradenitis suppurativa and pyoderma gangrenosum.

When Dr. Ortega identified PG as an area of interest in 2015, several major scientific gaps existed. Dr. Ortega now has more than 30 publications in PG alone. He has proposed a model for the pathogenesis in some publications which have been pivotal to drive gene expression studies. He is methodically addressing many of the gaps in PG including understanding the heterogeneity of the disease and rare clinical variants. He has also made contributions to the awareness, diagnosis and management of PG and its variants. He has demonstrated the unmet need for proper diagnostic tests in PG after highlighting the causes of misdiagnosis resulting in amputation.

Dr. Ortega, a native of Lima, Peru, obtained his medical degree from Universidad Nacional Mayor de San Marcos, the oldest medical school in Latin America. After working as a general practitioner in the Navy of Peru, he completed further research training at University of North Carolina-Chapel Hill (UNC-Chapel Hill), and then completed his clinical training in internal medicine and dermatology at Virginia Commonwealth University (VCU). Following his medical training, Dr. Ortega joined the faculty of VCU for a couple of years before coming to OHSU in May 2016.

In his spare time, Dr. Ortega enjoys spending time with his wife and children; when he gets time for himself he likes running, soccer, watching sports and reading biographies. 

Pyoderma Gangrenosum (PG) at OHSU