Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is uncommon and can sometimes be associated with systemic diseases. The pathophysiology underlying this condition is poorly understood, although recent advances suggest that local cutaneous abnormalities and functionally abnormal neutrophils may trigger ongoing innate and adaptive immune system activity. PG remains a difficult condition to diagnose, mainly because it was previously seen as a diagnosis of exclusion, although newer diagnostic criteria have been proposed in order to overcome this. Furthermore, many patients do not respond to conventional therapy for PG once diagnosed, and experience persistence or worsening of their condition over time. The advent of immune targeted therapies, however, may represent a new treatment option for these patients. This review focuses on the clinical features and diagnosis of PG, as well as providing an update in our understanding of the pathophysiology and treatment options available for this debilitating condition.
机构:
Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, Italy
Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Largo Agostino Gemelli 8, I-00168 Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Caldarola, Giacomo
Traini, Daniele O.
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Traini, Daniele O.
Falco, Gennaro M.
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Falco, Gennaro M.
Chiricozzi, Andrea
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Chiricozzi, Andrea
De Luca, Eleonora
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
De Luca, Eleonora
Mannino, Maria
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Mannino, Maria
Pellegrino, Luca
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Pellegrino, Luca
Peris, Ketty
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Peris, Ketty
De Simone, Clara
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Fdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
Univ Cattolica Sacro Cuore, Dipartimento Univ Med & Chirurg Traslaz, Dermatol, Rome, ItalyFdn Policlin Univ A Gemelli IRCCS, Dipartimento Sci Med & Chirurg, Dermatol, Rome, Italy
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Ohio State Univ, Coll Med, Columbus, OH 43210 USAOhio State Univ, Coll Med, Columbus, OH 43210 USA
Hoffman, Kalyn P.
Shearer, Sabrina
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Ohio State Univ, Dept Internal Med, Div Dermatol, Columbus, OH 43210 USAOhio State Univ, Coll Med, Columbus, OH 43210 USA
Shearer, Sabrina
Chung, Catherine
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Ohio State Univ, Dept Internal Med, Div Dermatol, Columbus, OH 43210 USA
Ohio State Univ, Dept Pathol, Columbus, OH 43210 USAOhio State Univ, Coll Med, Columbus, OH 43210 USA
Chung, Catherine
Kaffenberger, Jessica A.
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Ohio State Univ, Dept Internal Med, Div Dermatol, Columbus, OH 43210 USAOhio State Univ, Coll Med, Columbus, OH 43210 USA
Kaffenberger, Jessica A.
Kaffenberger, Benjamin H.
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Ohio State Univ, Dept Internal Med, Div Dermatol, Columbus, OH 43210 USAOhio State Univ, Coll Med, Columbus, OH 43210 USA
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Oregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
Oregon Hlth & Sci Univ, OHSU Wound & Hyperbar Med, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USAOregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
Becker, Sarah L.
Badawi, Ahmed H.
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Univ Kansas, Med Ctr, Div Dermatol, Kansas City, KS USA
Freeman Hlth Syst, Dermatol & Skin Canc Ctr, Joplin, MO USAOregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
Badawi, Ahmed H.
Thornton, Chase
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Wayne State Univ, Dept Internal Med, Detroit, MI USAOregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
Thornton, Chase
Ortega-Loayza, Alex G.
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Oregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
Oregon Hlth & Sci Univ, OHSU Wound & Hyperbar Med, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USAOregon Hlth & Sci Univ, Dept Dermatol, 3303 SW Bond Ave Ctr Hlth Healing Bldg 1,Suite 16, Portland, OR 97239 USA
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MAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USAMAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USA
POWELL, FC
SCHROETER, AL
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MAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USAMAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USA
SCHROETER, AL
SU, WPD
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MAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USAMAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USA
SU, WPD
PERRY, HO
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MAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USAMAYO CLIN & MAYO FDN, DEPT DERMATOL, 200 1ST ST SW, ROCHESTER, MN 55905 USA