Pyoderma Vegetans: Background, Pathophysiology, Etiology

Sections

Overview

Background

Pyoderma vegetans (PV) is a rare disorder clinically characterized by large verrucous plaques with elevated borders and multiple pustules. Pyoderma vegetans is an eruption of multiple pustular ulcerations; it may have a bacterial etiology similar to chancriform pyoderma.^{[1, 2, 3, 4]}

Hallopeau first characterized this process in 1898.^[5]Although its etiology is unknown, this disease has been attributed to bacterial infection in an individual who is immunocompromised^[1]; more recent reports support this theory.^{[2, 6, 7]}Pyoderma vegetans has been associated with ulcerative colitis,^{[8, 9, 10, 11]}diffuse T-cell lymphoma,^{[6, 12]} alcoholism with malnutrition,^[1]HIV infection,^[13]chronic myeloid leukemia (CML),^[14]and lupus nephritis.^[15]

Su et al reported 7 patients with clinically characteristic pyoderma vegetans.^[2]These patients had various conditions that compromised their immune systems; these conditions included pulmonary granuloma, chronic granulocytic leukemia, arthritis treated with azathioprine and prednisone, and seminoma treated with x-ray irradiation, which predisposed them to bacterial infections.^[2]After pyoderma vegetans was diagnosed, 1 patient developed squamous cell carcinoma and colonic carcinoma. Ishibashi described a patient with pyoderma vegetans without any history of an immunocompromising condition.^[16]

Pathophysiology

The etiology of pyoderma vegetans (PV) is not known, although it is often associated with staphylococcal and streptococcal infections in a patient with an immunosuppressive state or a dysfunction of the immune system. The immunological dysfunction is believed to induce the development of vegetations. In addition to bacterial infections, fungal infections have also been implicated in the context of immunosuppression, as demonstrated by a case of treatment-resistant Trichophyton mentagrophytes –induced tinea manuum resulting in a chronic pyoderma vegetans in a patient with decreased immunoglobulins and impaired phagocytosis.^[17]

Diffuse T-cell lymphoma,^[6]ulcerative colitis,^[8]and HIV infection^[13]have been associated with this condition. A patient was described with pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis who, upon immunofluorescence examination, demonstrated in vivo bound and circulating immunoglobulin G antibasement membrane zone antibodies.^[18]These antibodies reacted with the bullous pemphigoid antigen 230. The presence of circulating autoantibodies to the bullous pemphigoid antigen 230 in this patient was considered an epiphenomenon, resulting from epidermal damage induced by inflammation of the pyodermatitis-pyostomatitis vegetans. Pyoderma vegetans may also be associated with myelodysplastic syndromes.^[19]

Etiology

The etiology of pyoderma vegetans is not known.

Epidemiology

Pyoderma vegetans is rare.

Prognosis

With therapy, the prognosis is good, although this may prove untrue in the face of associated medical conditions, such as HIV, diffuse T-cell lymphoma, and CML, which must be properly addressed.

In patients in whom inflammatory bowel disease does not coexist, pyoderma vegetans has a good prognosis with therapy. In contrast, pemphigus vegetans has a poor prognosis without the continuous use of immunosuppressive drugs.

Clinical Presentation

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Media Gallery

A 24-year-old woman with rapidly evolving pyoderma vegetans.
Pyoderma vegetans.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Justin Brown, MD Dermatologist, The Dermatology Group

Justin Brown, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Medicis for review panel membership; Received honoraria from Triax for review panel membership.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Julie C Harper, MD Assistant Program Director, Assistant Professor, Department of Dermatology, University of Alabama at Birmingham

Julie C Harper, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Received honoraria from Stiefel for speaking and teaching; Received honoraria from Allergan for speaking and teaching; Received honoraria from Intendis for speaking and teaching; Received honoraria from Coria for speaking and teaching; Received honoraria from Sanofi-Aventis for speaking and teaching.

Pyoderma Vegetans

Background

Pathophysiology

Etiology

Epidemiology

Prognosis

References

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