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Dermatology > BACTERIAL INFECTIONS
Pyoderma Vegetans
Article Last Updated: Feb 1, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Justin Brown, MD, Staff Physician, Department of Dermatology, UMDNJ-New Jersey Medical School
Justin Brown is a member of the following medical societies: Alpha Omega Alpha and Sigma Xi
Coauthor(s):
Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Editors: Julie C Harper, MD, Assistant Program Director, Assistant Professor, Department of Dermatology, University of Alabama at Birmingham; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
PV, blastomycosis-like pyoderma, blastomycosislike pyoderma, mycosis-like pyoderma, mycosislike pyoderma
Background
Pyoderma vegetans (PV) is a rare disorder clinically characterized by large verrucous plaques with elevated borders and multiple pustules. PV is an eruption of multiple pustular ulcerations; it may have a bacterial etiology similar to chancriform pyoderma (Brown, 1957; Su, 1979; Zilberberg, 1960; Wozniacka, In press).
Hallopeau first characterized this process in 1898 (Hallopeau, 1898). Although its etiology is unknown, this disease has been attributed to bacterial infection in an individual who is immunocompromised (Brown, 1957); more recent reports support this theory (Su, 1979; Welch, 1989; Rongioletti, 1996). PV has been associated with ulcerative colitis (Brunsting, 1949), diffuse T-cell lymphoma (Welch, 1989), alcoholism with malnutrition (Brown, 1957), HIV infection (Potekaev, 1991), and chronic myeloid leukemia (CML) (Dutta, 1992).
Su et al reported 7 patients with clinically characteristic PV (Su, 1979). 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 (Su, 1979). After PV was diagnosed, 1 patient developed squamous cell carcinoma and colonic carcinoma. Ishibashi described a patient with PV without any history of an immunocompromising condition (Ishibashi, 1986).
Pathophysiology
The etiology of 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 PV in a patient with decreased immunoglobulins and impaired phagocytosis (Skorepova, 2006).
Diffuse T-cell lymphoma (Welch, 1989), ulcerative colitis (Brunsting, 1949), and HIV infection (Potekaev, 1991) 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 (Ahn, 2004). 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.
Frequency
United States
This condition is rare.
International
This condition is rare.
History
Some patients may have a history of hidradenitis suppurativa (Boyd, 1992; Papadopoulos, 2001).
Physical
Both clinical and histologic findings are necessary to make the diagnosis of PV.
- At clinical examination, multiple pustules and large verrucous plaques with an elevated border are identified.
- At histologic examination, pseudoepitheliomatous hyperplasia is demonstrated.
Causes
The etiology of PV is not known.
Halogenoderma
Keratoacanthoma
Mycobacterium Marinum Infection of the Skin
Pyoderma Gangrenosum
Squamous Cell Carcinoma
Other Problems to be Considered
PV mimics deep fungal infections at clinical examination. As a result of this clinical resemblance, PV has been labeled both mycosislike pyoderma and blastomycosislike pyoderma. North American blastomycosis and pemphigus vegetans, a rare variant of the autoimmune disorder pemphigus vulgaris, also clinically resemble PV. Rarely, other deep fungal infections, including phycomycosis and coccidioidomycosis, mimic the presentation of PV.
Botryomycosis, a rare bacterial infection, may present similarly to a fungal infection and may appear like PV. Cutaneous botryomycosis is differentiated by the presence of basophilic granules that resemble the sulfur granules of actinomyces at histologic examination. Depressed immunity is also common to cutaneous botryomycosis, as seen in acquired immunodeficiency syndrome (AIDS). In 1995, Marschalko et al suggested that cutaneous botryomycosis is a subtype of PV, and they proposed that PV and cutaneous botryomycosis should be classified together.
Bromoderma, mycobacterial infections, and giant keratoacanthomas are also included in the differential diagnosis.
Lab Studies
- Pathogenic organisms, such as Staphylococcus aureus, are grown from wound cultures, and cultures should also be taken for fungi and mycobacteria.
- Direct and indirect immunofluorescence can aid in differentiating PV and pemphigus vegetans (Boyd, 1992; Fujita, 1990).
- Hidradenitis suppurativa may cause an immune complex deposition in the skin, which precipitates the PV reaction. This reaction can be seen as a nonspecific immunoreactant deposition with direct immunofluorescence (Boyd, 1992).
Histologic Findings
Two predominant features are found in PV: pseudocarcinomatous hyperplasia and numerous abscesses in both the hyperplastic epidermis and the dermis. The abscesses can be made up of neutrophils or eosinophils.
Medical Care
No standardized treatment plan is available for PV, although antibiotic treatment has often been used with variable results.
- Topical wound care with copper sulfate or aluminum subacetate dressings and intravenous antibiotics successfully treated 5 of 7 patients with PV (Su, 1979).
- Topical aluminum acetate soaks with intravenous ceftriaxone had moderate success at clearing PV in another patient (Boyd, 1992).
- Over a course of 2 months, the application of zinc oxide along with the intralesional injection of corticosteroids completely cleared lesions on the perianal area of 1 patient (Fujita, 1990).
- A vegetating lesion on the dorsum of a patient's hand was completely cleared with 4 weeks of 2 g of ampicillin daily and x-ray irradiation (Marshalko, 1995).
- In the treatment of 1 patient whose lesions were refractory to antibiotic treatment, topical application of disodium chromoglycate, which is used for the management of chronic leg ulcers (Lip, 1978) and pyoderma gangrenosum (Massone, 1988), induced the healing of lesions (Rongioletti, 1996).
Surgical Care
Antibiotic treatment has been successfully supplemented with laser debridement or curettage to clear lesions (Sawchuk, 1986).
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Topical skin products
Applied locally, these products may dry affected area by reducing the secretory function of the skin glands. They may also toughen the skin to help prevent reinfections, and they may have antiseptic properties.
| Drug Name | Aluminum acetate (Burow solution) |
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| Description | Dissolve aluminum acetate tablets in water to get a 1:10 to 1:40 solution. Has a drying effect on vesicular or wet dermatoses. |
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| Adult Dose | Apply as a compress for 20-30 min 4-6 times/d |
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| Pediatric Dose | Apply as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy | A - Safe in pregnancy
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| Precautions | For external use only |
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| Drug Name | Zinc oxide |
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| Description | Provides relief of minor skin irritations. |
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| Adult Dose | Apply to affected areas prn |
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| Pediatric Dose | Apply as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | For external use only; do not apply to eyes |
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Drug Category: Antibiotics
Therapy must cover all likely pathogens in the context of this clinical setting.
| Drug Name | Ampicillin (Marcillin, Omnipen, Polycillin, Principen, Totacillin) |
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| Description | Bactericidal activity against susceptible organisms. Alternative to amoxicillin when unable to take medication orally. |
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| Adult Dose | 2 g/d PO/IV/IM divided qid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Probenecid and disulfiram increase levels; allopurinol decreases effects and has additive effects on ampicillin rash; may decrease effects of oral contraceptives |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Adjust dose in renal failure; evaluate rash and differentiate from hypersensitivity reaction |
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Further Outpatient Care
- Numerous treatment options are available with mixed results, and these different therapies should be tried if initial therapy fails.
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.
Medical/Legal Pitfalls
- The main medical pitfall is clinically misdiagnosing this rare condition. PV is easily confused with pemphigus vegetans and deep fungal infections. The clinical manifestations and pathohistology are similar for both PV and pemphigus vegetans, but immunofluorescence testing is useful in differentiating between the 2 conditions. In patients in whom inflammatory bowel disease does not coexist, PV has a good prognosis with therapy. In contrast, pemphigus vegetans has a poor prognosis without the continuous use of immunosuppressive drugs.
- Because PV is sometimes a cutaneous sign of cancer, misdiagnosis can be a serious medicolegal issue.
| Media file 1:
A 24-year-old woman with rapidly evolving pyoderma vegetans. |
 |  View Full Size Image | |
Media type: Photo
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Pyoderma Vegetans excerpt Article Last Updated: Feb 1, 2007
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