Pyoderma Vegetans


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. [1234]

Hallopeau first characterized this process in 1898. [5Although 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. [267Pyoderma vegetans has been associated with ulcerative colitis, [891011diffuse T-cell lymphoma, [6alcoholism with malnutrition, [1HIV infection, [12chronic myeloid leukemia (CML), [13and lupus nephritis. [14]

Su et al reported 7 patients with clinically characteristic pyoderma vegetans. [2These 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. [2After 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. [15]

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. [16]

Diffuse T-cell lymphoma, [6ulcerative colitis, [8and HIV infection [12have 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. [17These 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. [18]

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


History

Some patients with pyoderma vegetans (PV) may have a history of hidradenitis suppurativa. [1920This rare inflammatory dermatosis may involve skin and mucosa with pustular and vesicular plaques. In some patients, vegetating plaques may appear in the axillary, vulvar, and inguinal areas. [21Disfiguring facial pyoderma vegetans has been described. [22]

Physical Examination

Both clinical and histologic findings are necessary to make the diagnosis of pyoderma vegetans. The plaques are often multiple; some may appear purulent, others hyperkeratotic. [23At clinical examination, multiple pustules and large verrucous plaques with an elevated border are identified (note the images below). It may rarely appear on the penis. [24At histologic examination, pseudoepitheliomatous hyperplasia is demonstrated.

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

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