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  • Neoplasia and infectious diseases were ruled out and

    2018-11-15

    Neoplasia and infectious diseases were ruled out, and the patient\'s condition was diagnosed as vegetative PG. This diagnosis was based on the clinical appearance of the vegetative nodular and plaque lesions with superficial ulcers and by the neutrophilic abscess formation without vasculitis revealed during the pathology examination. He was treated with methylprednisolone at an initial dosage of 60 mg/d for 15 days, with no improvement. The methylprednisolone dosage was then increased to 80 mg/d for 10 days, and the lesions markedly improved. The dosage was then tapered to 60mg/d over 14 days, and the lesions markedly diminished and gradually healed. After several reductions of the methylprednisolone dose over another 2 months, a high degree of cribriform scarring developed at the sites of the previous lesions (Figure 1E). The steroid was continued but in tapering dosages. Eight months ago, the prednisone dose was tapered from 25 mg/d to 20 mg/d. However, after about 2 weeks at this final dosage, the patient presented with similar lesions on the trunk, so the prednisone dosage was increased to 40 mg/d for 1 month. The lesions regressed. The prednisone was continued but again at tapering doses until it reached 25 mg/d 1 month ago. To date, the patient has been followed-up for 2 years with no other underlying disease found.
    Discussion Vegetative PG, also termed superficial granulomatous pyoderma, is a recently recognized variant of PG with unique clinical, histological, and therapeutic characteristics. Characteristically, these lesions begin as a single furunculoid purple abscess, nodule, or plaque. They are most commonly present on the trunk. Over time, they evolve in an indolent fashion into a granulomatous or pyodermatous vegetative lesion with associated sinuses and cribriform scarring. Superficial, focal abscesses surrounded by peripheral palisading histiocytes and foreign body giant URB597 may be seen on histological examination. Cultures for deep fungal infection, mycobacterium, and atypical mycobacterium are negative. Vegetative PG is responsive to simple therapeutic modalities. It is not associated with any underlying systemic diseases. Our patient exhibited some unusual characteristics compared with previously described patients with vegetative PG. First, the lesions comprised multiple slowly enlarging pustules, papules, nodules, plaques, and ulcers. In particular, several nodular lesions and plaques 2–12 cm in diameter were seen on the trunk and left lower extremity. The plaques\' appearance mimicked that of neoplastic lesions such as malignant lymphoma, but there was no histopathological evidence of neoplasia. The plaques in our case were similar to those in previous reports, but in our case they were more proliferous. Second, the lesions involved almost the entire body, including the scalp, face, neck, trunk, and extremities. Lesions of vegetative PG most commonly involve the trunk, although other locations such as the face, extremities, and scrotum have been described. To the best of our knowledge, no previous reports have described lesions involving the entire body. Third, the patient\'s therapeutic response was unusual. Most cases of vegetative PG are relatively benign and respond to conservative therapy, including topical and intralesional steroids. Although some are refractory, these lesions uncommonly require oral corticosteroids and other immunosuppressive agents. Several reports have described patients with vegetative PG that required more aggressive systemic immunosuppressive agents. These cases were responsive to intravenous immunoglobulin, cyclosporine, or infliximab. The lesions in our case markedly abated with intravenous methylprednisolone at a dosage of 80 mg/d. They had not responded to a 15-day course of methylprednisolone at 60 mg/d. To diagnose vegetative PG with confidence, we excluded infections with similar clinical appearances. We performed excretion and tissue cultures for fungus. The culture results were negative. Chest radiography and a tuberculin test failed to show any signs of tuberculosis. As periodic acid-Schiff and acid-fast bacilli staining also gave negative results, we excluded deep fungal infections and tuberculosis. In patients with impaired immunity, blastomycosis-like pyoderma may result from an abnormal response to bacterial invasion of the epidermis. We evaluated several smears and cultures for bacteria. S. aureus and B. proteus were cultured only once, so we considered them contaminating bacteria. The patient received ofloxacin and fosfomycin treatment early in the course of his treatment, but his condition only worsened. The antibiotic treatment was discontinued when vegetative PG was diagnosed. Methylprednisolone treatment was commenced, and the lesions markedly diminished. Also, the patient\'s systemic examination proved normal, and he had no other diseases. We therefore excluded blastomycosis-like pyoderma.