Pyoderma gangrenosum

Pulmonary manifestations of pyoderma gangrenosum are relatively rare. We report the case of a 45-year-old patient with multiple pulmonary nodules with central necrosis as assessed by CT scan. The patient had a 4-year history of pyoderma gangrenosum with only minor skin manifestations. A CT-guided, fine-needle biopsy of the lung revealed a nonspecific, inflammatory, aseptic necrotic process, which was comparable to the skin biopsy of one pyoderma lesion. Following the initiation of oral prednisolone therapy, a rapid resolution of the pulmonary nodules occurred. We conclude that pulmonary nodules represent a rare pulmonary manifestation of pyoderma gangrenosum. (CHEST 2001; 119:977-978)

Key words: pulmonary nodules; pyoderma gangrenosum

Pyoderma gangrenosum is a chronic ulcerative skin disease of unknown origin. The disease has been reported in association with several systemic diseases (ie, ulcerative colitis, polyarthritis, and paraproteinemia). However, pulmonary involvement in association with pyoderma gangrenosum is a rare finding. We report a case of a patient with pyoderma gangrenosum and multiple aseptic pulmonary nodules containing central necrosis.

CASE REPORT

A 45-year-old Turkish housewife was admitted to the hospital with a 3-week history of cough, chest pain, and weakness. The patient had a 4-year history of pyoderma gangrenosum and had received intermittent prednisolone treatment. The patient had non-insulin-dependent diabetes mellitus for 2 years, was a nonsmoker, and reported no dyspnea or hemoptysis. A clinical examination was unremarkable except for multiple skin scars due to former episodes of pyoderma gangrenosum. On admission, there was only a small acute lesion on the left breast and a pyodermic lesion on the right lateral side of the tongue.

Chest radiography revealed multiple pulmonary nodules in both lungs. A contrast CT scan of the chest demonstrated multiple pulmonary nodules with central necrosis (Fig 1). The results of pulmonary function tests were normal. The results of all biochemical screening tests were within normal limits, except for slight thrombocytosis (436,000/[micro][L.sup.3], a mild elevation of the C-reactive protein level (40 mg/L), and elevated blood sugar level (153 mg/dL).

[Figure 1 ILLUSTRATION OMITTED]

The results of assays for Ig analysis and rheumatoid factor were within the normal range. The results of tests for both cytoplasmatic antineutrophil cytoplasmatic autoantibody and perinuclear cytoplasmatic autoantibody were negative. The results of testing for antinuclear antibody were positive with a titer of 1:40, and those for extranuclear and anti-DNA antibodies were negative. The results of serologic screening tests for viruses, Mycoplasma, Legionella, Chlamydia, Aspergillus, and Candida were negative. Repeated sputum cultures for aerobic and anaerobic organisms, fungi, and mycobacterial organisms yielded no growth. The results of the testing were also negative. Fiberoptic bronchoscopy showed normal structures within the bronchial system.

BAL fluid from the middle lobe showed 23% lymphocytes, 77% macrophages and monocytes, and [is less than] 1% granulocytes. A further lymphocyte subpopulation analysis of BAL fluid showed a T4/T8 ratio of 1.7:1. The findings of a microbiological examination of the bronchial washing fluid were negative. There was no evidence for involvement of the upper or lower respiratory tract or for renal disease as a possible manifestation of Wegener's granulomatosis. Two-dimensional transthoracic echocardiography showed no pathology at all, especially no evidence of valvular vegetations. There was no evidence of septic emboli. To clearly identify the pulmonary nodules, a CT-guided, fine-needle lung biopsy (20-gauge needle) was performed. The biopsy from one nodule in the right middle lobe revealed an inflammatory aseptic and necrotic process (Fig 2). Six days later, the patient underwent a biopsy of the skin lesion of the left breast with histologic findings comparable to an inflammatory aseptic and necrotic process with extensive infiltration of lymphocytes, neutrophilic granulocytes, and plasma cells. Material from the CT-guided biopsy also was examined for microbial growth, but no bacteria were found.

[Figure 2 ILLUSTRATION OMITTED]

The above findings confirmed the diagnosis of a pulmonary manifestation of pyoderma gangrenosum. Treatment with prednisolone, 100 mg/d orally, was initiated. At 1 week, the patient's symptoms had improved substantially. The size of the pulmonary nodules decreased rapidly over 4 weeks. No nodules were detectable on conventional chest radiography at the 4-week follow-up. Prednisolone was reduced stepwise to 10 mg/d. During a 6-month follow-up, there was no evidence of the reoccurrence of symptoms or of pyoderma manifestations.

DISCUSSION

Since its first description by Brunsting et al[1] in 1930, pyoderma gangrenosum has been reported in association with various systemic diseases with basic immunologic disorders, such as ulcerative colitis, Crohn's disease, polyarthritis, vasculitis, lymphoma, paraproteinemia, leukemia, or active chronic hepatitis.[2]

The patient presented here showed immunologic abnormalities (ie, positive results of tests for antinuclear autoantibodies without the manifestation of other clinical complications except pulmonary nodules). Pulmonary manifestations in pyoderma gangrenosum are rare. With one exception/ pulmonary disease in association with pyoderma gangrenosum occurred as a single unilateral opacity on a chest radiograph.[4-6] Kasuga et al[3] were the first authors to report a patient with multiple pulmonary nodules in association with pyoderma gangrenosum. In this particular case, the nodules were solid and were located in the peripheral portions of the lung.

The finding of multiple pulmonary nodules with central necrosis in both lungs in association with pyoderma gangrenosum has not yet been described. Histologic examination of the pulmonary manifestations of pyoderma gangrenosum revealed aseptic inflammatory lesions similar to the findings presented here. As reported earlier, the pulmonary lesions rapidly decreased with steroid therapy.

In our patient, the histologic findings of the skin biopsy corresponded closely to the findings of the lung biopsy. CT-guided biopsies of lung lesions have a very high sensitivity and specificity. In a study by Belfiore et al,[7] the cytologic diagnosis of CT-guided, fine-needle biopsy was confirmed in all 267 patients who underwent surgical or clinical follow-up. The ideal evaluation of these necrotic lung lesions would have been the acquisition of larger pieces of tissue (eg, by surgical lung biopsy). However, the patient refused this procedure and only gave consent for CT-guided, fine-needle biopsy.

With regard to the central necrotic pulmonary nodules detected on the CT scan, the most favorable radiologic diagnosis would have been Wegener's granulomatosis. However, Wegener's granulomatosis as well as septic emboli, sarcoidosis, tuberculosis, and metastatic malignant disease were ruled out by histologic and clinical findings.

In patients with pyoderma gangrenosum with the presence of radiologically visible multiple central necrotic pulmonary nodules, a pulmonary manifestation of the underlying disease should be considered

REFERENCES

[1] Brunsting LA, Goeckerman WE, O'Leary PA, Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol 1930; 22:655-661

[2] Holt PJA, Davies MG, Saunders KC, et al. Pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine 1980; 59:114-133

[3] Kasuga I, Yanagisawa N, Takeo C, et al. Multiple pulmonary nodules in association with pyoderma gangrenosum. Respir Med 1997; 91:493-495

[4] Lebbe C, Moulonguet-Michau I, Perrin P, et al. Steroid-responsive pyoderma gangrenosum with vulvar and pulmonary involvement. J Am Acad Dermatol 1992; 27:623-625

[5] Mc Culloch AJ, Mc Evoy A, Jackson JD, et al. Severe steroid responsive pneumonitis associated with pyoderma gangrenosum and ulcerative colitis. Thorax 1985; 40:314-315

[6] Vignon-Pennammen MD, Zelinsky-Gurung A, Janssen F, et al. Pyoderma gangrenosum with pulmonary involvement. Arch Dermatol 1989; 125:1239-1242

[7] Belfiore G, Di Filippo S, Guida C, et al. CT-guided biopsy of lung lesions. Nucl Med Biol 1994; 21:713-719

(*) From the Medical Clinic I (Drs. Kruger, Breuer, and Schwarz), Department of Radiology (Dr. Piroth), and Institute of Pathology (Dr. Takyi), University Hospital, University of Technology, Aachen, Germany.

Manuscript received May 17, 1999; revision accepted August 31, 2000.

Correspondence to: Stefan Kruger, MD, Medizinische Klinik I, Universitatsklinikum Rheinisch Westfalische Technische Hochschule, PauwelsstraBe 30, 52057 Aachen, Germany

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