CLINICAL HISTORY:

Signs and symptoms:  A 60 year-old Hispanic female with a two-year history of idiopathic crescentic glomerulonephritis presented with chest pain and shortness of breath. Cardiac ischemia was excluded with serial lab studies and intravenous heparin was started for a potential pulmonary embolism. On hospital day three, subcutaneous nodules were noted on her forehead. These nodules rapidly increased in size and number over a 24-48 hour period. They spread rapidly over the face, neck, upper trunk, and upper extremities. The subcutaneous nodules transitioned to exophytic and weeping lesions. On hospital day seven, she developed a productive cough with blood-tinged sputum and a chest radiograph showed diffuse bilateral infiltrates. A pulmonary angiogram was negative for an embolism and anticoagulation was stopped. A bronchoscopy revealed nodules lining the bronchi and diffuse alveolar hemorrhage.

Previous Treatment: 

Other information: 

PHYSICAL EXAM:

Subcutaneous nodules becoming exophytic and weeping with crust over face, neck, upper trunk, and upper extremities. There was a 2cm. hemorrhagic bullous lesion on the left palm. A few oral mucosal ulcers were present.

LABORATORY TESTS:

PAS, Gram’s stain, Fite stain, and cultures were all negative. Leukocytosis with a normal eosinophil count. ESR and CRP elevated. Positive P-ANCA and C-ANCA

DERMATOHISTOPATHOLOGY:

Microscopic description: Biopsies showed a large amount of tissue edema, full thickness necrosis of a medium sized arteriole, neutrophilic infiltrate, and karyorrhexis.

DIFFERENTIAL DIAGNOSIS:

1.   Churg-Strauss Syndrome
2.   Lymphomatoid Granulomatosis
3.   Wegener’s Granulomatosis
4.   Temporal Arteritis
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Wegener’s Granulomatosis

DISCUSSION:

Wegener’s Granulomatosis is an idiopathic systemic vasculitis with classic involvement of the upper and lower respiratory tracts and the kidneys, yet any organ system can be involved. Cutaneous manifestations of Wegener’s Granulomatosis are uncommon as the initial presentation, occurring in only 13 percent, but are present in 30-50 percent of patients throughout the course of the disease. The skin lesions are non-specific and include palpable purpura, necrotizing ulcers, papules, nodules, vesicles, bullae, petechiae, pustules, macules, pyoderma gangrenosum, erythema, and ecchymosis. Unlike our patient, the skin disease is usually minor and rarely dominates the clinical picture. Skin involvement indicates active disease, parallels disease activity in other organs, and usually responds well to treatment. No skin biopsy is diagnostic of Wegener’s Granulomatosis. Only 14-28 percent of skin biopsies demonstrate a specific histopathology. There is a stronger correlation between active disease and a specific histopathologic finding. Necrotizing vasculitis is the most frequently reported specific biopsy. Biopsies from other organ systems are no better than skin biopsies, which are easier and less invasive to obtain. The clinical presentation of Wegener’s Granulomatosis is very diverse which may delay the diagnosis. The differential diagnosis includes infections, Henoch Schonlein purpura, Behcet’s syndrome, systemic lupus erythematosis, sarcoid, microscopic polyarteritis, and drug reactions. Although no cutaneous lesion or histopathologic finding is specific for Wegener’s Granulomatosis, together with other clinical and radiographic information, the lesions can facilitate an early diagnosis.

TREATMENT:

Actual treatment for this patient:

Prednisone 60mg/day and cyclophosphamide 1.5mg/kg/day. There was significant improvement after 2 weeks of treatment with no new lesions developing. The lesions healed with scarring after 6 weeks of treatment.

REFERENCES:

1. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener Granulomatosis: An analysis of 158 patients. Ann Intern Med. 1992;116:488-498.


2. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s Granulomatosis: Prospective Clinical and Therapeutic Experience with 85 Patients for 21 years. Ann Intern Med. 1983;98:76-85.

3. Daoud MS, Gibson LE, DeRemee RA, Specks U, el-Azhary RA, Su WP. Cutaneous Wegener’s Granulomatosis: Clinical, Histopathologic, and Immuunopathologic Features of Thirty Patients. J Am Acad Dermatol. 1994;31:605-612.

4. Patten SF, Tomecki KJ. Wegener’s Granulomatosis: Cutaneous and Oral Mucosal Disease. J Am Acad Dermatol. 1993;28:710-718.

5. Frances C, Huong Du LT, Piette JC, Saada V, Boisnic S, Wechsler B, et al. Wegener’s Granulomatosis: Dermatological Manifestations in 75 Cases With Clinicopathologic Correlation. Arch Dermatol. 1994;130:861-867.

6. Hu CH, O’Loughlin S, Winkelmann RK. Cutaneous Manifestations of Wegener’s Granulomatosis. Arch Dermatol. 1977;113:175-182.

7. Barksdale SK, Hallahan CW, Kerr GS, Fauci AS, Stern JB, Travis WD. Cutaneous Pathology in Wegener’s Granulomatosis: A Clinicopathologic Study of 75 Biopsies in 46 Patients. Am J Surg Pathol. 1995;19(2):161-172.

8. Stein SL, Miller LC, Konnikov N. Wegener’s Granulomatosis: Case Report and Literature Review. Pediatr Dermatol. 1998;15:352-356.

Additional Comment:

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