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Cutaneous vasculitis associated with pheochromocytoma.
C L Kulp-Shorten1, R H Rhodes, H Peterson
1Division of Dermatology, School of Medicine, University of Louisville, Kentucky 40202.
Arthritis and Rheumatism
|December 1, 1990
Summary
A patient with multiple endocrine neoplasia type II experienced purpuric skin lesions and severe hypertension. Removal of pheochromocytomas resolved the cutaneous leukocytoclastic vasculitis, highlighting this rare association.
Area of Science:
- Endocrinology
- Dermatology
- Oncology
Background:
- Multiple endocrine neoplasia type II (MEN2) is a rare genetic disorder characterized by tumors in endocrine glands.
- Cutaneous leukocytoclastic vasculitis is an inflammation of small blood vessels in the skin.
- Pheochromocytomas are tumors of the adrenal medulla that produce excess catecholamines, leading to hypertension.
Observation:
- A patient presented with constitutional symptoms, severe hypertension, and purpuric lesions on the knees, thighs, and penis.
- Initial presentation suggested a systemic inflammatory process.
Findings:
- The patient was diagnosed with MEN2 and cutaneous leukocytoclastic vasculitis.
- Vasculitis persisted despite high-dose systemic corticosteroid therapy.
- Rapid resolution of cutaneous vasculitis occurred after bilateral pheochromocytoma removal.
Implications:
- This case highlights a rare association between pheochromocytoma and cutaneous leukocytoclastic vasculitis in MEN2.
- Pheochromocytoma should be considered in the differential diagnosis of unexplained vasculitis, particularly in MEN2 patients.
- Surgical management of pheochromocytoma may be crucial for resolving associated vasculitis.