Cutaneous vasculitis associated with Pheochromocytoma

Abstract

We describe a patient who presented with constitutional symptoms, severe hypertension, and purpuric lesions over the knees, thighs, and penis. The patient was eventually diagnosed as having multiple endocrine neoplasia type II, with cutaneous leukocytoclastic vasculitis. The cutaneous vasculitis persisted despite treatment with highdose systemic corticosteroids, but rapidly resolved after the removal of bilateral pheochromocytomas. This case demonstrates cutaneous leukocytmlastic vasculitis in association with pheochromocytoma.Necrotizing vasculitis is a term used to describe the inflammation and necrosis of blood vessels that is seen in a broad spectrum of disorders. The hypersensitivity group of the necrotizing vasculitides is commonly encountered (1) and is characterized by small vessel involvement. Hypersensitivity vasculitis, or leukocytoclastic vasculitis (LV), as it is commonly called, has many potential etiologies (2). Among the possible causes of LV are drugs, infections, connective tissue disease, or on rare occasions, malignancy (3). We describe a patient who had cutaneous LV in association with multiple endocrine neoplasia (MEN) type 11, and the LV was related, specifically, in our opinion, to bilateral pheochromocytomas. To our knowledge, this is the first report of LV associated with pheochromocytoma. Case report. The patient, a 32-year-old white heterosexual man, presented in January 1987 with a 3 4 d a y history of fever, chills, headache, anorexia, nausea, vomiting, arthralgias, myalgias, heat intolerance, and lesions on his legs. His medical history was significant for acute hepatitis B infection in August 1986, moderate alcohol use until the diagnosis of hepatitis, peptic ulcer disease in 1984, and congenital duodenal atresia. During hospitalization in 1986, he was normotensive, and his medications at the time of this admission included promethazine, acetaminophen, and aspirin.On admission to the hospital, the patient was thin and flushed, but well-oriented. His blood pressure was 210/150 mm Hg, heart rate was 128 beatdminute, and temperature was 993°F. His sclerae were ictenc, and there were retinal hemorrhages in both eyegrounds. He had a grade IYVI systolic ejection murmur at the left upper sternal border, which radiated into the carotid arteries bilaterally. The abdomen was diffusely tender, but there was no rebound tenderness. His bowel sounds were normal. Tender, slightly palpable, reticulated purpuric lesions were distributed over the penile shaft, thighs, and knees (Figure 1). Seven cafk-au-lait spots, measuring 2.0-5.0 cm in diameter, were present on the patient's trunk and extremities, and axillary freckling was noted. Physical examination results were negative for thyromegaly , mucosal neuromas, neurofibromas, and localizing neurologic signs.Laboratory evaluation at admission showed the following values: white blood cell count 20,8W/mm3, with a normal differential cell count, erythrocyte sedimentation rate (Westergren) 34 mm/hour (normal 0-20), blood urea nitrogen 72 mg/dl (normal 5...

The Spectrum of Paraneoplastic Cutaneous Vasculitis in a Defined Population

“…Table 4 shows a series of cases of cutaneous vasculitis as a presenting manifestation of an underlying solid malignancy. 1 , 9 , 12 , 15 , 16 , 21 , 23 , 25 , 26 , 30 , 32 , 39 , 42 , 43 , 47 , 53 , 54 , 56 , 57 , 59 , 66 , 69 , 73 – 75 , 77 , 83 , 85 , 86 , 89 , 91 , 96 …”

Cutaneous small-vessel vasculitis associated with solid organ malignancies: The Mayo Clinic experience, 1996 to 2009

“…[1][2][3][4][5]13,34,35 Also, there are reports of CSVVoccurring less commonly with other solid organ malignancies, including ovarian cancer, 32 hepatocellular carcinoma, 30 pancreatic cancer, 27 and pheochromocytoma. 28 We describe 17 cases of CSVV associated with solid organ malignancy seen at Mayo Clinic between 1996 and 2009. We also provide a thorough review of the literature regarding the relationship between CSVV and solid organ malignancy.…”

Paraganglioma E Angioedema

“…In sum, we report on an extremely unusual case of a patient with an extra-adrenal pheochromocytoma (paraganglioma), in whom angioedema episodes were synchronous with paroxystic systemic crises and that experienced no further crises, ever since surgical removal of the tumour was undertaken, 17 years ago. • Necrotizing vasculitis 11 • Violaceous macules on the feet • Palmar plantar keratoderma 16 • Addison-like hypermelanosis 17 • Granulosis Rubra Nasi 18 • Leukocytoclastic vasculitis 19 • Chronic urticaria 20…”