Sec 28 The Skin in Inflammatory and Other Vascular Disorders

Question 1

Erythematous papules that do not blanch when the skin is pressed

Answer 1

Palpable purpura

Question 2

A multisystem vasculitis of small vessels and sometimes also of medium-sized vessels. Pauciimmune glomerulonephritis develops in the majority of patients, and pulmonary hemorrhage, peripheral and cranial neuropathy, musculoskeletal, and constitutional symptoms are also common; cardiac and gastrointestinal involvement are less common.

Answer 2

Microscopic polyangiitis

Question 3

Encompasses all the features of microscopic polyangiitis but also many additional manifestations caused by necrotizing granulomatous inflammation. Chronic inflammation of the upper airway (nasal cavity, sinuses, auditory tube, and middle ear) is present in about 90% of patients, often but not always as the initial manifestation. Vasculitis of the eye (scleritis and episcleritis) is also much more common. The majority ANCA type in this disease is C-ANCA/ antiproteinase-3 (PR3).

Answer 3

Granulomatosis with Polyangiitis

Question 4

The most prominent being a history of asthma (often severe or poorly controlled), and blood eosinophilia. Nasal polyps, constitutional symptoms, and rashes, all typical of atopy, are also common. The presence of pulmonary infiltrates on chest imaging (eosinophilic pneumonia) provides an important distinction from asthma. The most common presentation of severe vasculitis is acute peripheral neuropathy with involvement of the heart, gastrointestinal tract, brain, and eyes less common.

Answer 4

Churg-Strauss Syndrome

Question 5

Strictly a disease of adults older than 50 years of age that is markedly more common with advancing age. It is mostly a disease of people of Northern European ancestry. Cranial arteritis is a common feature with this term indicating stenosis or occlusion of one or more branches of the carotid artery to produce headache (70%–80%), jaw claudication (50%), and monocular (and rarely binocular) blindness (15%). Polymyalgia rheumatica, which includes pain and stiffness of the shoulder and hip girdles, is seen in at least 30%–40% of patients and may occur without cranial disease. Diagnosis is usually confirmed by temporal artery biopsy

Answer 5

Giant Cell Arteritis

Question 6

A rare form of vasculitis (prevalence <1:100,000) involving the aorta and its major branches. 64–69 Many patients are diagnosed as young adults, and 90% are female. The typical presentation of Takayasu’s is limb claudication. Dizziness, constitutional symptoms, and severe hypertension (from renal artery stenosis) are also common. Cerebral infarction can occur. Coronary occlusions with angina or infarction and bowel ischemia are less common but life-threatening complication. Absent pulse(s), abnormal blood-pressure readings, and arterial bruits are common but not universal findings. Diagnosis is made by angiography.

Answer 6

Takayasu Arteritis