Clinical Case Vignettes: Vasculitis



Fig. 42.1
Purpuric papules over the tip of the toes in a 9-year-old girl



Complete blood count, prothrombin time, partial thromboplastin time, and fibrinogen level were within normal limits. The results of laboratory evaluation of a possible hypercoagulable state, including protein C, antithrombin III, homocysteine, and factor V Leiden, were negative. Echocardiogram and CT angiography of both lower limbs were normal.

A dermatologist suggested a possibility of chilblains. On further questioning, she admitted to hiking in the snow a day before onset of lesions. The patient was discharged with close followup. She was advised to avoid contact sports, to keep extremities warm, and to limit cold exposure. She was allowed weight bearing as tolerated and treated with acetaminophen as needed for pain. The lesions resolved within 1 week.

She had a second episode 1 year later with similar lesions on the toes of her left foot after wearing opentoed shoes at an outdoor winter school function. Her lesions resolved after 2 weeks. She was counseled to avoid cold exposure, smoking, obesity, prolonged sitting, use of estrogens (birth control pills), and other factors that increase the risk for thrombosis.




Differential Diagnosis


The differential diagnoses for purpuric or petechial lesions on bilateral toes are hypercoagulable conditions, thrombotic or embolic process, vasculitis, and chilblains (also known as pernio or perniosis). Perniosis associated with lupus erythematosus is probably the most important condition in differential diagnosis because cutaneous lesions can be similar. Therefore, it is beneficial to do antinuclear antibody screen when there is a clinical suspicion or family history of a rheumatologic disorder.

Chilblains are itchy, tender, red or purple papules that occur in response to prolonged cold exposure. The lesions start as red areas with itching and burning a few hours after cold exposure and typically resolve in 7–14 days but may thicken and persist for months [9]. The small blood vessels constrict with cold exposure, and the blood leaks into surrounding tissues with rewarming. It is more common in children, women, and elderly people. It is seen almost exclusively in the winter months and in damp or wet, cold conditions. There can be a positive family history of chilblains. It has also been associated with smoking, hyperlipidemia, low body weight, poor nutrition, connective tissue diseases (most commonly lupus), cryoglobulins, and cold agglutinin disease [10]. Lesions most commonly occur on the fingers, toes, nose, and ears.

Chilblains are usually a clinical diagnosis; however, if necessary, a skin biopsy may be performed. It is also important to rule out associated conditions. It responds poorly to treatment, but the lesions usually resolve in 7–14 days without any specific treatment. Treatment options include a 2- to 3-day course of topical corticosteroid cream, which may also help with itching and swelling. Topical or oral antibiotics may be used for any secondary infection. For chilblains, prevention is the key, including avoidance of smoking, secondhand smoke exposure, or medications that cause blood vessel constriction, such as decongestants. Keeping the extremities warm and dry and wearing wool or moisture-resistant socks and mittens may help prevent development of new lesions or exacerbation of existing ones. In extreme cases, nifedipine (vasodilator) may be prophylactically prescribed before the onset of cold weather and taken throughout the winter season [11]. Prognosis is very good in children with complete recovery seen in a majority [12].


Take-Home Messages




  1. 1.


    Chilblains are benign and self-limiting.

     

  2. 2.


    Diagnosis is clinical, but other causes of vasculitis must be ruled out.

     


Clinical Case

A 6-year-old boy presented with high-grade fever and purpuric rash over both legs of 2 weeks duration. On examination, he looked pale, sick, and tachypneic. He had vasculitic lesions over the pulps of his fingers. The rest of systemic examination was within normal limits. Laboratory examination revealed anemia with polymorphonuclear leukocytosis (white cell count 19,800 cells/μl with 68 % polymorphs). Platelet count was normal. Serum creatinine was raised at 1.8 mg/dL with blood urea of 54 mg/dL. Urine examination revealed 2+ proteinuria and microscopic hematuria. Inflammatory parameters were raised with ESR of 96 mm/h and CRP of 140 mg/dL. Chest radiography showed two cavitating lesions in the right lung. Ultrasound examination disclosed normal kidney size with increased renal parenchymal echogenicity.

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Oct 25, 2017 | Posted by in RHEUMATOLOGY | Comments Off on Clinical Case Vignettes: Vasculitis

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