A 70+ year old female presents with a new eruption. She had a significant history of uncontrolled urticaria that had spontaneously disappeared 6 months prior. Patient feels as though her hives returned after gardening outdoors in the sunshine. She denies systemic symptoms, but states the lesions are tender and itch. Patient denies recent infection, weight loss, cough, dyspnea, headaches, and abdominal pain. Exam shows warm, edematous pink plaques with dusky concentric rings on trunk and extremities.
Labs were ordered (CBC W Auto Differential, CMP, ANA, DSDNA, SSA, SSB, SM/RNP, RNP ab). ANA was found to be positive 1:80 positive speckled (pt had underlying autoimmune disease ruled out in the previous 6 months by rheumatology after previously elevated ANA), WBC elevated, all other labs stable. Biopsy was performed at office visit.
What systemic screening would you order?
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Screen for infections such as hepatitis, drug review, complement levels, ANA screen, SPEP and malignancy screening/imaging.
Sections show a normal cornified layer overlying a normal thickness epidermis. There is a superficial perivascular and interstitial inflammatory infiltrate. The infiltrate is composed of lymphocytes, numerous eosinophils, and scattered neutrophils. There are extrasavated red blood cells and focal leukocytoclasia without evidence of vascular damage. No fungal forms were seen on PAS stain. Both neutrophil dermatosis and gyrate erythema including Erythema Gyratum Repens may be considered. The eosinophils and neutrophils would be uncommon findings in gyrate erythema. The clinical appearance and presence of red blood cells favor urticarial vasculitis. Often there is no true vasculitis evidenced by fibrin deposition in urticarial vasculitis and the diagnosis depends on correlation between the clinical and histologic findings.
DIAGNOSIS & CASE SUMMARY:
Urticarial vasculitis is considered a small vessel vasculitis that can be divided into normal vs. low complement levels. Patient with low complement can be associated with autoimmune disease such as lupus. All patients should be questioned about systemic symptoms of abdominal pain, chest pain, joint pain, fever and angioedema. Clinically, lesions last longer than 24 hours, versus other urticarial eruptions.
Patients should be screened for connective tissue disease, gammopathies, and viral infections. Certain drug-induced cases are associated with antibiotics, ACE inhibitors, thiazides, and fluoxetine. Malignancy screening is important, as in the case of this patient.
When this case study was shared internally, another Forefront Dermatologist suggested an additional PET/CT scan even though the patient repeatedly denied dyspnea, abdominal pain, fatigue, weight loss, health changes. A CT was ordered and identified a 2.1cm right upper lobe pulmonary nodule and some shotty lymphadenopathy in the left subpectoral region. Pt was notified and she remembers getting her flu shot a few days before the CT in her left arm, thought to be the cause of the lymphadenopathy. She denied dyspnea, cough, fatigue, or weight loss. Former cigarette smoker until age 26. The patient was referred to oncology who did a Full body PET and abdominal CT with contrast to rule out malignancy, all of which were negative. Needle biopsy of lung nodule diagnosed of Carcinoid Tumor of the Lung. Patient is pursuing treatment of Carcinoid tumor and rash resolved on prednisone.
By: Dr. Betsy Wernli, MD; Ms. Rikki Hammel, NP; and Dr. Kelli Hutchens, MD