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Home , Bruise, Purpura fulminans

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Diana M. Sarmiento, MD; Thomas F. Northrup, PhD; on the ears and body Yu Wah, MD, ABIHM University of Texas Health Could this patient’s worsening bruises be explained by Science Center at Houston her history of drug use? [email protected] DEPARTMENT EDITOR Richard P. Usatine, MD University of Texas Health Science Center at San Antonio Over the course of a month, this 34-year- On her most recent visit, her vitals and The authors reported no potential old woman had sought care at our facility— physical examination were unremarkable, conflict of interest relevant to this and another—on 3 separate occasions for apart from the findings. Her complete article. painful bruises (visits #1 and #3) and deep vein blood count, complete metabolic panel, and (DVT; visit #2). The bruises first urinalysis were unremarkable. On her previ- appeared acutely on her arms (FIGURE 1A), ous admissions, the patient’s urine drug test prompting her first visit to our ED and leading had been positive for cocaine. She’d also tested to a hospital stay. Several weeks later, the pa- positive for cytoplasmic antineutrophil cy- tient developed new -like lesions on her toplasmic antibodies (c-ANCA), antinuclear earlobes (FIGURE 1B), face, trunk, and lower antibodies (ANA), anti-double stranded DNA extremities. In between these 2 visits, the pa- (anti-dsDNA), and anticardiolipin IgM. tient was seen in another ED (and admitted) for right upper extremity DVT and was started on enoxaparin, followed by . The patient had no history of trauma, but ● WHAT IS YOUR DIAGNOSIS? did have a 7-year history of cocaine abuse. The initial bruises appeared one week after using ● HOW WOULD YOU TREAT THIS cocaine from a different dealer. PATIENT?

FIGURE 1 Bruising on arm and earlobe A B PHOTOS COURTESY OF: DIANA M. SARMIENTO, MD

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Diagnosis: Levamisole-induced and penis. Microscopic examination reveals cutaneous vasculopathy bland thrombi with no of the The patient was given a diagnosis of vessel wall.5 levamisole-induced cutaneous vasculopathy ❚ ANCA-associated small-vessel vascu- based on her history of cocaine use; the typi- litis includes microscopic polyangiitis, Weg- cal, painful palpable with angulated ner’s granulomatosis, and Churg-Strauss syn- borders and a necrotic center (retiform pur- drome. With these conditions, palpable pur- pura); and positive immunologic markers.1-5 puras are more commonly seen in areas that DVT has also been reported in association are dependent on, or affected by, venous sta- with levamisole-induced vasculopathy.6 sis. Microscopic evaluation reveals leukocy- ❚ Intended for livestock. Levamisole is a toclastic . Perinuclear ANCA is more pharmaceutical agent typically used as an an- often positive than c-ANCA.5 thelmintic in livestock, but, since 2007, it has ❚ is a medical emer- increasingly been found as an adulterant in gency characterized by skin and dis- cocaine.7 According to the Drug Enforcement seminated intravascular . It can Administration, 71% of cocaine tested in 2009 rapidly lead to multi- failure. Micro- contained levamisole.7 scopic evaluation reveals thrombotic occlu- Experts speculate that levamisole is used sion of small- and medium-sized vessels. It af- in the production process of cocaine to in- fects neonates and children, and is associated While crease its volume and enhance its psychoactive with severe or an autoimmune response levamisole is a effects.1-4 In humans, levamisole’s immuno- to an otherwise benign childhood infection. It pharmaceutical modulatory properties were once used to treat may also be a symptom of hereditary agent typically various cancers and immunologic conditions, or deficiency.8 used as an but it was withdrawn from the US market in ❚ Cholesterol emboli are more common anthelmintic 2000 due to adverse effects.1,2,4 Severe adverse in men ages 50 and older with atherosclerotic in livestock, it reactions associated with levamisole include disease, hypertension, or tobacco use. Abrupt has increasingly agranulocytosis, vascular occlusive disease, onset of livedo reticularis may be followed by been found as and thrombotic vasculopathy with or without retiform purpura, ulcers, nodules, and gan- an adulterant vasculitis.1,2 grene. Lesions most often appear on the dis- in cocaine. The incidence of levamisole-induced cu- tal lower extremities and buttocks. Systemic taneous vasculopathy is unknown. That said, symptoms may include fever, weight loss, it’s important to suspect the condition in co- myalgia, and altered mental status. Multiple caine users who present with retiform pur- organ systems may be involved. Frozen sec- pura. Earlobe lesions are characteristic, while tions reveal needle-shaped clefts and doubly- involvement of other areas is variable.2-4 refractile crystals.5

Differential includes other Evolving skin lesions, causes of purpura relevant lab findings A number of conditions make up the differen- Initially, patients with levamisole-induced tial. While each of these presents with areas of cutaneous vasculopathy will develop pain- skin necrosis or purpura, a thorough history ful, palpable purpura with angulated borders can be revealing. Factors such as substance and a necrotic center. The lesions can prog- abuse, recent treatment with a vitamin K an- ress, though, to bullae, necrosis, or eschar tagonist, or even a recent infection can be the formation.1,3,4 key to making the diagnosis. Patients with this condition frequently ❚ Warfarin-induced skin necrosis pres- test positive for ANCA and, even more ents with large, irregular bullae that even- frequently, for c-ANCA, ANA, antiphos- tually become necrotic. It appears one to pholipid antibodies, leukopenia, and neu- 10 days after treatment with a vitamin K tropenia. Other immunologic markers for antagonist and typically affects areas of the which patients may test positive include anti- body with greater subcutaneous adipose tis- cardiolipin antibodies, lupus , sue, including the breasts, thighs, buttocks, anti-dsDNA, myeloperoxidase, and anti-

574 THE JOURNAL OF FAMILY PRACTICE | SEPTEMBER 2017 | VOL 66, NO 9 Visit us @ jfponline.com

Sjögren’s-syndrome-related antigen A (also known as anti-Ro) antibodies.1-4 Natural progression of levamisole- induced cutaneous vasculopathy is generally benign. Most clinical signs and symptoms— as well as serologic manifestations—resolve What FPs need to know without intervention after cessation of cocaine IN about naloxone kits use. However, signs and symptoms may recur 3 3 Edwin A. Salsitz, MD VIDEO with subsequent exposure. 3 take-home points There is no specific therapy for levamisole- in 3 minutes (or less) induced cutaneous vasculopathy, but pred- nisone and other immunosuppressive agents can be used in patients with severe or systemic symptoms.1-4 Necrotic lesions and eschar for- mation may be complicated by infection and require debridement and/or skin grafts. Need an edge with T2DM? ❚ Our patient was discharged after a brief hospital stay, as she had no indication of sys- The case for team-based care temic involvement and no new or worsening  Doug Campos-Outcalt, MD, MPA skin lesions. She was given care instruc- tions and advised to stop using cocaine. The INSTANT POLL patient was counseled at bedside by a physician and given information on community resources What is the single biggest challenge you face (outpatient treatment, support groups, etc) by in lowering blood pressure in your patients social services. However, the patient continued ≥75 years of age? to use the substance and had several readmis- sions with worsening skin lesions complicated ONLINE EXCLUSIVES by secondary bacterial infection. She did not have systemic complications, but required anti- • APPLIED EVIDENCE Individualizing immunization for international biotics, multiple wound debridement sessions, travelers and subsequent skin grafts. JFP • CASE REPORT CORRESPONDENCE Yu Wah, MD, ABIHM, University of Texas Health Science Cen- Abdominal pain and distention • nausea ter at Houston, 6431 Fannin Street, Suite JJL 308, Houston, • declining appetite • Dx? TX 77030; [email protected]. •  PHOTO ROUNDS Periorbital ecchymoses and breathlessness • Dx? References • HELPDESK ANSWERS 1. Gaertner EM, Switlyk SA. Dermatologic complications from levam- isole-contaminated cocaine: a case report and review of the litera- Does treating obstructive sleep apnea improve ture. Cutis. 2014;93:102-106. control of Tx-resistant hypertension? 2. Strazzula L, Brown KK, Brieva JC, et al. Levamisole toxicity mimick- ing autoimmune disease. J Am Acad Dermatol. 2013;69:954-959. PHOTO ROUNDS FRIDAY  3. Espinoza LR, Perez Alamino R. Cocaine-induced vasculitis: clinical and immunological spectrum. Curr Rheumatol Rep. 2012;14:532- 538. Test your diagnostic skills at www.jfponline.com/ 4. Chung C, Tumeh PC, Birnbaum R, et al. Characteristic purpura of articles/photo-rounds-friday.html the ears, vasculitis, and neutropenia–a potential public health epi- demic associated with levamisole-adulterated cocaine. J Am Acad Dermatol. 2011;65:722-725. PLUS  5. Wysong A, Venkatesan P. An approach to the patient with retiform purpura. Dermatol Ther. 2011;24:151-172. Today’s headlines in family medicine 6. Wilson L, Hull C, Petersen M, et al. End organ damage in levamisole adulterated cocaine: More than just purpura and agranulocytosis. J Am Acad Dermatol. 2013;68:AB9. GET UPDATES FROM US ON 7. US Department of Justice. National Drug Threat Assessment 2010. Impact of drugs on society. Available at: http://www.justice.gov/ar- chive/ndic/pubs38/38661/drugImpact.htm. Accessed July 26, 2017. FACEBOOK TWITTER GOOGLE+

8. Chalmers E, Cooper P, Forman K, et al. Purpura fulminans: recogni- www.facebook.com/JFamPract http://twitter.com/JFamPract http://bit.ly/JFP_GooglePlus tion, diagnosis and management. Arch Dis Child. 2011;96:1066-1071.

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