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A Case of Postinfectious Protein S Deficiency Masquerading As Henoch

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A Case of Postinfectious Protein S Deficiency Masquerading As Henoch CPJXXX10.1177/0009922815590116Clinical PediatricsWampole et al 590116research-article2015 Brief Report Clinical Pediatrics 1 –4 A Case of Postinfectious Protein S © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav Deficiency Masquerading as Henoch– DOI: 10.1177/0009922815590116 Schönlein Purpura cpj.sagepub.com Anthony J. Wampole, MD1, Halie M. Anderson, MD1, and Ellen R. Wald, MD1 Case Report (Presentation) inability to urinate brought him back to the ED. His exam was notable for tachycardia; he had scattered pete- The patient is a previously healthy 4-year-old male who chiae on chest and arms and diffuse “nonpalpable” pur- is admitted for a purpuric rash. Ten days before admis- pura on legs, buttocks, perineum, and penis with sion he experienced mild, transient cold symptoms. Four surrounding edema. His scrotum was purpuric and days before admission he developed hives on his lower grossly edematous, with overlying bullae. Urine was abdomen and mild swelling of his feet; symptoms grossly bloody. Repeat laboratory studies were notable improved with diphenhydramine. Two days later the pri- for leukocytosis, anemia, and thrombocytopenia. mary care provider (PCP) was informed of slight perior- Complete metabolic panel, including electrolytes, kid- bital puffiness and ongoing swelling (with a slight ney function, and transaminases, was notable for a “bluish-hue”) on the dorsum of his feet bilaterally. He mildly low CO (21 mEq/L) and albumin (2.8 g/L). 2 was otherwise well, without fever or discomfort, except Coagulation studies were grossly abnormal with PT/ for mild itching between doses of diphenhydramine. INR = 8.0, partial thromboplastin time (PTT) = 70.2, Family was instructed to seek medical care if he devel- fibrinogen <40, and D-dimer >10 000. Blood cultures oped joint pain or swelling, difficulty with mobility, were obtained; he was given a dose of ceftriaxone, nor- blue-purple rash, or fever. Twelve hours later, a dark mal saline bolus, and fresh frozen plasma and trans- purple rash evolved over his legs. He was taken to a ferred to our critical care unit. On arrival he was local emergency department (ED) where he was afebrile hemodynamically stable but showed ongoing, severe with normal vital signs. He was documented to have coagulopathy. He had deep purple plaques over thighs purpuric lesions on his lower extremities and scattered and lower extremities, including lateral hips and penis. hives on his torso. Laboratory studies were remarkable Feet were relatively spared with some purple discolor- for a low normal platelet count and prothrombin time ation of toes. There were no petechiae or purpura above (PT)/international normalized ratio (INR) = 1.4 (see the waistline. His penis and scrotum were extremely Table 1 for timeline of presentation, including vital swollen and deeply ecchymotic, with overlying bullae signs, pertinent labs, and physical exam findings). He (see Figure 1). He had a normal blood gas, with a lactate was discharged home with a presumptive diagnosis of of 2.1 mEq/l. He was diagnosed with purpura fulminans Henoch–Schönlein purpura (HSP). His PCP saw him the (PF) and disseminated intravascular coagulation of following day; his vital signs were normal and he was uncertain etiology. noted to have swelling and redness of his penis, as well Further diagnostic workup was undertaken to deter- as “deep bruising” of the posterior thighs and buttock. mine the etiology of the PF. Blood drawn on the day of Urinalysis did not show hematuria. The PCP agreed admission (before receiving any blood products) was with the diagnosis of HSP; the patient was sent home retrospectively found to have suppressed protein S with anticipatory guidance. That night, he developed activity (49%; reference range = 75% to 140%). After worsening bruising and swelling and progressive penile the initiation of plasmapheresis, the DIC stabilized and pain that inhibited his ability to void. The escalation of pain prompted a second visit to his local ED. He 1University of Wisconsin School of Medicine and Public Health, remained afebrile without vital sign changes. His exam Madison, WI, USA was notable for progressive ecchymoses of the lower extremities and buttocks. His penile shaft was described Corresponding Author: Anthony J. Wampole, Department of Pediatrics, University of as “moderately to severely edematous and ecchymotic.” Wisconsin School of Medicine and Public Health, Box 4108, 600 No laboratory studies were obtained. He was started on Highland Avenue, Madison, WI 53792, USA. oral prednisone and discharged. Unremitting pain and Email: [email protected] Downloaded from cpj.sagepub.com at UNIV OF WISCONSIN-MADISON on June 22, 2015 2 Clinical Pediatrics Table 1. Timeline and Associated Laboratory Findings. First ED Visit (2 days PCP Office (1.5 days Second ED Visit (1 day PTA) PTA) PTA) Third ED Visit Admission to PICU Vital signs Afebrile; HR 139; BP Afebrile; HR 128; BP Afebrile; HR 121; BP Afebrile; HR 154; BP Afebrile; HR 145; BP 108/59; RR 24 104/50; RR 24 106/68; RR 26 106/73; RR 28 114/68; RR 22 Examination Purpuric lesions on Swelling and redness of Ecchymoses of the Scattered petechiae Deep purple plaques lower extremities; his penis, as well as lower extremities on chest and arms; over thighs and scattered hives on “deep bruising” of the and buttocks; penile diffuse “nonpalpable” lower extremities. torso posterior thighs and shaft was described purpura on legs, Feet relatively spared buttock as “moderately to buttocks, perineum, with some purple severely edematous and penis with discoloration of and ecchymotic” surrounding edema; toes. No petechiae scrotum purpuric and or purpura above grossly edematous, the waistline. with overlying bullae Penis and scrotum were extremely swollen and deeply ecchymotic, with overlying bullae. WBC (K/µL) 10.3; 70% PMNs, — — 16.7; 87% PMNs; 11% 10.5; 81% PMNs; 16 24% lymphs lymphs lymphs Hgb (g/dL)/Hct (%) 12.5/35.6 — — 9.5/27.9 5.3/15 Platelets (K/µL) 152 — — 41 17 CO (mEq/L) 22 — — 21 23 2 BUN (mg/dL) 13 — — 18 20 Creatinine (mg/dL) 0.35 — — 0.47 0.43 Urinalysis Unable to be (+) ketones; 2-5 WBCs — Grossly bloody — obtained PT/INR 1.4 — — 8 1.3 Diagnosis HSP HSP HSP DIC Purpura fulminans Intervention Anticipatory Anticipatory guidance Prednisone (1 mg/kg, Blood products, fluids, guidance BID) transfer to PICU Abbreviations: ED, emergency department; PTA, prior to admission; PCP, primary care physician; PICU, pediatric intensive care unit; HR, heart rate; BP, blood pressure; RR, respiratory rate; WBC, white blood cell count; PMN, neutrophils; lymphs, lymphocytes; Hgb, hemoglobin; Hct, hematocrit; BUN, blood urea nitrogen; PT, prothrombin time; INR, international normalized ratio; HSP, Henoch–Schönlein purpura; DIC, disseminated intravascular dissemination. the protein S activity normalized rapidly, supporting the Henoch–Schönlein Purpura diagnosis of a postinfectious acquired antibody to pro- tein. He underwent a series of debridements and cadav- HSP is the most common small vessel vasculitis in childhood, affecting approximately 20/100 000 children eric skin grafts, but ultimately required bilateral 1 below-knee amputations. His scrotum re-epithelialized; annually. It is the most common cause of petechiae and a urinary catheter was placed for urethral patency and he purpura in children (more common than idiopathic had suprapubic catheter placement for urinary diversion. thrombocytopenic purpura [ITP] and childhood leuke- Pain and agitation were management issues throughout mias). HSP can occur anytime in childhood with a peak his hospitalization. He experienced several skin and uri- incidence at 4 to 5 years. It is preceded by a viral upper nary tract infections. He will ultimately require penile respiratory tract infection in more than half of cases. The reconstruction and bladder augmentation procedures. hallmark of HSP is palpable purpura, classically involv- His total hospital stay was 84 days. ing pressure-dependent areas of the lower extremities and buttocks. The rash can initially appear as urticarial- like lesions and often progresses to varying-sized pete- Discussion chiae and coalesced, raised purpura. Other commonly associated findings include edema, abdominal pain The patient’s initial presentation seemed most consistent (75%), migratory arthralgias/arthritis affecting joints of with HSP; however, his clinical trajectory diverged sub- lower extremities (80%), renal involvement (30% to stantially from the expected course. He was diagnosed 50%), and, more rarely, scrotal involvement and intus- to have postinfectious PF, which ultimately threatened susception. HSP is generally a self-limited illness. Most his life and compromised his limbs. children have complete resolution of symptoms within Downloaded from cpj.sagepub.com at UNIV OF WISCONSIN-MADISON on June 22, 2015 Wampole et al 3 Figure 1. Physical exam findings. Exam findings on presentation (top photo and left photo) and evolution over first 12 hours of inpatient admission (right photo). He had purple plaques over thighs and lower extremities, including lateral hips and penis. Feet were relatively spared with some purple discoloration of toes. There were no petechiae or purpura above the waistline. His penis and scrotum were extremely swollen and deeply ecchymotic, with overlying bullae. 4 to 6 weeks with about one third of patients experienc- general pediatricians in our community to determine ing relapse of symptoms within the first year. Renal their typical diagnostic evaluation
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