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Henoch-Schönlein Purpura Secondary to Subacute Bacterial Endocarditis

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Henoch-Schönlein Purpura Secondary to Subacute Bacterial Endocarditis Continuing Medical Education Henoch-Schönlein Purpura Secondary to Subacute Bacterial Endocarditis Noreen A. Galaria, MD; Nick P. LoPressti, MD; Cynthia M. Magro, MD GOAL To recognize Henoch-Schönlein purpura (HSP) as a possible skin manifestation associated with subacute bacterial endocarditis (SBE) OBJECTIVES Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Describe the clinical features of HSP. 2. Understand the characteristics of bacterial endocarditis and its association with HSP. 3. Differentiate between HSP and other skin manifestations associated with SBE. CME Test on page 280. This article has been peer reviewed and Medicine is accredited by the ACCME to provide approved by Michael Fisher, MD, Professor of continuing medical education for physicians. Medicine, Albert Einstein College of Medicine. Albert Einstein College of Medicine designates Review date: March 2002. this educational activity for a maximum of 1.0 hour This activity has been planned and implemented in category 1 credit toward the AMA Physician’s in accordance with the Essential Areas and Policies Recognition Award. Each physician should claim of the Accreditation Council for Continuing Medical only those hours of credit that he/she actually spent Education through the joint sponsorship of Albert in the educational activity. Einstein College of Medicine and Quadrant This activity has been planned and produced in HealthCom, Inc. The Albert Einstein College of accordance with ACCME Essentials. Henoch-Schönlein purpura (HSP), a systemic, otherwise healthy 41-year-old white woman. The small-vessel vasculitic syndrome, is character- patient presented with a purpuric rash and ized by a nonthrombocytopenic purpuric rash, arthralgia and was found to have left-sided arthralgia, abdominal pain, and nephritis. These streptococcal SBE. She subsequently developed signs and symptoms may occur in any order, and abdominal pain and immune complex glomeru- not all are necessary for the diagnosis. Although lonephritis. The bacterial endocarditis was most common in 4- to 7-year-olds, HSP is well treated with antibiotics and mitral valve replace- documented in adults and is often preceded by ment, followed by a spontaneous resolution of a history of mucosal-based infections, especially the associated signs and symptoms of HSP. of the upper respiratory tract. We report a case of HSP that occurred coincident with the onset of enoch-Schönlein purpura (HSP) is a sys- subacute bacterial endocarditis (SBE) in an temic, small-vessel vasculitic syndrome H characterized by a nonthrombocytopenic Dr. Galaria is a Dermatology Resident, Department of Dermatology, purpuric rash, arthralgia, abdominal pain, and Strong Memorial Hospital, University of Rochester, New York. nephritis. This symptom complex may resemble Dr. LoPressti is in private practice in Camden, New Jersey. Dr. Magro is Assistant Professor, Department of Pathology, Ohio that of other multiorgan vasculitic syndromes, State University, Columbus. including mixed cryoglobulinemia, microscopic Reprints: Cynthia M. Magro, MD (e-mail: [email protected]). polyarteritis nodosa, systemic lupus erythematosus, VOLUME 69, APRIL 2002 269 Henoch-Schönlein Purpura Figure 1. Hemorrhagic bullae and palpable purpura on Figure 2. Extensive palpable purpura on lower extremities. left hand and forearm. and chronic septic vasculitis. These syndromes numerous other inciting agents (eg, insect bites, characteristically involve the skin, kidney, and food allgeries, pharmacologic drugs) have been joints. Immunofluorescent testing is the primary reported.1 In this article, we report a case of sub- method of distinguishing HSP from these syn- acute bacterial endocarditis (SBE) leading to HSP dromes. This testing method reveals prominent in an adult. The pathogenic mechanism is immune and dominant deposits of immunoglobulin A (IgA) complex deposition in the small vessels of the skin within the cutaneous vasculature and renal and mesangium; the complex consists of IgA and glomeruli. The characteristic signs and symptoms an antigen. The immunogenic stimulus is typically of HSP may occur in any order, and not all are nec- a mucosal-based nonviable microbial antigen.1 essary for the diagnosis. However, the rash occurs Rarely, a nonmucosal-based infectious stimulus is at some point in the illness in 100% of cases and is implicated (when this happens, the basis for pref- the presenting complaint in 50% of cases. HSP erential induction of IgA is more elusive). rash classically consists of urticaria, palpable pur- pura, or erythematous maculopapules and charac- Case Report teristically involves the upper and lower A 41-year-old white woman presented with a his- extremities and the buttocks.1 Although most com- tory of mitral regurgitation dating back to child- mon in 4- to 7-year-olds, HSP is well documented hood but was otherwise in relatively good health. in adults. Seventy-five percent of patients have a Five days before admission, she developed a rash presyndrome history of mucosal-based infections, on her hands and ankles (Figure 1); this rash pro- especially of the upper respiratory tract, but gressed from tiny papules to confluent areas of 270 CUTIS® Henoch-Schönlein Purpura Figure 3. Superficial and deep perivascular infiltrate involving capillaries and venules (H&E, original magni- fication ϫ20). striking palpable purpura that extended to involve severe regurgitation. Following the valve replace- large areas of the legs and arms (Figure 2). She also ment, the rash, abdominal pain, oligoarthritis, and had joint pain in the wrists and proximal interpha- glomerulonephritis resolved slowly, and she was dis- langeal joints. The day of admission, she was charged. The patient returned on a few occasions to evaluated in the emergency room. Results of the emergency room with chest pains but no recur- laboratory tests were positive for antineutrophil rence of other symptoms. cytoplasmic antibodies (ANCA). Test results Histology/Immunofluorescence—On day 5, results also revealed that the patient had normocytic of a 4-mm punch biopsy from the leg showed a anemia (hemoglobin, 6.3; white blood cell count, striking vascular reaction involving the venules 10.3, with normal differential; platelet count, 320). and capillaries of both the superficial and deep Erythrocyte sedimentation rate was 97, and coagu- dermis (Figure 3). Neutrophils infiltrated the ves- lation screen and sequential multichannel auto- sel wall, and mural and luminal fibrin depositions analyzer 7 were within normal limits. Blood were extensive. Extravascular neutrophilia was cultures were positive for Streptococcus sanguis. An prominent, with neutrophils present along the echocardiogram was performed, showing the presence dermal–epidermal junction and permeating the of vegetations on the anterior and posterior areas epidermis (Figure 4). These findings defined a of the mitral valve, and the patient was diagnosed case of severe leukocytoclastic vasculitis with with SBE (SBE is characterized by the presence prominent extravascular neutrophilia, which was, of vegetations on the endocardium or in it, therefore, compatible with pustular vasculitis. normally associated with a heart valve). Treatment Gomori methenamine-silver stains, periodic acid- was begun with 2-g intravenous ampicillin and Schiff stain, acid-fast bacillus stain, and gram gentamicin every 8 hours. Later during her hospital stain were negative for fungal, mycobacterial, and stay, the patient developed oligoarthritis, vague bacterial elements. abdominal pain, and renal failure secondary to Results of immunofluorescent studies performed immune complex glomerulonephritis. She received on skin from the right leg showed striking granular methylprednisolone sodium succinate intravenously, deposits of IgA, with codominant deposition of docusate sodium, sucralfate, calcium carbonate, fibrin, IgG, IgM, and C3 within the cutaneous vascu- zolpidem tartrate, and oxycodone plus aceta- lature as well as focal deposits of IgA and IgM along minophen. Before hospitalization, she had not been the dermal–epidermal junction. taking any medications and had no allergies. The patient underwent mitral valve replacement Comment with a porcine valve because of her deteriorating We presented a case of HSP secondary to SBE. The condition and the fear of heart failure secondary to infecting organism in our patient was S sanguis, VOLUME 69, APRIL 2002 271 Henoch-Schönlein Purpura Figure 4. Prominent extravascular neutro- philia with neutrophils along the dermal– epidermal junction and permeating the epider- mis (H&E, original magnification ϫ40). the most common viridans streptococcus involved thrombocytopenic purpura–like (TTP-like) syn- in endocarditis. This organism accounts for drome.6,7 These cases of bacterial endocarditis approximately 60% of cases of native-valve endo- often present with petechiae, particularly on the carditis.2 As in our patient, the entry point in an palate and upper extremities, and with renal dys- initiating episode of infection with Streptococcus function that is reversible with treatment of the viridans usually is not apparent. bacteremia.6,7 However, it is unlikely that the case Circulating immune complexes (CICs) are pres- presented involves a variant of TTP-like syndrome, ent in infective endocarditis, and their measure- for several reasons. First are the clinical differences, ment may be used to monitor a patient’s clinical including the nature of purpura (ie, palpable vs
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