An Unusual Case of Henoch-Schönlein Purpura in an Elderly Male Jeffrey Kushner, DO,* David Posnick, DO,** Joan M. Mones, DO,*** Adriana Ros, DO, FAOCD*** *PGY-1 Traditional Rotating Intern, Largo Medical Center, Largo, FL **PGY-3 Dermatology Resident, Palisades Medical Center, North Bergen, NJ ***Program Director, Dermatopathology Fellowship, New York College of Osteopathic Medicine/Ackerman Academy of Dermatopathology, New York, NY ****Program Director, Dermatology Residency, Palisades Medical Center, North Bergen, NJ Abstract Henoch-Schönlein purpura (HSP) is a subset of cutaneous small vessel vasculitis (CSVV) characterized by IgA deposition in the walls of small blood vessels leading to non-thrombocytopenic palpable purpura, typically of the lower extremities. Other immune factors such as IgM, IgG, complement, and fibrinogen may be found in vessels. The disease is characterized by a tetrad of manifestations including palpable purpura, arthralgia/arthritis, abdominal pain, and renal disease.1 Morbidity in the HSP patient population is correlated with chronic renal failure secondary to glomerulonephritis. HSP is rarely seen in the adult and geriatric population; approximately 90% of patients are children. We present a case of HSP in a nonverbal, nonambulatory 62-year-old Caucasian male. Henoch-SchönleinIntroduction purpura (HSP) is a cutaneous ACase 62-year-old, Report minimally verbal, non-ambulatory small vessel vasculitis with deposition of IgA and Caucasian male presented to the dermatology other immune factors within the vessel walls. outpatient clinic complaining of a new-onset The disease was originally identified in 1801 by rash on his lower extremities for one week. Johann Schönlein and his student, Eduard Henoch, The patient denied any symptoms of itching who described the clinical signs and symptoms.1 or pain. Clotrimazole/betamethasone topical It is highlighted by a tetrad of symptoms and cream prescribed by his internist showed no complications—palpable purpura, arthralgia/ improvement. The patient reported no known arthritis, abdominal pain, and renal disease. allergies and no significant family history of Although HSP is significantly more common in vasculitis or autoimmune disease. The patient’s the pediatric population, failure to diagnosis and extensive past medical history included: treat adults can have serious ramifications. We congestive heart failure, cerebral vascular report a case of HSP in a 62-year-old patient with accident, dementia, epilepsy, and dysphagia. an extensive past medical history who initially Medication list included clonazepam, warfarin, presented to the clinic with a lower extremity phenytoin, simvastatin, digoxin, furosemide, and rash. diltiazem. The patient did admit to a history of tobacco use. Figure 1. Multiple discrete, non-blanchable, purpuric papules with hemorrhagic crust on the anterior lower legs symmetrically. Page 26 AN UNUSUAL CASE OF HENOCH-SCHÖNLEIN PURPURA IN AN ELDERLY MALE Physical exam revealed multiple discrete, non- While HSP is associated with deposition of IgA in will develop chronic cases.1 blanchable, purpuric papules with hemorrhagic small blood vessels, there has also been literature Commonly reported extracutaneous crust on the anterior lower legs symmetrically reporting alterations in glycosylation of IgA and manifestations include arthritis, abdominal pain, (Figure 1). Provisional diagnoses included: elevated levels of IgA anticardiolipin antibodies.1 and renal disease. Of the reported symptoms, leukocytoclastic vasculitis secondary to drug, In adults, vascular IgA deposits are highly joint pain was the most common extracutaneous connective-tissue disease or HSP, folliculitis, specific for HSP, although not all patients may manifestation, occurring in up to 84 percent neurotic excoriations, scabies, insect bites, have a positive DIF.11 Additionally, complement of patients.1 The pain is usually transient and lymphomatoid papulosis, and pityriasis activation, glomerular crescent formation and oligoarticular and often afflicts the lower lichenoides et varioliformis acuta. vascular damage have been identified as important extremities, resulting in pain with ambulation. Two 3 mm punch biopsies were performed and mechanisms underlying renal involvement in Gastrointestinal symptoms are varied, ranging sent for routine hematoxylin and eosin (H&E) HSP. Multiple triggers for the condition have from mild nausea, vomiting and abdominal pain staining and direct immunofluorescence (DIF). been suggested, although the actual cause still to more emergent cases of intussusception, bowel One biopsy was obtained from the left superior remains unknown. In children, symptoms may be ischemia and perforation. The abdominal pain lateral tibia and another from the left inferior preceded by an upper respiratory infection, more has been attributed to submucosal hemorrhage medial tibia. A complete metabolic panel, specifically attributed to group A beta-hemolytic and edema. In cases with gastrointestinal 3 complete blood count with differential, urinalysis, streptococcus. Multiple disease states and drugs involvement, subclinical laboratory findings that and hepatitis panel were ordered. Labs on the have been implicated in the development of HSP may indicate more advanced disease include a a patient were unremarkable except for anemia including pregnancy, 1-antitrypisin deficiency, positive fecal occult blood test, hypoalbuminemia alcohol, vaccinations, chlorpromazine, losartan, (hemoglobin 11.1). Renal function was stable and a positive a1-antitripysin. (BUN: 11 creatinine: 0.6). H&E biopsy showed aspirin, and antimicrobials such as penicillin, 4-6 Adult manifestations of HSP do not necessarily evidence of early leukocytoclastic vasculitis ampicillin, clarithromycin, and erythromycin. present like those seen in the pediatric population. (Figure 2). DIF studies were compatible with HSP is characterized by both cutaneous and When compared to children, adults have a lower extracutaneous manifestations. Children almost incidence of prior upper respiratory infection universally present with erythematous, urticarial upon development of HSP. Abdominal pain and papules that rapidly develop into petechiae and fever are less prevalent during the course of the palpable purpura. Vesicles, bullae and necrotic disease in adults, while joint complaints and renal ulcers may also be present. The most common disease are increased.7 A higher frequency of locations for these findings are on pressure- nephrotic syndrome, hypertension and elevated dependent areas such as the buttocks and lower serum creatinine may be seen in the adult extremities, although the elbow and knees may population, which can be especially concerning also be involved.7 Individual lesions usually fade in the presence of comorbidities.8,9 Furthermore, within a week, resulting in hyperpigmentation, literature suggests that renal manifestations although recurrent lesions are possible. become even more prominent in elderly patients Cumulative skin manifestations usually last for compared to adults less than 60 years old.4 six to 16 weeks, although 5% to 10% of patients Figure 2. The changes of early leukocytoclastic A clinical diagnosis of HSP is usually made in vasculitis, including increased numbers children with the prevalence of cutaneous and of neutrophils, incipient nuclear dust of extracutanous manifestations, which are often neutrophils, and extravasation of erythrocytes present. However, the varied presentation in (H&E stain). adult patients may prove more challenging diagnostically. Purpura can be seen with infection, HSP/IgA vasculitis (Figure 3). There was also hypersensitivity vasculitis, rheumatoid arthritis, granular perivascular deposition of C3 and and other small vessel vasculitides. These smooth perivascular deposition of fibrinogen can include granulomatosis with polyangiitis but no perivascular deposition of IgM on DIF. (Wegener’s), microscopic polyangiitis, and Treatment plan included clobetasol 0.05% eosinophilic granulomatosis with polyangiitis 1 ointment every 12 hours to the lower extremities (Churg-Strauss syndrome). Rheumatoid arthritis for two weeks as well as nephrology consultation and systemic lupus erythematosus can mimic (pending). the joint complaints, while IgA nephropathy or Berger’s disease could present similar renal Discussion findings.8 HSP has an annual incidence of about 20 per A definitive diagnosis is usually based upon 100,000 children less than 17 years of age with both clinical manifestations and biopsy. A a peak incidence of 70 per 100,000 in children biopsy is more valuable in the adult population 2 between the ages of four and six years old. because HSP is less common and extracutaneous There is a slight male predominance. The disease symptoms are not always apparent. When occurs less frequently in African Americans, and assessing skin histopathology, proper sampling is more prevalent in the fall and winter months. techniques are paramount to yielding accurate The incidence of HSP in the adult population is Figure 3. Granular perivascular deposition of diagnostic results. A punch biopsy from the edge approximately 1.3 per 100,000 patients, and data IgA present in superficial dermal blood vessels of a fresh lesion, ideally less than 24 hours old, on the elderly population is even more sparse.3,4 (direct immunofluorescence). is the most effective and accurate technique.5 On KUSHNER, POSNICK, MONES, ROS Page 27 H&E staining, tissue will show a leukocytoclastic correlated with