Henoch-Schönlein Purpura Presenting as a Rash and Proteinuria in a 59-Year-Old Woman

Abstract

Henoch-Schönlein purpura is a disease of unknown etiology. it is predominantly seen in children, although adults are also affected, and comprises a rash, arthritis, abdominal pain, and renal dysfunction. there is no effective treatment, although the syndrome is self-limiting and generally subsides without sequelae. The authors present a case of Henoch-Schönlein purpura in a 59-year-old woman who presented with a rash of six months’ duration and a high level of urinary protein.

• Physicians are often presented with various presentations of vasculitis and should be aware of the clinical presentations and diagnostic findings that occur in Henoch-Schönlein purpura. Although it is predominantly a disease of children, adults are also affected. Therefore, it should be considered in the differential diagnosis for acute abdomen, purpura, and renal dysfunction. We present a case report of a 59-year-old woman who presented with a rash and renal dysfunction and was finally diagnosed with Henoch-Schönlein purpura. The case reflects the significant features of the history, presentation, and course of the disease.

Case Report

A 59-year-old woman with a significant medical history of allergic rhinitis, myalgia, and leukocytoclastic vasculitis of six months’ duration and a one-month histoiy of hypertension returned to the clinic due to proteinuria, which was recently note6d by her dermatologist during an exacerbation of her vasculitis.

Six months before, the patient had developed a skin rash over her arms and legs, which was diagnosed as leukocytoclastic vasculitis by skin biopsy. She described her skin lesions as palpable lesions that were more pronounced on the buttocks, waist, and sock line, came in groups, and recurred. The color of the lesions progressed from red, to purple, and finally to brown. The patient had been treated with prednisone (60 mg by mouth every day) and acyclovir (800 mg by mouth five times a day for seven days) without any obvious effect. She was not on any medications for vasculitis.

A review of systems revealed neither pedal or periorbital edema nor weight loss. She complained of occasional knee arthralgia, but there was no swelling or involvement of the small joints of her hands. She denied urinary frequency, urinary urgency, or dysuria, but had been told by her dermatologist about the loss of protein in her urine. No abdominal pain, nausea, vomiting, or bleeding wits present. There was no history of bleeding from any site. She denied any cardiopulmonary symptoms except for hypertension, which had been diagnosed a month before. She also denied having visual symptoms and headaches. However, she did admit to feeling anxious and frustrated by her skin problem.

Her medications included conjugated estrogens (0.625 mg daily), loratadine (1 to 2 tablets as needed), and fluticasone nasal spray (as needed). She was allergic to aspirin, which she said caused her stomach upset and a skin ntsh described as a pruritic erythematous rash over the extremities.

The patient’s family history was significant for myocardial infarction (father), familial paralysis (sister), and lupus (cousin). Her surgical history included a hysterectomy 14 years earlier and an appendectomy 7 years earlier.

The physical examination revealed an alert, oriented, and ambulatory white woman. She was afebrile, and her vital signs included a pulse of 74 beats per minute, a blood pressure of 148/70 mm Hg, a respiratory rate of 16 breaths per minute, and a weight of 151.5 lbs

The skin examination showed new and fading purpuric papules and macules over the extensor surfaces of her forearms, legs, and feet. The head, eyes, ears, nose, and throat examination was entirely normal and showed no oral mucosal lesions. Her neck was supple, with no thyromegaly, carotid bruit, or lymphadenopathy. The lungs were clear on auscultation. Cardiac auscultation included S₁ + S₂ + 0.

The patient’s abdomen was soft and nontender, with no organomegaly or bruits, and normal bowel sounds were present. A rectal examination did not reveal occult blood. An examination of her back showed no sacral edema. An examination of her extremities revealed no edema, normal joints, good peripheral pulses, and skin lesions as mentioned above. Neurologically, she was alert, oriented, and grossly nonfocal. There was no evidence of peripheral or cranial nerve abnormality.

Her laboratory data included the following peripheral blood count levels: white blood cells. 6,100/mm³; hemoglobin, 12.6 gm/dL; hematocrit, 38.9%; platelets, 206,000/mm³ with normal differential count; sedimentation rate, 39 mm/hr; hepatitis-B, negative; hepatitis-C, negative, sodium, 141 mEq/L; potassium, 4.7 mEq/L; chloride, 103 mEq/L; CO₂, 31 mEq/L; BUN, 29 mg/dL; and creatinine, 1.3 mg/dL.

The urinalysis results were as follows: pH, 7; protein, 3+; blood, 3+, then 4+; glucose, normal; leucocyte esterase, normal; red blood cell count, 30/HPF; white blood cell count, 10/HPF; squamous cells, 3/HPF; and no casts. The tests for rheumatoid factor were negative; complement C3, complement C4, and total hemolytic complement levels were normal; the antinuclear antibody tiler was 1:160; the antideoxyribonuclear antibody was absent; and the serum protein electrophoresis showed a decrease in albumin and total protein. The 24-hour urinary protein level was 537 mg/dL.

Abdominal ultrasonography revealed normal kidney size without stones. A skin biopsy revealed leukocytoclastic vasculitis, and direct immunofluororescence revealed deposition of immunoglobulin A and complement C3 in the vessel wall.

Finally, the patient underwent a percutaneous right renal biopsy. Light microscopy revealed approximately 30 glomeruli in the total specimen. Mesangial cell proliferation and the mesangial accumulation of hyaline material was noted. Tubules, interstitium, and blood vessels were normal. There was no fibrosis (Figure 1). Immunofluorescent microscopy revealed positive immunoglobulin A staining with no other evidence of immunoglobulin deposition. Immunoglobulin G, complements C1, C2, C3, C4, and albumin levels were all normal (Figure 2). Electron microscopy demonstrated prominent immunoglobulin A deposits in the widened mesangial stalk (Figure 3).

Figure 1—

Light microscopy showing mesangial cell proliferation and the mesangial accumulation of hyaline material.

Figure 2—

Immunofluorescent microscopy revealing positive immunoglobulin A staining.

Figure 3—

Electron microscopy demonstrating prominent immunoglobulin A deposits in the widened mesangial stalk.

Diagnosis

In retrospect, the patient reported viral pharyngitis with cervical adenopathy and palpable purpura over the legs and feet and, to a lesser extent, the forearms, as well as leg edema and joint tenderness occurring with the illness’ onset.

Moreover, the patient’s lesions never fully resolved on steroids and, in fact, continued to occur in waves. However, the severity and frequency of occurrence of the skin lesions had diminished with time.

Her renal laboratory results also continued to improve, with decreasing blood urea nitrogen levels of 29, then 23, 22, and 12 mg/dL and decreasing creatinine levels of 1.3, then 1.2, 1.1, and 0.9 mg/dL.

Therefore, the presence of a typical purpuric rash over a typical location, the history of pharyngitis and pedal edema at the onset of illness, the lack of response to steroids, the hypersensitivity to aspirin, the gradually improving rash, the maintenance of normal renal function, and the findings on kidney biopsy confirm the diagnosis of Henoch-Schönlein purpura.

The patient was started on an angiotensin-converting enzyme inhibitor to control hypertension and reduce angiotensin II levels, which should blunt any tendency to fibrosis and mesangial proliferation in the setting of immunoglobulin A nephropathy.

Discussion

Henoch-Schönlein purpura is a form of purpura of unknown etiology. The underlying pathology is a leukocytoclastic vasculitis that principally affects postcapillary venules. Although the disease is predominantly seen in children. adults are also affected. The syndrome comprises rash, arthritis, abdominal pain, and renal dysfunction (Tables 1 and 2).^1,2

Table 1.

1990 Criteria for the Classification of Henoch-Schönlein Purpura²

S. No.	Criterion
1	Palpable purpura
2	Age ≤ 20 at disease onset
3	Bowel angina
4	Wall granulocytes on biopsy

A patient shall be said to have Henoch-Schönlein purpura if at least two of these four criteria are present. The presence of any two or more criteria yields a sensitivity of 87.1% and a specificity of 87.7%.

Table 2.

Prevalence of Organ/System involvement in Henoch-Schönlein Purpura

Organ/System	Percent of Cases
Gastrointestinal	20 to 40
Renal	50
Joints	80
Skin	up to 100

An upper respiratory tract infection typically precedes the onset of Henoch-Schönlein purpura. Regional subcutaneous edema of the extremities, scalp, eyes, or scrotum can be seen early in the course of the disease.

The cutaneous lesions are described as palpable purpura that are typically located on the lower extremities but also appear on the hands, arms, and trunk. They tend to accentuate in areas of pressure, such as the waist or sock line. These lesions come in groups, recur, and progress from red, to purple, and to brown. The lesions do not disappear on pressure, thereby signifying intradermal bleeding.^1,2,3

Abdominal involvement generally presents as crampy, periumbilical pain in 20% to 40% of patients within a week of the appearance of the rash. Since abdominal pain is secondary to vasculitis, the involvement of the gastrointestinal tract is often associated with bleeding, infarction, intussusception, and perforation.¹

Fifty percent of patients develop nephritis and progress from microscopic hematuria, to protein-uria. to nephrotic syndrome, to renal failure. However, the renal changes are usually reversible and only occasionally progress to renal insufficiency. Five to ten percent of children develop serious renal disease within one to three months of onset, particularly those with diffuse lesions or those who develop nephrotic syndrome. Glomerulonephritis varies from focal mesangial proliferation to crescentic glomerulonephritis, but it is always associated with mesangial immunoglobulin A deposition. There is progressive renal failure in those with crescents. However, immunoglobulin A nephropathy can also be associated with other diseases, as shown in Table 2, and is the most common type of adult primary glomerulonephritis.^1-4

Eighty percent of Henoch-Schönlein patients develop arthritis or arthralgia of large joints. Small joints may also be affected, but no long-tenn arthritic deformities occur.

Hypersensitivity to aspirin and some food additives may be seen in some of these patients.^5,6

Laboratory findings are non-contributory. Platelet count, if normal, rules out thrombocytopenic purpura. Other findings include an elevated white blood cell count; normochromic anemia; increased serum immunoglobulin A/immunoglobulin G (50%); antinuclear antibodies, negative; Rh factor, negative; elevated sedimentation rate; and a skin biopsy showing leukocytoclastic vasculitis with deposition of immunoglobulin A and complement C3 in the vessel wall on direct immunofluorescence.^1,7

Henoch-Schönlein purpura is usually self-limited, lasts one to six weeks, and subsides without sequelae if renal involvement is not severe. There is no effective treatment. However, management includes hydration and nutritional support. Systemic steroids are indicated in patients with severe gastrointestinal disease. Steroids alone are not helpfull in renal disease; however, immunosuppressives have shown some success in the nephropathy associated with this syndrome.⁷

The disease may recur up to two years later.^4,7

Conclusion

Henoch-Schönlein purpura usually presents with a purpuric rash over the extensor surfaces and the bultocks. There may be nephritis (one third of patients), joint involvement, or abdominal pain. Leukocytoclastic vasculitis is present on histopathology, and the platelet count is normal. The disease often occurs after an acute respiratory tract infection and usually follows a benign course over a period of weeks or months. Complications include massive gastrointestinal bleeding, ileus, and renal failure. Henoch-Schönlein purpura should not be forgotten in the differential diagnosis of vasculitis manifesting as purpura, abdominal symptoms, or renal dysfunction, regardless of the age of the patient. •

Table 3.

Differentiation of Henoch-Schönlein Glomerulonephritis from Other Types of Nephropathies

Condition	Light Microscopy	Electron Microscopy	Immunofluorescence	Associated Features
Henoch-Schönlein glomerulonephritis	Mesangial cell proliferation, frequently crescentic	Mesangial deposits	Mesangial IgA, C3	Purpura, arthritis, abdominal pain
Immunoglobulin A nephropathy	Mesangial cell proliferation	Mesangial deposits	Mesangial IgA, C3	Intermittent hematuria
Antiglomerular basement membrane disease	Frequently segmental, often necrotizing	Negative	Linear IgG, C3	± Pulmonary hemorrhage
Lupus glomerulonephritis	Mesangial and endothelial proliferation	Various deposits, “fingerprinting” of deposits, tubulovesicular bodies	“Full house” pattern of immune complex deposits	± Systemic syndrome, antinuclear factor, etc.
Bacterial endocarditis	Mesangial and endothelial proliferation, segmental	Variable	Variable	Cardiac murmur, fever
Systemic vasculitis	Often crescentic, segmental necrosis, focal arteritis	Negative	Negative	Systemic symptoms

Biography

Muhammad Wasif Saif, M.D.

Dr. Saif is Clinical Fellow (FY-Il) in the Division of Hematology-Oncology at the National Institutes of Health in Bethesda, Maryland. He received his M B.B.S. from King Edward Medical College in Lahore, Pakistan, and completed a residency in internal medicine at the University of Connecticut School of Medicine in Farmington.

Diane L. Whitaker-Worth, M.D.

Dr Whitaker-Worth is Assistant Professor and Director of Cliniad Education in the Department of Dermatology at the University of Connecticut Health Center in Farmington. After receiving her medical degree from the University of Connecticut School of Medicine, she completed an internship in transitional medicine at Hanford Hospital in Connecticut and a residency in dermatology at Brown University in Providence, Rhode Island

Joseph Palmisano, M.D.

Dr. Palmisano is Professor of Clinical Medicine at the University of Connecticut School of Medicine in Farmington. After receiving his medical degree from the State University of New York Downstate College of Medicine in Brooklyn, he completed a residency in medicine at Baltimore City Hospital in Maryland a fellowship in medicine at Johns Hopkins Hospital in Baltimore, and clinical and research fellowships in nephrology at Boston University School of Medicine in Massachusetts.

Richard Cagna, M.D.

Dr. Cagna is Assistant Clinical Professor of Medicine at the University of Connecticut School of Medicine in Farmington and at Mount Sinai Hospital in Hartford. After receiving his medical degree from St. George’s University School of Medicine in Grenada, West Indies, he, completed a residency in internal medicine at Mount Sinai Hospital, where he later served as Chief Resident in Internal Medicine.

Harold T. Yamase, M.D.

Dr. Yamase is Associate Professor of Pathology at the University of Connecticut School of Medicine in Farmington. He received his medical degree from Tulane University School of Medicine in New Orleans, Louislana, and completed a residency in pathology at Charity Hospital, Tulane Division in New Orleans.