Thursday, November 30, 2023
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The following slideshow explores cutaneous and other clinical findings of rheumatic disease—or mimics of rheumatologic disease—involving the hand, but which are not arthritis. Some of the findings are relatively rare, but several cases illustrate involvement with diseases more commonly seen by primary care providers such as hepatitis C, cirrhosis, acute frost-bite injury, and skin disease secondary to angiographic intervention. In addition, findings secondary to an underlying infection (gonorrhea and syphilis) will be discussed; these conditions are often first evaluated by primary care providers. As such, we hope that this slideshow will be helpful not only to specialists in rheumatology but also to those providers on the front lines seeing patients in primary care clinics, urgent care, and the emergency department.
Image from iStock
A 55-year-old woman presented with a sudden onset of a nonblanching purpuric rash on her extremities, largely on her hands and feet. She had a history of intravenous drug use 25 years earlier. Blood testing revealed abnormal liver function tests, elevated creatinine, and her urinalysis had elevated protein and red blood cells. She also had an elevated rheumatoid factor, low C4, and a normal C3 level. She had positive cryoglobulins, and her hepatitis C antibody was positive.
Diagnosis: Hepatitis C-related cryoglobulinemia causing purpuric skin rash and renal failure.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
In this image, the dense purplish discoloration of the patient's fingers developed after the placement of an arterial line in the radial artery and subsequent thrombosis of that artery reflecting that patients with this form of vasculitis may be prone to worsening damage due to vascular procedures.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 60-year-old man presented with intermittent pain and discoloration in his fingers and toes. He was a nonsmoker but had received a blood transfusion in 1982 after a motor vehicle accident. Blood testing revealed mildly elevated transaminases, normal renal function, and urinalysis. His rheumatoid factor was mildly elevated, and his complement C3 and C4 levels were normal. A hepatitis C antibody was positive.
Diagnosis: Hepatitis C-related cryoglobulinemia causing digital ischemia.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
Similar to Case 1, this patient has hepatitis C-related cryoglobulinemia; however, his case is much more benign. Other diseases on the differential for this patient would be embolic phenomenon from a central source (eg, atrial thrombus) or Berger disease, although this latter disease is highly unusual in nonsmokers. This image shows ischemia and ulceration of the digits in the setting of hepatitis C-related cryoglobulinemic vasculitis.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 42-year-old man presented with painful discoloration and necrosis of his fingertips. He smoked approximately 40 cigarettes daily. Laboratory testing revealed negative hepatitis C antibody testing.
Diagnosis: Buerger disease (thromboangiitis obliterans).
Discussion: There can be multiple causes of digital ischemia including vasculitis, severe vasospasm, local thrombosis, or embolic phenomenon. In this case, the patient's heavy smoking suggested Buerger disease. The diagnosis was confirmed with angiographic studies as well as elimination of other potential causes.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 56-year-old woman with systemic sclerosis presents with the onset of finger pain and darkening over the preceding 2 weeks.
Diagnosis: Critical digit ischemia due to systemic sclerosis-related vasculopathy.
Discussion: The etiology of tissue ischemia in systemic sclerosis can arise from several sources: chronic vasculopathy (terminal vessel narrowing at the level of the palmar arch and distal digital arteries), more proximal occlusive disease at the level of ulnar or radial artery, vasculitis, thromboembolic disease, or smoking-related vessel disease (Buerger disease). Given the relatively acute onset and predilection for only one finger, lab testing and an angiogram were obtained. Lab testing was negative for antiphospholipid antibodies, cryoglobulins, and antineutrophil cytoplasmic antibodies. An angiogram showed normal proximal arterial flow at the level of the radial and ulnar artery, with severe vessel tapering at the level of the palmar arch in the right hand with no collateral flow seen in the fourth digit. There was no improvement in blood flow with vasodilator challenge.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
In the acute setting, limited systemic sclerosis with digital gangrene can be treated with with vasodilators (such as calcium channel blockers and intravenous prostacyclin analogues) alongside platelet inhibitors, and/or anticoagulation can be attempted. In addition, digital sympathetic blocks with lidocaine and botulinum toxin have been described as well as surgical sympathectomy. In this case, the absence of any collateral blood flow to the involved digit, lack of response to vasodilator challenge during angiogram, and black eschar formation all suggested that treatment should be intensified to prevent worsening but would not likely result in tissue recovery. This slide shows limited systemic sclerosis digital gangrene at 4 weeks (left) and 4 months (right) after presentation. Autoamputation, rather than surgical removal, is preferred unless signs of underlying osteomyelitis or soft tissue infection are present.
Images courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 48-year-old man presented with painful skin changes to his fingers and toes. He had a history of intravenous drug use in the distant past. Laboratory testing revealed a mild elevation of his aspartate aminotransferase, normal alanine aminotransferase, and negative hepatitis C antibody. Further history identified that 2 days prior, he was intoxicated and slept outside when temperatures had dropped to below 10 degrees Fahrenheit.
Diagnosis: Cold-induced digital injury (frostbite).
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 23-year-old woman presented with several days of migratory joint pain and stiffness and on the day of presentation had pain and stiffness in her right wrist. She also reported feeling intermittently feverish, and she reported several skin lesions that looked to her like big pimples. Several weeks earlier, she had had unprotected sex with her new boyfriend. Joint fluid from her right wrist as well as a culture of one of the skin lesions grew Neisseria gonorrhea. This image shows swelling of the right wrist and several pustular skin lesions on the dorsum of her left hand.
Diagnosis: Disseminated gonorrhea with arthritis.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
One of the most common bacterial causes of monoarthritis or oligoarthritis in a sexually active person is N gonorrhea; therefore, this organism should be considered in these patients. While testing can be done at mucosal sites using polymerase chain reaction-based tests, the organism requires special media to identify in joint fluid; therefore, clinicians should discuss with their laboratory what specific methods to use to identify this organism.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 50-year-old man presents with a 4-month history of finger swelling and decreased range of motion. He was referred for possible new-onset rheumatoid arthritis, and his initial lab work-up revealed a negative rheumatoid factor and anticyclic citrullinated peptide antibody but a positive antinuclear antibody with a nucleolar pattern. His history was largely unremarkable other than a 2-year history of vitiligo and pruritis, for which he was under the care of a dermatologist. At his initial visit, he described symptoms of color changes in response to cold at fingers and toes. Examination reveals puffy fingers bilaterally, mild cyanosis right greater than left hand, skin tightening at the fingers bilaterally as well as the face, with facial hypopigmentation changes. Mild crackles were heard at the lung bases. A CT scan of the lungs revealed bibasilar fibrotic changes with a dilated esophagus. This image reveals the taut, shiny skin that can occur in systemic sclerosis, in this case over the face.
Diagnosis: Limited systemic sclerosis.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
The patient's clinical features of skin tightening, pruritis, puffiness at fingers, and Raynaud phenomenon are consistent with a diagnosis of systemic sclerosis. The clinical diagnosis is supported by laboratory evidence of an antinuclear antibody with nucleolar pattern as well as imaging findings of interstitial lung disease and esophageal dilation. This magnified image shows the characteristic "salt-and-pepper" depigmentation that can occur in systemic sclerosis due to sparing of pigment loss around hair follicles. The etiology for the depigmentation is uncertain but is thought to be due to the inflammatory and fibrotic skin changes that typify the disease.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
A 74-year-old woman with diffuse systemic sclerosis presents with increasing pain and skin erythema overlying the proximal interphalangeal (PIP) joints. X-rays were obtained (image not shown) and revealed no obvious changes of osteomyelitis. Radio-opaque deposits were seen in the soft tissues surrounding the dorsal aspect of several digits in the right hand.
Diagnosis: Systemic sclerosis-associated calcinosis cutis.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
Ulcerations are common in patients with systemic sclerosis and often are secondary to the underlying vasculopathy that characterizes the disease. These ulcerations can be found at the digital tips but can also be found on the dorsal surface of the PIP joints secondary to microtrauma from day-to-day activities in the face of chronic tissue ischemia (often referred to as digital infarcts). Additional causes of ulceration that need to be considered include underlying soft-tissue calcification or a small or medium vessel vasculitis. Treatment for scleroderma-related calcinosis has been disappointing. Options that have been tried, with variable success, include diltiazem, warfarin, minocycline, colchicine, bisphosphonates, as well as topical and intravenous forms of sodium thiosulfate. This x-ray is from a separate patient from the one described above. Notice the soft-tissue calcifications along the ulnar aspect of the carpal bones.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
This image of our patient depicts the facial features that can arise in diffuse systemic sclerosis. Facial skin tightening and drawn pursed lips are present. These changes, along with associated muscular wasting of the temporal, facial, and neck muscles, may result in the development of a "mouse facies" (Mauskopf, or mousehead). The picture on the right shows severe skin thickening of the forearm, hand, and fingers, with resultant flexion contracture of the fingers.
Images courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
The rheumatology service is asked to evaluate a 60-year-old woman with longstanding joint pain and suspected osteoarthritis. She describes hip, knee, and base of thumb pain, in addition to distal interphalangeal (DIP) joint pain and bony enlargement. More recently, her DIPs have become more acutely painful, with the development of "white spots" at her fingertips.
Diagnosis: Hyperuricemia with acute gouty arthritis of the left second DIP.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
Soft-tissue deposits of uric acid (tophi) most commonly occur in periarticular sites such as the olecranon bursae and the soft tissue around the first metatarsal phalangeal joint. However, subcutaneous deposits can occur in virtually any site, including the pinna of the ear classically. The finger pad was cleaned with sterile solution and lanced with a small needle. The creamy fluid was viewed under polarized microscopy, and numerous negatively birefringent crystals (consistent with uric acid) were seen.
Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD
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