Leukocytoclastic Vasculitis: A Case Report

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LCV, also known as cutaneous small-vessel vasculitis, is the most common dermatologic vasculitis, with an estimated incidence of 15 million to 38 million people per year worldwide.¹ It is a hypersensitivity vasculitis that is characterized by inflammation and leukocytoclasia of postcapillary venules in the dermis due to infiltrating granulocytes, resulting in necrosis of the vascular wall and subsequent thrombosis and erythrocyte extravasation.^1-4 It is believed that venules are more susceptible to injury due to their lower oxygen saturation as compared with the slower flow and stasis of arterioles.⁵ The exact etiology of LCV is most commonly idiopathic; however, secondary causes include infection, autoimmune inflammatory disorders, neoplasm, genetic disorders, and exposure to certain drugs.⁶ In the setting of infection and drug-induced LCV, the vasculitis typically presents 7 to 10 days following medication intake or infectious provocation.⁴ 

LCV classically manifests as palpable purpura, yet it can also present as a maculopapular or petechial rash, urticarial wheal, bullae, papule, plaque, nodule, ulcer, or livedo reticularis; however, chronic lesions present as vesicles, nodules, necrotic ulcerations, and hemorrhagic bullae.¹ These lesions range in size from pinpoint up to 2 cm, but it is not uncommon for these lesions to become confluent.^1,3,7 The lesions most commonly affect the lower extremities because of turbulent flow and hydrostatic forces that occur at the vessel bifurcation.⁵ 

Symptoms associated with LCV include malaise, fever, myalgias, arthralgias, pruritus, burning or stinging of skin, and abdominal pain. Approximately half of cases are associated with an underlying systemic condition such as systemic vasculitis, autoimmune disorders, connective tissue disease, and malignancy, with renal, gastrointestinal, pulmonary, cardiac, and central nervous systems being affected.⁵ 

LCV is typically a diagnosis of exclusion, but it can be confirmed by skin biopsy. The 5 American College of Rheumatology criteria used to assist in the diagnosis of LCV are age 16 years or older, maculopapular exanthema, palpable purpura, positive histopathology, and association with a medication.² A positive histopathology is one that demonstrates 3 distinct findings: neutrophil infiltration around vessel walls, fibrinoid necrosis of vessel walls, and vessel wall injury.¹ Some laboratory test findings, including elevated erythrocyte sedimentation rate, hematuria, proteinuria, and elevated serum creatinine level, are sensitive but not specific to LCV.⁵

Diagnosis of LCV is challenging because the disease process mimics other more common lower extremity wounds. Typically, patients with mild forms of LCV without systemic involvement require no treatment, and there is no uniform treatment of more progressive forms of LCV. The case study described herein demonstrates challenges of treating chronic lower extremity LCV with systemic manifestation.

A 50-year-old male presented to the wound care center with bilateral wounds to the dorsal foot. He described noticing a painful pruritic rash that developed shortly after purchasing a new mattress. The rash progressed to pustules and eschars. He initially was treated in the emergency department, where he was prescribed oral prednisone and cephalexin and was referred to the wound clinic. Past medical history was significant for type 2 diabetes mellitus, hypertension, hyperlipidemia, and chronic kidney disease. The patient denied any current or previous alcohol, tobacco, or recreational drug use.

On initial presentation to the clinic, 5 wounds were observed. The first was a full-thickness wound located on the medial aspect of the right leg (Figure 1). This wound measured 4 cm × 1 cm × 0.2 cm, with exposed adipose tissue. A moderate amount of odorless serosanguinous drainage from the area was noted. The second wound was located on the anterior aspect of the right leg with eschar noted. The wound measured 1 cm × 0.7 cm and demonstrated no active drainage (Figure 2). The third wound was located on the dorsal aspect of the right foot with eschar noted. The wound measured 10 cm × 8.5 cm and exhibited a moderate amount of serosanguinous drainage that was odorless (Figure 3). The fourth wound was located on the dorsal aspect of the left foot with eschar noted. The wound measured 15 cm × 10.5 cm, with a moderate amount of serosanguinous drainage that was odorless (Figure 4). The fifth wound was located on the posterior aspect of the left leg. The wound measured 2.7 cm x 1.4 cm, with a moderate amount of serosanguinous drainage that was odorless (Figure 5). Additionally, there were diffuse, scattered pustules of the bilateral lower extremities. Of note, none of the aforementioned wounds demonstrated any evidence of infection at the time of initial clinical evaluation. A thorough physical examination demonstrated faintly palpable dorsalis
pedis pulses bilaterally and diminished posterior tibial pulses bilaterally. Sensation to light touch was diminished bilaterally. The patient’s current body mass index was 42.4, his current blood glucose was 112 g/dL per his wearing glucose tracking device, and his most recent hemoglobin A1c value was 8.5.

Following his initial visit, the patient was prescribed triamcinolone topical ointment, which he was advised to apply to his bilateral lower extremities every day. He was also advised to continue taking cephalexin for 1 additional week. At this time, several differential diagnoses were considered based on the appearance of the patient’s wounds as well as his medical comorbidities, including but not limited to venous stasis ulcerations, diabetic neuropathic ulcerations, LCV, and pyoderma gangrenosum. As a result, the patient was referred to dermatology and vascular surgery for further workup, diagnosis, and treatment.

The patient was seen the following week by the dermatology team, who biopsied the lesions for proper wound classification. The biopsies confirmed the diagnosis of LCV. The dermatology team prescribed oral prednisone, triamcinolone, and colchicine. Vascular surgery workup revealed bilateral small vessel disease, which did not warrant intervention. The inciting pathogenesis for LCV in this patient was theorized to be either idiopathic or infectious. Autoimmune LCV was considered unlikely given that the patient’s past medical history was negative for rheumatoid arthritis and systemic lupus erythematosus. 

Over the next several weeks, the patient was seen in the office on a weekly basis for reevaluation and dressing changes, which included a combination of medicinal honey and Unna boots. The patient was encouraged to continue taking the oral prednisone and colchicine as prescribed by dermatology. Local wound care was continued, with full resolution of wounds 1, 2, and 5 occurring after 2 months (Figures 6-8). Wound 3 began to increase in size and later became grossly fibrotic, with exposed adipose tissue and signs of acute soft tissue infection (Figure 9). Radiographs demonstrated cortical erosions of the right fifth digit, consistent with changes associated with osteomyelitis. As a result, the patient underwent a right partial fifth ray amputation and allograft placement. He was also prescribed oral antibiotics for possible residual soft tissue infection. Additionally, the fourth wound, which on initial presentation was an eschar, now had visible extensor tendons (Figure 10). Following a similar treatment protocol as for wound 3, the fourth wound was treated with sharp excisional debridement followed by allograft placement. Of note, during this hospitalization the patient developed an AKI, which was deemed to be a progression of his stage 3 chronic kidney disease. It is plausible that the AKI developed due to treatment with colchicine, which is known to exacerbate kidney disease; however, the AKI resolved during the course of the hospitalization and there was no progression of his chronic kidney disease. A study published by Kim et al⁸ in 2020 demonstrated that colchicine has a low risk of adverse kidney outcomes in patients with chronic kidney disease. A bilateral lower extremity angiogram of this case demonstrated no obstructive lesions, with 3 vessel run-offs noted bilaterally.

In addition to the aforementioned treatment, after approximately 6 months of local wound care hyperbaric oxygen therapy (100% oxygen at 2.0 atmospheres absolute for 90 minutes, twice weekly for 60 days) was incorporated into the treatment algorithm. The patient was seen in the office for weekly dressing changes until wounds 3 and 4 were ready for allograft application. As of this writing, the patient continues to be seen in the wound care center weekly for local wound care with continued noted improvement without complete resolution of wounds 3 and 4 (Figures 11-12).

The diagnosis and treatment of LCV is extremely challenging. LCV is commonly misdiagnosed as diabetic or vascular ulcerations. Additionally, other misdiagnoses include eosinophilia, drug reaction with eosinophilia and systemic symptoms syndrome, amyloidosis, antiphospholipid syndrome, atrial myxoma, Behҫet disease, Churg-Strauss syndrome, granulomatosis with polyangiitis, Henoch-Schönlein purpura, urticarial vasculitis, immune thrombocytopenic purpura, Ehlers-Danlos syndrome, scurvy, steroid purpura, solar purpura, exercise-induced purpura, disseminated gonococcal infection, Rocky Mountain spotted fever, disseminated intravascular coagulation, monoclonal paraproteinemia, Waldenström disease, thrombotic thrombocytopenic purpura, cardiac emboli, cholesterol emboli, and meningococcemia.^1,6 

To avoid delays in diagnosis, physicians should have a low threshold to perform a skin biopsy to confirm the diagnosis of LCV.¹ LCV is commonly recalcitrant to local wound care modalities; however, treatment of recalcitrant LCV falls into 2 categories: skin-limited and organ involvement. Overall, skin-limited LCV is typically managed with corticosteroids. However, it is crucial to determine if the condition is primarily cutaneous or drug-induced.¹ Primarily cutaneous LCV can be managed with corticosteroids, cytotoxic agents, antihistamines, nonsteroidal anti-
inflammatory drugs, and/or dapsone.^1,3,5 Drug-induced LCV is managed similarly with the aforementioned treatments but also requires cessation of the drug. Colchicine, an anti-inflammatory medication, has become among the most popular agents used to treat LCV. Colchicine acts by inhibiting polymorphonuclear leukocyte chemotaxis, stabilizing lysosomal membranes, and blocking lysosomal formation.² LCV with organ involvement requires aggressive therapy with immunosuppressants, monoclonal antibodies (rituximab), and/or plasma exchange.¹ 

The present case report emphasizes the importance of early diagnosis of LCV. Despite early diagnosis and treatment with corticosteroids, colchicine, and local wound care, the patient still developed deep infectious wounds necessitating amputation and applications of advanced cellular tissue products. Full resolution of all wounds was achieved 1 year following initial presentation. This timeline contradicts previous studies that report resolution after 3 to 4 weeks.^3,9,10 Although resolution occurs in a matter of weeks to months in a majority of instances, approximately 8% to 10% of patients experience recurrence.⁹ 

Bouiller et al¹¹ reported a 99% 1-year survival rate in a retrospective study of 112 patients with LCV. However, at 3 years, survivorship decreased to 83%. Fraticelli et al¹ demonstrated that prognosis is largely dependent on the severity of organ involvement and the extent of the skin disorder. 

The present report has limitations, the main being a retrospective case study of a single patient. Therefore, it is not possible to state that these results are reproducible.

LCV is a rare dermatological condition that affects the lower extremities. Early and accurate diagnosis and treatment are challenging due to the similarities with other conditions. A multidisciplinary approach is warranted to achieve early identification and wound resolution; however, complications such as deep wound infections and/or osteomyelitis may occur despite early treatment. The present case report aims to add to the body of literature pertaining to LCV in an effort to highlight the importance of early recognition and treatment. More studies, including a large retrospective or prospective cohort, would further elucidate the validity of this report.