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Wells' Syndrome Proceeded by Leukocytoclastic Vasculitis

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Wells' Syndrome Proceeded by Leukocytoclastic Vasculitis Wells’ Syndrome Proceeded by Leukocytoclastic Vasculitis: A Discussion with New Insights into Analogous Pathophysiology S. Brandon Nickle, DO,* Roxanne Rajaii, MS, DO,** Zachary Hopkins, BS,*** Nelson Charlie, MD**** *Dermatology Resident , 4th year, Broward Health Medical Center, Ft. Lauderdale, FL **Dermatology Resident, 2nd year, Beaumont Hospital, Farmington Hills, MI ***Medical Student, 4th year, University of Utah School of Medicine, Salt Lake City, UT ****Attending Dermatologist, Lauderdale Academic Dermatology at Broward Medical Center, Ft. Lauderdale, FL Disclosures: None Correspondence: Brandon Nickle, DO; 4331 SW 78th Dr., Davie, FL 33328; Fax: 801-318-1431; [email protected] Abstract Wells’ syndrome, also referred to as eosinophilic cellulitis, is a rare inflammatory dermatosis. Although its etiology is unknown, it is thought to be an abnormal eosinophilic response to one of various causative agents. Its cutaneous manifestations vary in morphology and severity, but the disease often follows a relapsing-remitting course. We report the case of a patient with Wells’ syndrome preceded by leukocytoclastic vasculitis (LCV). Introduction bulla on the left lateral leg, and one perilesional syndrome. The patient was given a one-month Wells’ syndrome, also referred to as eosinophilic biopsy from skin directly adjacent to the bulla. course of prednisone and was free of symptoms cellulitis, is an uncommon inflammatory They were sent for direct immunofluorescence at three-month follow-up. She was lost to dermatosis, with fewer than 200 cases reported (DIF), which revealed an overwhelming number further follow-up. worldwide.2 It was first described in 1971 by of eosinophils in the superficial and deep dermis George Wells, noted as a rare entity with an (Figures 5, 6 [p. 46]). The biopsy was read as Discussion 1 Clinical Presentation unknown etiology. It often presents with bullous pemphigoid vs. Wells’ syndrome. The Wells’ syndrome, first described in 1971 as a a prodromal burning or pruritic sensation, negative DIF together with appropriate clinical “granulomatous dermatitis with eosinophilia,” is which is followed by a widespread eruption and pathologic correlation pointed to Wells’ commonly referred to as eosinophilic cellulitis consisting of urticarial erythematous papules, and characterized by acute pruritic dermatitis. plaques, and bullae of the trunk and upper and The clinical appearance can vary depending lower extremities. Head and neck involvement on the presenting variant. To date, there have has also been reported. Often, the cutaneous 1 been seven clinical variants described in the manifestations recur. Although the exact literature, including plaque, annular granuloma- pathophysiology of the disease is unknown, many like, urticaria-like, papulovesicular, bullous, associations have been reported. A diagnostic 4 papulonodular, and fixed drug eruption-like. criterion has been developed to aid physicians Morphologically, one of the most common in this uncommon diagnosis, but many cases of 3 presentations is a sudden onset of one to several Wells’ syndrome are still misdiagnosed. pruritic or painful, edematous, erythematous papules, nodules and/or plaques.1,5,6 Other Here, we present a patient with a one-year history of Figure 1. Multiple, 2 cm to 3 cm, non- LCV who subsequently developed Wells’ syndrome, blanchable purpuric vesicular plaques on left an association not yet reported in the literature. Whether this case depicts a causal association or lower extremity, clinically and histologically reflects a shared pathophysiology is unknown, but consistent with LCV. it may spur awareness of and further research into Wells’ syndrome in an effort to better understand the disease’s pathophysiology, diagnosis, and treatment options. Case Report A 55-year-old African-American female with a past medical history of hypertension presented to our clinic with a two-month history of a purpuric vesicular eruption of the lower extremities that was clinically and Figure 3. Multiple erythematous urticarial pathologically consistent with leukocytoclastic plaques on the chest. vasculitis (LCV) (Figures 1, 2). Her LCV was thought to be secondary to hydrochlorothiazide (HCTZ) therapy, which she had recently started for hypertension management. She was switched from HCTZ to lisinopril and attained a partial resolution of her symptoms. At three-month follow-up, the patient presented with a more widespread eruption consisting of scattered urticarial papules, nodules, and plaques involving the trunk and upper and lower Figure 2. Biopsy of left lateral leg revealing extremities, with occasional vesicles and bullae epidermal necrosis with neutrophils and in a similar distribution (Figures 3, 4). The neutrophilic debris in the epidermis. clinical differential was expanded to include In underlying dermis, neutrophils and bullous pemphigoid, arthropod assault, bullous Sweet’s syndrome, and Wells’ syndrome with or eosinophils are present along with significant without coexisting LCV. We performed two 4-0 leukocytoclastic debris, vessel fibrin, and Figure 4. Multiple erythematous urticarial mm punch biopsies: one lesional biopsy from a thrombi (H&E, 20x). plaques on the back. WELLS’ SYNDROME PROCEEDED BY LEUKOCYTOCLASTIC VASCULITIS: A DISCUSSION WITH NEW INSIGHTS INTO ANALOGOUS PATHOPHYSIOLOGY presentations include a sudden eruption of evidence that abnormal “homing” of eosinophils to systemic illness; (5) lack of evidence of triggering cellulitic lesions with blistering, and annular or target tissue via abnormal levels of IL-5 is involved factors, including insect bites, bacterial, viral, and circinate, erythematous plaques with infiltrated in the underlying pathophysiology.17,18 parasitic infections or infestations, as well as any borders.1 Lesions evolve over two to three days, kind of drug intake; (6) histopathologic changes with complete resolution in about two to eight Pathophysiology of eosinophilic dermatosis, with or without weeks. The disease recurs in many patients.1,7,8 In While the unique presentation of LCV and granulomatous features, with possible presence of a comprehensive review, Sinno et al. calculated Wells’ syndrome in this case may not represent typical flame-shaped figures; (7) negative direct a recurrence probability of approximately 56%.2 associated processes, it may offer insight into the immunofluorescence findings; and (8) inconsistent underlying pathophysiology of both diseases. blood eosinophilia.”3 Etiology Like Churg-Strauss and Sweet’s syndrome, The etiology of Wells’ syndrome is uncertain; LCV is a form of vasculitis characterized Histologically, dermal eosinophilic infiltration however, some important associations have by inappropriate infiltration of vessels by and, in the acute phase, peripheral eosinophilia been documented in the literature. Multiple inflammatory cells such as neutrophils.8 This differentiate Wells’ syndrome from infectious medications have been cited as causal agents, process is thought to be due to a dysregulation cellulitis.5 During the early phase of disease, including antibiotics, anticholinergic agents, in the complex process of inflammatory-cell dermal edema and diffuse dermal infiltration anesthetics, non-steroidal anti-inflammatory recruitment and homing to specific tissues. of eosinophils is seen. In the subacute phase, a agents, thyroid medications, chemotherapeutic In the case of LCV, one of the most common characteristic infiltrate of phagocytic histiocytes agents, thiomersal-containing vaccinations, findings is that of immune complex deposition and flame figures is seen on histopathology. and anti-TNF agents.1 Tugnet et al. reported a leading to the inappropriate recruitment of In later phases of disease, fewer eosinophils, case of Wells’ syndrome secondary to infliximab neutrophils to a tissue. Some studies also note histiocytes, giant cells, and flame figures are therapy.9 Of note, the thiazide group of drugs is dysregulation of key integrins and selectins present. Although important in the histologic known to cause a variety of cutaneous reactions, involved in leukocyte rolling and homing.19 diagnosis of Wells’ syndrome, flame figures are including LCV.1 However, an association not pathognomonic.1 Flame figures may be between thiazides and Wells’ syndrome has not In the case of Wells’ syndrome, one case study present in various other dermatologic conditions been reported. suggests that altered levels of IL-5 are involved including pemphigoid, severe prurigo, eczema, in inappropriate recruitment and homing of arthropod bites, Churg-Strauss and other 17 Wells’ syndrome has been linked to colonic eosinophils to tissues. Furthermore, an animal inflammatory conditions characterized by adenocarcinoma and several hematologic study in mice noted the importance of IL-5 eosinophilia.4 Thus, clinical correlation with diseases, including chronic lymphoid leukemia, and other cytokines in eosinophilia and tissue histologic findings is essential. malignant lymphoma, and polycythemia vera. homing, demonstrating how dysregulation of Nakazato et al. reported a case of Wells’ syndrome IL-5 could plausibly lead to eosinophilia and Based on the infiltrate pattern seen on in a patient with CML.10 Due to the association inappropriate recruitment of eosinophils into histopathology, a differential diagnosis for Wells’ between Wells’ syndrome and underlying specific tissues.18 Thus, while the exact underlying syndrome may include granuloma annulare, malignancy, investigation for underlying
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