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A Rare Case of Well's Syndrome

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A Rare Case of Well's Syndrome A Rare Case of Well’s Syndrome Donna Tran, DO,* Nicole Rouse, BS,** Paul Shitabata, MD,*** Leela Athalye, DO,**** Navid Nami, DO***** *Dermatology resident, PGY4, Western University of Health Sciences, Pomona, CA **Medical student, Western University of Health Sciences, Pomona, CA ***Director of dermatopathology, Western University of Health Sciences, Torrance, CA ****Dermatologist, Island Dermatology, Newport Beach, CA *****Program director, Dermatology Residency Program, Western University of Health Sciences, Pomona, CA Disclosures: None Correspondence: Donna Tran, DO; [email protected] Abstract Wells’ syndrome is a rare, idiopathic dermatosis characterized clinically by an acute, erythematous plaque resembling cellulitis and histopathologically by an eosinophilic dermal infiltration and flame figures. Also known as eosinophilic cellulitis, it was first described by Wells in 1971, and since then only 80 to 100 cases have been reported. The etiology of Wells’ syndrome is largely unknown.3-6 Although often self-limited, the disease has a tendency to recur. We describe a rare case of Wells’ syndrome presenting on the left lower leg of a 68-year-old woman and review the literature on the topic. Case Report significantly with topical high-potency steroids. infiltration results in bullae formation. Blisters A 68-year-old woman with no significant of Wells’ syndrome contain eosinophils and are medical history presented with a pruritic eruption predominantly subepidermal, multiloculated, and Discussion 6 on her left lower extremity of several months’ Wells’ syndrome, also known as eosinophilic spongiotic. duration. She denied history of insect bite or cellulitis, is a rare but usually benign dermatosis Peripheral blood eosinophilia may or may not be trauma prior to the onset of the lesion. Physical that has been reported in all age groups.6 It often found. Other laboratory findings associated with examination revealed a large erythematous presents as a mildly pruritic, tender, erythematous Wells’ Syndrome include increased eosinophilic plaque with surrounding erythematous papules plaque that most frequently affects the cation protein (ECP), and increased interleukin-5 over the lateral aspect of the left lower extremity extremities. The edges are annular or arcuate with (IL-5). ECP and IL-5 levels correlate with (Figure 1). Laboratory investigations, including a violaceous borders.8 The plaque’s color generally severity of disease. complete blood count and total leukocyte counts, evolves from bright red to gray over the course of Although its etiology is largely unknown, the were within normal limits. a few days, and it often resolves without scarring.6 proposed mechanism of Wells’ syndrome is Although Wells’ syndrome most commonly Histopathologic examination from a biopsy of the an activated line of CD3+ and CD4+ T-cells presents as a plaque or nodule, it may also present plaque revealed a superficial and deep infiltrate of that result in hyper-secretion of IL-5. This as papules, vesicles, or hemorrhagic bullae. lymphocytes and eosinophils with flame figures cytokine promotes eosinophil recruitment and (Figures 2, 3). Based on clinicopathologic The diagnosis of Wells’ syndrome is based on degranulation.10 Eosinophils play a role in correlation, a diagnosis of Wells’ syndrome clinical features and histopathologic findings of parasitic and allergic reactions. They contain toxic was established. Her skin lesions improved eosinophilic infiltration limited to the epidermis cationic proteins that play a role in inflammation and dermis, though it may extend into the and local tissue damage. MBP damages parasites, Figure 1 subcutaneous tissue and underlying muscle. An but also damages mammalian cells and tissue. infiltrate of eosinophils, eosinophilic debris, and Eosinophil-derived neurotoxin plays a role histiocytes between collagen bundles form the against viral infection, but as the name implies, classic “flame figures.” Eosinophilic degranulation it is neurotoxic. Other proteins secreted by may also be visualized with immunofluorescent eosinophils promote histamine release, neutrophil stains showing eosinophilic major basic protein activation, tumor lysis, peroxidation, serotonin (MBP).9 Infiltration into the epidermis causes release, and clot formation.6 The various functions epidermal spongiosis and vesiculation. Subdermal of eosinophils are useful in many circumstances, Figure 2 Figure 3 Figure 2. (H&E, 10x) Low-magnification view Figure 1. Erythematous plaque on lateral aspect revealing a dense superficial and deep dermal Figure 3. (H&E, 40x) High-magnification view of left lower extremity. infiltrate of eosinophils and lymphocytes. revealing flame figure. TRAN, ROUSE, SHITABATA, ATHALYE, NAVID NAMI Page 49 but may generate edema, erythema, pruritus, and References 18. Omiya W, Fujita Y, Baba K, Shibaki A, Odani tissue damage in the presence of eosinophilia. 1. Wells GC. Recurrent granulomatous dermatitis T, Shimizu H. Unusual cutaneous manifestations Although flame figures are a hallmark of Wells’ with eosinophilia. Trans St Johns Hosp Dermatol of Churg-Strauss syndrome mimicking Wells’ syndrome, they are not a specific finding. They Soc. 1971;57(1):46-56. syndrome. Eur J Immunol. 2012;22(4):576-8. can be found in other conditions with eosinophil- 2. Wells GC, Smith NP. Eosinophilic cellulitis. 19. Spinelli M, Frigerio E, Cozzi A, Garutti C, rich infiltrate, such as atopic dermatitis, contact Br J Dermatol. Jan 1979;100(1):101-9. Garavaglia MC, Altomare G. Bullous Wells’ dermatitis, tinea, scabies, arthropod bites, and syndrome associated with non-Hodgkin’s bullous pemphigoid. Infectious cellulitis and 3. Hirsch K, Ludwig RJ, Wolter M, et al. lymphocytic lymphoma. Acta Derm Venereol. erysipelas are very similar in clinical presentation Eosinophilic cellulitis (Wells’ syndrome) 2008;88(5):530-1. to Wells’ syndrome, but differ in that their associated with colon carcinoma. J Dtsch 15 Dermatol Ges. Jul 2005;3(7):530-1. 20. Herr H, Koh JK. Eosinophilic cellulitis (Wells’ infiltrates are predominantly neutrophilic. syndrome) successfully treated with low-dose Febrile eosinophilic cellulitis with toxocariasis 4. Kaufmann D, Pichler W, Beer JH. cyclosporine. J Korean Med Sci. 2001;16(5):664- infection presents with eosinophilia, migrating Severe episode of high fever with rash, 16 8. cellulitis, and Toxocara larvae on serology. lymphadenopathy, neutropenia, and eosinophilia Other differentials to consider include drug after minocycline therapy for acne. Arch Intern eruption, granuloma annulare, hypereosinophilic Med. 1994;154(17):1983-4. syndrome, chronic urticaria, and Churg-Strauss syndrome. 5. Koga C, Sugita K, Kabashima K, Matsuoka H, Nakamura M, Tokura Y. High responses of Wells’ syndrome has an excellent prognosis; peripheral lymphocytes to mosquito salivary however, it has the tendency to recur, which gland extracts in patients with Wells syndrome. J may prolong resolution.6 Systemic associations Am Acad Dermatol. Jul 2010;63(1):160-1. occur in fewer than 25% of patients with Wells’ syndrome and can include malaise, fever, 6. Bolognia JL, Jorizzo JL, Schaffer JV. arthralgia, and asthma, among other conditions. Dermatology. Elsevier Saunders; 2012. An association between the bullous form of Wells’ 7. Brehmer-Andersson E, Kaaman T, Skog E, disease and non-Hodgkin lymphoma has been Frithz A. The histopathogenesis of the flame reported.19 The most common complication of figure in Wells’ syndrome based on five cases. Wells’ syndrome is blistering; however, long-term Acta Derm Venereol. 1986;66(3):213-9. complications, such as reticular pigmentation and 8. Fisher GB, Greer KE, Cooper PH. Eosinophilic scarring alopecia, do rarely occur.6 cellulitis (Wells’ syndrome). Int J Dermatol. Treatment of Wells’ syndrome depends on its 1985;24(2):101-7. severity. Mild cases may resolve with potent topical 9. Peters MS, Schroeter AL, Gleich GJ. corticosteroids. Severe cases usually improve Immunofluorescence identification of eosinophil dramatically with systemic corticosteroids. granule major basic protein in the flame Calcineurin inhibitors, griseofulvin, figures of Wells’ syndrome. Br J Dermatol. Aug antihistamines, cyclosporine, dapsone, colchicine, 1983;109(2):141-8. minocycline, and antimalarials have also been proven effective.20 10. Simon HU, Plotz S, Simon D, et al. Interleukin-2 primes eosinophil degranulation in hypereosinophilia and Wells’ syndrome. Eur J Conclusion Immunol. 2003;33(4):834-9. Wells’ syndrome is a rare condition with few documented cases and should be included in 11. Blanchard C, Rothenberg ME. Biology of the the differential of resistant cellulitis, granuloma eosinophil. Adv Immunol. 2009;101:81-121. annulare, chronic urticaria, and drug eruption. 12. Rothenberg ME, Hogan SP. The eosinophil. A detailed history regarding triggers and Annu Rev Immunol. 2006;24:147-174. medications should be done to exclude other diseases that can mimic Wells’ syndrome clinically 13. Aberer W, Konrad K, Wolff K. Wells’ and histologically. The lesions generally respond syndrome is a distinctive disease entity and not well to treatment but have a tendency to recur. a histologic diagnosis. J Am Acad Dermatol. 1988;18:105-114. 14. Haddad F, Helm TN. Wells syndrome. Cutis. Jan 2014;93(1):17,38-9. 15. Falagas ME, Vergidis PI. Narrative review: diseases that masquerade as infectious cellulitis. Ann Intern Med. 2005;142:47-55. 16. Bassukas ID, Gaitanis G, Zioga A, Boboyianni C, Stergiopoulou C. Febrile “migrating” eosinophilic cellulitis with hepatosplenomegaly: adult toxocariasis - a case report. Cases J. 2008;1:356. 17. Shams M, Hudgens J, Lesher JL Jr., Florentino F. Wells’ syndrome presenting as a noninfectious bullous cellulitis in a child. Ped Dermatol. 2012;29(2):224-6. Page 50 A RARE CASE OF WELL’S SYNDROME.
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