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Erysipelas-Like Presentation of Wells' Syndrome (Eosinophilic Cellulitis)

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Erysipelas-Like Presentation of Wells' Syndrome (Eosinophilic Cellulitis) CASE REPORT Reumatismo, 2019; 71 (4): 226-229 Erysipelas-like presentation of Wells’ syndrome (eosinophilic cellulitis) N. Belfeki1, E. Gharbi1, C. Flateau2, S. Diamantis1 1Department of Internal Medicine; 2Department of Infectious Disease, Groupe Hospitalier Sud Ile de France, Melun, France SUMMARY Wells’ syndrome, also called eosinophilic cellulitis, is a rare eosinophilic dermatosis characterized by an unspe- cific inflammatory erythematous eruption often associated with systemic symptoms. Here we report the case of a 57-year-old female with bilateral painful pitting and pruritic feet progressive for two weeks despite one week of oral antibiotics. Skin biopsy was performed showing dermal eosinophilic infiltration. The patient showed a spontaneous progressive improvement of the condition. The presented case demonstrates both clinical and histo- logic presence of lesions of Wells’ syndrome in the course of the disease. A careful diagnostic approach is needed because of the lack of specific signs.The global outcome is favorable and spontaneous resolution is possible. Key words: Wells’ syndrome; eosinophilic cellulitis. only Reumatismo, 2019; 71 (4): 226-229 use n INTRODUCTION pain which appeared later. She had pre- viously received an ineffective course of ells’ syndrome, also called eosino- oral antibiotics (amoxicillin 1 g t.i.d for Wphilic cellulitis, is a rare eosinophilic 10 days) and denied any recent drug in- dermatosis characterized by an unspecific take, travel, insect bite or allergy. Physical inflammatory erythematous eruption often examination showed a high temperature associated with systemic symptoms. Histo- of 39°C, warm erythematous plaque of logically, the lesions are characterized by both feet associated with painful edema a superficial and deep-mixed inflammatory (Figure 1), swollen ankles and bilateral cell infiltrate containing eosinophils. It be- positive squeeze test. Other examinations longs to the large entity of eosinophilic der- showed normal cardiopulmonary auscul- matosesNon-commercial which can be idiopathic or associ- ated with autoimmune diseases or hemato- logical malignancy (1). Thus, a meticulous diagnostic approach is strongly needed to rule out alternative differential diagnoses. We report hereby the case of a female pa- tient presenting with bilateral feet swelling and general symptoms, and illustrate the diagnosis challenge in such a rare situation. n CASE REPORT A 57-year old female patient without Corresponding author: Nabil Belfeki past medical history was referred from Department of Internal Medicine, the emergency room for bilateral pain- Groupe Hospitalier Sud Ile de France, 77000 Melun, France ful pitting and pruritic feet lasting two Figure 1 - Diffuse erythematous plaque of E-mail: [email protected] weeks with prolonged fever, and joint both feet. 226 Reumatismo 4/2019 CASE Erysipelas-like presentation of Wells’ syndrome (eosinophilic cellulitis) REPORT Figure 2 - Skin biopsy taken from the feet Figure 3 - Sclerodermiform-like evolution of showing dermal edema and dermal infiltra- both feet 4 weeks after diagnosis. tion by eosinophils consistent with Wells’ syn- drome. Hematoxylin and eosin stain. only tation, and neither lymph nodes enlarge- ruled out ANCA mediated vasculitis. Feet ment nor hepatomegaly or splenomegaly. MRI did not show imagesuse of fasciitis and Routine biological investigation showed eosinophilic fasciitis (Shulman’s fasciitis) an inflammatory syndrome with elevated was ruled out. The patient received symp- C reactive protein (200 mg/dL), normal tomatic treatment. Paracetamol (1 g quid) cell blood count, normal electrolytes, for fever and joint pain and cetirizine (5 creatinine and liver enzymes. Hepatitis mg bid). She responded to bed rest. Af- B, C, and HIV serologies were negative. ter one week in hospital, her temperature 24-h-proteinuria and urine test strips were dropped while the joint pains and pruri- normal. Anti-neutrophil cytoplasmic an- tus disappeared. The skin lesions healed tibodies (ANCA), antinuclear antibodies progressively with at first a slight hyper- (ANA), rheumatoid factors (RF), and an- pigmentation resembling systemic scle- ti-cyclic citrullinated peptides antibodies rosis (Figure 3) 4 weeks after discharge, (ACPA) were negative. Thoracoabdomi- which disappeared completely with ad nopelvic scanner was normal and feet integrum restitution three months later. MRI demonstrated swellingNon-commercial limited to the At 12 months, clinical evaluation did not subcutaneous tissue. Skin biopsy showed show relapse of cellulitis or symptoms edema in the dermis and infiltration of the and signs related to an associated autoim- dermis by eosinophils. No signs of vascu- mune disease or malignancy. litis were shown. Tissue direct immuno- fluorescence was negative for IgG, IgA, n DISCUSSION IgM, IgE, and complement (Figure 2). AND CONCLUSIONS Bacterial cellulitis was ruled out because of negative blood culture and an inefficient Wells’ syndrome is a rare inflammatory antibiotics course. Anamnesis did not find skin condition presenting commonly with any past history of allergy or recent drug pruritic cellulitis-like plaques. There is a intake, there were no eosinophilia on CBC large polymorphism in the clinical fea- and Ig E level was normal. Thus, allergic tures with the development of annular contact dermatitis and/or drug reaction or circinate erythematous-edematous with eosinophilia and systemic symp- plaques or blistering, nodules, papulov- tom (DRESS) were excluded. Moreover, esicular eruptions, and excoriated pap- clinical and immunological investigations ules have been reported. In our case, the Reumatismo 4/2019 227 CASE REPORT N. Belfeki, E. Gharbi, C. Flateau, et al. patient had a common presentation, with That is to say that Well’s syndrome can bilateral feet progressive cellulitis mim- be idiopathic. Neither did we reveal blood icking erysipela and absence of response eosinophilia, which is reported in 50% of to antibiotics. Anamnesis ruled out the cases, and its level seems to be correlated hypothesis of insect bites, drug intake or to the severity of presentation (6). Once allergic contact dermatitis that may mimic the diagnosis of Wells’ syndrome is sus- this presentation. Extra dermatological pected, as based on clinical findings, it reported features were fever and inflam- is corroborated by histopathological ex- matory joints pain with markedly elevated amination of a skin biopsy specimen. A inflammatory parameters. Throughout her careful diagnostic approach is needed to course in hospital, the CT scan ruled out rule out differential diagnoses, which is a occult infection, hematological malig- real challenge because of its rarity. Only nancy, or solid tumor. Moreover, feet MRI 200 cases have been reported among pub- ruled out necrotizing fasciitis and showed lished data (7). Based on the case reports swelling limited to the subcutaneous tis- reviewed, Wells’ syndrome is often mis- sue. A skin biopsy was performed and diagnosed and, thus, inappropriately treat- pathological findings showed mild edema ed. Many treatments have been used with with dermal eosinophilic infiltration with- variable success.only It should be first noted out signs of vasculitis. The absence of past that antibiotic therapy is characteristically history of asthma, polyneuropathy, ab- ineffective. The most common and effec- dominal pain, eosinophilia and negativity tive treatment is steroids (oral and/or top- of anti-neutrophil cytoplasmic antibodies useic). Antihistamines can be administered could rule out the hypothesis of eosino- to relieve pruritus, but they are ineffec- philic granulomatosis with polyangiitis. tive in clearing cutaneous lesions (1, 8). The literature review (The PubMed and In case of relapses, ciclosporin or dapsone Ovid MEDLINE database Embase us- were effective when used alone and as an ing the keywords eosinophilic cellulitis adjunct to systemic steroids to avoid the and Wells’ syndrome) of histopathological negative side effects of long-term high- findings revealed that dermal edema and dose steroid use (9). In case of association eosinophilic infiltration were present in all with blood eosinophilia, mepolizumab cases and had to be considered as the gold (anti IL-5 monoclonal antibody) seems to standard for diagnosis of Wells’ syndrome be an interesting treatment to target option (1). Histopathological findings in Wells’ according to Busse et al. (9). We did not syndrome varies between biopsy times, a introduce steroids and treated the patient fact that complicates interpretation. Dur- symptomatically. The patient recovered ing Non-commercialthe acute phase, we observe mainly progressively and skin lesions disappeared dermic eosinophilic infiltration while progressively 3 months later. In fact, 12% during the subacute phase, flame figures of the reported cases showed a favorable are the hallmark picture. When chronic spontaneous outcome (7). Further studies lesions are biopsied, we may have gran- are needed to carify the best approaches uloma with giant cells (2, 3). The flame to treatment. figures consist of eosinophilic major ba- sic protein deposited on collagen bundles, n REFERENCES and widespread degranulation of eosino- phils is not to be considered as a pathog- 1. Sinno H, Lacroix JP, Lee J. Diagnosis and nomonic histopathological indicator of management of eosinophilic cellulitis (Wells’ Well’s syndrome. They may be detected syndrome): A case series and literature review. in other conditions such as bullous pem- Can
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