Indian Dermatology Online Journal

: 2020  |  Volume : 11  |  Issue : 6  |  Page : 979--982

Two cases of wells syndrome with marked swelling in the hands

Yukina Watanabe, Miyuki Yamamoto, Syohei Igari, Toshiyuki Yamamoto 
 Department of Dermatology, Fukushima Medical University, Fukushima, Japan

Correspondence Address:
Yukina Watanabe
Department of Dermatology, Fukushima Medical University, Hikarigaoka 1, Fukushima - 960-1295


Wells syndrome or eosinophilic cellulitis is an idiopathic inflammatory dermatitis characterized by a benign but recurrent evolution. It often causes edematous urticarial plaques on the extremities. Herein, we report two rare cases of Wells syndrome with marked swelling in the hands. Both cases exhibited various clinical features. Case 1 was Wells syndrome with collagen disease-like sclerotic edema in the fingers and annular erythema on the trunk. Case 2 was Wells syndrome with diffuse plate-like hardening of the forearm mimicking cellulitis, which required fasciotomy due to suspected compartment syndrome at the emergency room. Wells syndrome should be included in the differential diagnosis of patients presenting with marked diffuse swelling in the hands.

How to cite this article:
Watanabe Y, Yamamoto M, Igari S, Yamamoto T. Two cases of wells syndrome with marked swelling in the hands.Indian Dermatol Online J 2020;11:979-982

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Watanabe Y, Yamamoto M, Igari S, Yamamoto T. Two cases of wells syndrome with marked swelling in the hands. Indian Dermatol Online J [serial online] 2020 [cited 2021 Jan 24 ];11:979-982
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Full Text


Wells syndrome is a relapsing eosinophilic dermatitis with variable clinical appearance. Due to symptoms of erythema and swelling of the limbs, it is often misdiagnosed as cellulitis or contact dermatitis. However, cases of Wells syndrome with extending swelling from the arms to hands are rare. Herein, we describe two cases of Wells syndrome with marked swelling in the hands.

 Case Reports

Case 1

A 61-year-old woman with hemorrhoids and arterial hypertension presented to our hospital with painful swelling, erythema, purple spots, and blisters on both hands, from fingers to wrists. Hemorrhagic plaques were observed in the palms. She had difficulty in bending her fingers and had finger sclerotic edema, which exhibited sausage-like swelling [Figure 1]a and [Figure 1]b. In addition, her trunk and extremities were erythematous with itching. The initial clinical diagnosis made by a local hospital had been contact dermatitis, and she was treated with oral prednisolone (10 mg/day), which improved her symptoms. However, she was referred to our hospital because of recurrence. Laboratory examination revealed an elevated C-reactive protein level (1.57 mg/dL), although complete blood count, liver function test, renal function tests and serum complements were normal. A biopsy specimen taken from the right thumb revealed inflammatory infiltrate of eosinophils in all layers of the dermis, and flame figures were observed in the dermis [Figure 2]a and [Figure 2]b. There was no vasculitis. The patient was diagnosed with Wells syndrome and was successfully treated with betamethasone butyrate propionate ointment and olopatadine hydrochloride tablets.{Figure 1}{Figure 2}

Case 2

A 13-year-old boy presented with swelling and pain on his left arm. He had noticed insect bites on his left palm 10 days before the presentation. At an internal medicine hospital, he was suspected to have cellulitis and underwent treatment for it with oral antibiotics for 3 days. However, the swelling further extended to his left fingers and shoulder, and purpuric lesions were scattered on the flexion aspect of the forearm [Figure 3]. The patient was, therefore, admitted to our hospital with suspected necrotizing fasciitis. His white blood cell count was within normal limits, but his blood eosinophil count was elevated (33%). The compartmental pressure of his left forearm was elevated (50–60 mmHg), and the patient was diagnosed as having compartment syndrome. He underwent fasciotomy, and no necrosis was observed from the subcutaneous tissue to the muscle tissue. A biopsy specimen taken from the forearm revealed inflammatory infiltrate of eosinophils in all layers of the dermis, as well as flame figures [Figure 4]a and [Figure 4]b, and all bacterial cultures were sterile. He was successfully treated with prednisolone (25 mg/day), which was gradually tapered within 3 months.{Figure 3}{Figure 4}


Wells syndrome affects both adults and children,[1] and presents with tender urticarial erythematous plaques, vesicles, bullae, or nodules.[2] To date, only six cases of Wells syndrome with marked swelling in the hands have been reported.[3],[4],[5],[6],[7],[8] The patient characteristics of those cases, along with the present cases, are shown in [Table 1]. All patients had lesions on one side, and three patients had pruritic erythema on the trunk and extremities. All cases were successfully treated with topical and systemic corticosteroids, but two patients experienced recurrence. The usual course of the condition is spontaneous remission. In Case 1, spontaneously resolution may be considered. Both of our cases developed hemorrhagic plaques or purpuric lesions. Ratzinger et al.[9] suggested that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells syndrome and Churg–Strauss syndrome. Eosinophilic leukocytoclastic vasculitis has a mild course and can be cured after an episode, while patients with Churg–Strauss syndrome have multiple flare-ups. Whether Wells syndrome is to be classified as vasculitis or not is still controversial; and in our cases, vasculitis was not observed histologically.{Table 1}

Case 1 additionally developed annular erythema on the right lower extremity, which may require the differentiation with eosinophilic annular erythema (EAE). In the original paper by Wells et al.,[10] various clinical features of Wells syndrome were reported, including cellulitis-like erythema with or without some blistering and annular or circinate erythematous plaques. Thereafter, several cases of Wells syndrome with an annular erythematous lesion have been reported. There are a number of inflammatory skin diseases presenting with annular lesions, and Wells syndrome should be included in such disorders. Some authors view EAE as a variant of Wells syndrome, while other authors believe that slight differences can make this differential diagnosis possible.[11],[12] EAE histologically lacks flame figures and degranulation of eosinophils, whereas perivascular infiltration of mononuclear cells and eosinophils are observed in the superficial and deep dermis. Therefore, whether EAE is a distinct entity or not depends on further studies. Eosinophil-derived proteins, that is, major basic proteins play roles in allergic inflammation, tissue damage and repair, remodeling, and fibrosis. The secretion of eosinophil proteins may play a role in the induction of prominent swelling of the hands with transient joint contracture. Wells syndrome should be included in the differential diagnosis of patients presenting with marked diffuse swelling in the acral sites.

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