Eosinophilic cellulitis and dermographism

Nathalie Q Nguyen MD and Linglei Ma MD PhD
Dermatology Online Journal 11 (4): 7 Department of Dermatology,
New York University School of Medicine

Abstract

A 26-year-old man presented with a history of intermittent erythematous plaques on his hands and legs. A peripheral blood eosinophilia was noted. Histopathologic examination showed numerous eosinophils and characteristic flame figures. The clinical presentation and histopathologic alterations are consistent with the diagnosis of Wells' syndrome, which is also known as eosinophilic cellulitis. Wells' syndrome is a rare condition of unclear etiology. We discuss its diagnosis and possible association with other conditions that manifest peripheral eosinophilia.

A 26-year-old man presented to the Charles C. Harris Skin and Cancer Pavilion with an eruption on his hands that had been recurring for 4 years. During the summer months, he noted the appearance of tender, red plaques and nodules on his palms, which spontaneously resolved. Over the past year, the red plaques and nodules recurred on his palms, and he noticed new lesions on his thighs. At one point, blisters developed on one of the red plaques on his left hand. The patient was treated with doxycycline, dicloxacillin, and clarithromycin, which failed to improve his symptoms. The patient has received several courses of prednisone, with clearance of his eruptions. He denies fevers, chills, malaise, or weight loss. Past medical history includes allergic rhinitis, eczema, and asthma.

Multiple, erythematous plaques were present on the palms and dorsal aspects of the hands. There were several, well-circumscribed, hyperpigmented macules. On the anterior thighs were multiple, circinate, erythematous plaques. A brisk, mechanical stroke elicited a linear wheal.

A complete blood count showed 16.7 percent eosinophils, with an absolute eosinophil count of 1653 cells/mcL. Basic metabolic panel, liver function tests, thyroid function tests, and serum protein electrophoresis were normal.

Histopathology reveals a superficial and deep, perivascular and interstitial, mixed-cell infiltrate of lymphocytes with innumerable eosinophils, which extends into the subcutaneous adipose tissue. Among dermal collagen bundles, there are collections of eosinophils with eosinophilic granules (flame figures).

Comment

Wells' syndrome was first described in 1971 and later renamed eosinophilic cellulitis [1]. Typical lesions present as single or multiple, erythematous patches and plaques that clinically resemble acute cellulitis. However, the plaques fail to resolve with antibiotic therapy. Acute, erythematous and edematous plaques extend peripherally. The intensity and rapidity of progression mimics that of an acute cellulitis. However, lesions are often cool to the touch. Most patients experience mild burning and itching. Although erythematous, edematous plaques are the most common presentation, nodules, bullae, or a papulonodular eruption may occur [2, 3, 4]. Within a few days, the inflammation subsides, and the erythema is replaced with a gray-blue hue.

The diagnosis of eosinophilic cellulitis is based on clinical and histopathologic features. In addition to the cutaneous manifestations, patients also present with peripheral blood eosinophilia. Histopathologic examination shows a dense infiltrate of eosinophils with degranulation. In older lesions, the granular material aggregates focally around collagen fibers to form the characteristic flame figures [5]. These eosinophilic bodies are not pathognomonic for eosinophilic cellulitis and have been described in other inflammatory conditions, such as eczema, prurigo, herpes gestationis, scabies, and bullous pemphigoid [6].

Eosinophilic cellulitis has been proposed to be in the same spectrum of diseases as other disorders with peripheral eosinophilia, such as idiopathic hypereosinophilic syndrome (HES) and Churg-Strauss syndrome [7]. However, patients with eosinophilic cellulitis rarely show systemic symptoms unlike patients with HES, who present with pulmonary, gastrointestinal, or neurologic symptoms. Cutaneous involvement is found in approximately 50 percent of HES patients, and it has been postulated that perhaps eosinophilic cellulitis may be a cutaneous manifestation of HES rather than a separate entity [7, 8]. Wells' syndrome has also been reported to be precipitated by arthropod bites, fungal infections, medications, and parasites.
Treatment options include antihistamines, prednisone, and cyclosporine. Anecdotal use of griseofluvin, minocycline, and dapsone has been reported [4].

References1.

Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol 1979;100:1012.
Ghislain PD, Van Eeckhout P. Eosinophilic cellulits of papulonodular presentation (Wells' syndrome). Eur Acad Dermatol Venereol 2005;19:2263.
Holme SA, McHenry P. Nodular presentation of eosinophilic cellulitis (Wells' syndrome). Clin Exp Dermatol 2001;26:6774.
Ling TC, et al. Two cases of bullous eosinophilic cellulitis. Br J Dermatol 2002; 146:1605.
Abell E. Inflammatory diseases of the epidermal appendages and of cartilage. In Elder D, et al, eds. Lever's Histopathology. 8th ed. Philadelphia: Lippincott Williams & Wilkins;1997;18:409.6. Ferreli C, et al. Eosinophilic cellulits (Wells' syndrome): a new case description. J Eur Acad Dermatol Venereol 1999;13:417.
Fujii K, et al. Eosinophilic cellulitis as a cutaneous manifestation of idiopathic hypereosinophilic syndrome. J Am Acad Dermatol 2003;49:11748.
Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994; 83: 2759

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