Recurrent and Diffuse Eczema with Severe Itch and Peripheral Eosinophilia in Healthy Middle Aged Woman

A 45-year-old woman presented with generalized itchy skin lesions particularly more on extremities and back. Lesions were present since past 3 years and were not related to injury, insect bites or drug intake. There was no history of similar complaints in her family.

On examination, there were multiple, hyperpigmented patches and plaques varying from 3 × 10 to 5 × 15 cms in size with many excoriated papules and crusts [Figures 1 and 2]. The systemic examination was unremarkable and peripheral blood examination showed high eosinophilic count of 40% and absolute count of 6640. Chest X-ray was normal as well as renal and liver function tests were normal. For clinical diagnosis, a punch biopsy was taken from the back and histopathological report showed changes as shown in [Figures 3 and 4].

Figure 1.

Excoriated nodules over ear pinna

Figure 2.

Typical hyperpigmented plaques with excoriations over lower limbs

Figure 3.

Dermis showing inflammatory cellualar infiltration of eosino-lympho-histiocytes and flame figures in deeper dermis (H and E, ×400)

Figure 4.

Close up view of flame figure (H and E, ×400)

Question

What is your diagnosis?

Answer

Well's Cellulitis (Eosinophilic cellulitis).

Microscopic findings and clinical course

Histopathological report showed changes mainly in deeper dermis like diffuse eosinophilic infiltrations and diagnostic flame figures (bright red on H and E stains) in [Figures 3 and 4]. With the features pictures [Figures 1 and 2] and haematological changes point towards gross peripheral eosinophilia.

Patient responded to tapering courses of systemic steroids and antihistamines. Response to treatment was temporary and relapses were common.

Discussion

Wells syndrome is a rare clinical condition of skin which mimics many common dermatological conditions. It belongs to small group of conditions due to hyper eosinophilic infiltration. It has chronic and relapsing course for few years, which responds to steroids. effectively.

This condition described by George Crichton Wells in 1971, has a diagnostic histopathological feature i,e. eosinophilic flame figure, was named as “recurrent granulomatous dermatitis with eosinophilia”.[1] However, in 1979, the name was simplified to the present term “eosinophilic cellulites” or “Wells syndrome".[2] Approximately, more than 100 cases have been reported, which are seen from new born to aged, but association with systemic diseases is rare.[3] Blood eosinophilia is usual but not invariable.

Although the exact etiology is unknown, it is probably due to hypersensitivity reaction to various agents like parasitic infection, dermatophyte infection, insect bites, and drugs. Rarely, reaction to injections of etanercept[4] and adalimumab[5] are also known to produce a similar picture.

Clinically, the course is divided in to an acute phase called eosinophilic cellulitis (as it resembles to bacterial celluliits), which lasts for a week, and a chronic phase called granulomatous phase of severe itching, lasting for nearly 1-3 months.

Histopathology is usually diagnostic showing mainly dermal changes of eosinophilic panniculitis which consists of fragmented eosinophilic granules (flame figure). The collagen in the middle of the flame figure is bright red on H and E stains, but periphery shows nuclear debris, eosinophilic granules, macrophages and rarely foreign body giant cells. However, no vasculitis changes are seen.[6]

The condition has to be differentiated clinically from bacterial cellulitis, insect bite and erysipelas in the early stages. Later lesions have to be differentiated meticulously from erythema elevatum diutinum, chronic urticarias, morphea, lymphomas, atropy etc.[7]

Wells syndrome can be a benign end of the hypereosinophilic syndrome spectrum but it is confined to cutaneous system. Allergic granulomatosis is similar to eosinophilic cellulitis histopathologically: Nevertheless, it is a true vasculitis and has basophilic rather than eosinophilic necrobiosis.[8]

In treatment, oral steroids will give a dramatic relief within days and course is typically tapered over one month. Other drugs used are low-dose cyclosporine,[9] griseofulvin,[10] dapsone, minocycline,[11] etc. Mild cases may respond to oral antihistamines and topical steroids.

Take home messages: Do not despose chronic cases without doing a skin biopsy, biopsy should be done routinely in such cases for diagnosis and therapeutic purpose.