Unusual presentation of wells syndrome: case reportAbstract:Wells syndrome is an uncommon diseases, typically presents as edematous erythematous plaques usually preceded by burning or itching of the skin. Histopathological examination shows dense dermal eosinophilic infiltrates in an edematous dermis at the acute phase of lesions. Some of the identified triggering factors include infection, arthropod bites, hematologicalmalignancies, thimerosal containing vaccines and drugs such as Penicillin, leucomycins, tetracycline, minocycline and ampicillin.
Here we describe a case of Wells’ syndrome in a 75 -year-old female that its outstanding feature was its large size. Although this case was resistant to our treatment, the condition improved spontaneously after several weeks without administering any other alternative treatments. On the other hand, despite its large size, this case had no identifiable trigger.Key words: wells syndrome; eosinophilic cellulitis; flame figures. Introduction:Wells syndrome (WS) is an uncommon inflammatory diseases initially described in 1971 by DrGC Wells (1). It is characterized by pruritic annular erythematous plaques with infiltrated borders which mainly affects the limbs or trunk and has a relapsing and remitting course (2-6).Typical lesions include a wide spectrum from a mild form circinate red plaques to more severe forms such as blistering or a sudden eruption of cellulitis-like lesions, sometimes associated with burning or itching sensation. It may persist for over months to years but usually resolves spontaneously without scarring (2, 3, 6, 7).
Wells’ syndrome usually doesn’t have any systemic involvement, but peripheral blood eosinophilia is a common feature (5, 8).There are various therapeutic options but Low-dose oral corticosteroids is the first-line treatment in Wells’ syndrome (5, 9).We describe a case of Wells’ syndrome in a 75 -year-old female that its outstanding feature was its large size, although this case was resistant to our treatment the condition improved spontaneously after several weeks without administering other treatments. On the other hand, this case had no identifiable trigger.Case repot:A 75 year-Old Iranian woman presented with 1-month history of itching, an infiltrating erythematous plaque on abdomen which initiates from peri umbilical but extends in size and spreads bilaterally to the flanks. It was the first episode having no similar or recurrent lesions before.
She denied neither systemic nor cutaneous associated symptoms other than itching of the lesion. There was no history of any drug intake, insect bites or any known food or other substances as a trigger for the condition. Her medical history, family history and drug history were all negative.Physical examination revealed an erythematous – violaceous, well-defined, annular indurated plaque affecting abdomen skin approximately 80 cm* 35 cm in size without any vesicles or bullae on the surface. The indurated plaque had a pseud orange appearance. In addition, it was nor tender on palpation neither scaly on observation (Figure 1). General physical examination was completely normal .The patient was afebrile and there was no associated lymphadenopathy.
Laboratory results were unremarkable, except mild serum eosinophilia (6%, 384/mm3).CBC, ESR, CRP and other routine biochemical tests were within normal limits.The punch biopsy was taken with differential diagnosis of eosinophilic cellulitis, inflammatory morphea, granuloma annular, sarcoidosis, and interstitial granulomatous disease. A biopsy of the plaque revealed a patchy infiltration of lymphocytes and eosinophils extending from superficial to deep dermis .There was also mild vasculopathy and scattered flame figures, which represented eosinophilic granules encrusted on dermal collagen (Figure 2, 3).
These findings were consistent with a diagnosis of Wells syndrome.After two weeks the centrifugal expansion of plaque was obvious by its discrete raised, infiltrative sharply red border along with the fading hyper pigmented center. Prednisolone was initiated at a dose of 40 mg daily (0.5 /kg), betamethasone valerate cream along with emollient _eucerin _ were applied topically and oral cetirizine was administered for the pruritus.
However, the patient discontinue the therapy after 2 weeks science there was no improvement of the skin lesions. Despite there was no additional medical intervention, the lesions began to clear spontaneously without scarring or dyspigmentation after three weeks. Discussion:Wells syndrome (eosinophilic cellulitis), is a rare condition of unknown etiology, (4, 5, 10)typically presents as erythematous patches or non-scaly edematous plaques usually preceded by burning or itching of the skin. The severity of the symptoms vary from a mild to severe pruritus as well as tenderness of the lesions (4, 6, 9). There are also various manifestations have been reported in the literature including vesicles, bullae, granuloma-like, multiple arcuate erythematous plaques , as well as widespread papulonodular or even fixed drug eruption-like lesions (2,4,5,9).The typical course of disease often includes a central involution initially and turning from a red-brown color to a grey-blue color while becoming indurated in later stages , frequently the lesions involute over a period of two to eight weeks and finally the skin clears without scarring.
However it sometimes results in morphea-like atrophic hypopigmented patches (4, 6, 8). It occasionally may be associated with systemic involvement such as fever, arthralgia, lymphadenopathy and rarely anterior uveitis (10).Although the etiology of the disease remains unclear, a type IV hypersensitivity reaction and abnormal eosinophilic response to several exogenous and endogenous causative agents is considered to be the main underlying pathophysiology of eosinophilic cellulitis (4, 10).
Some of the aforementioned triggering factors include infection, arthropod bites, hematological disorders malignancies, thimerosal containing vaccines and drugs such as Penicillin, lincomycin, tetracycline, minocycline, and ampicillin (5, 9, and 10).Histopathologically, there are three stages: acute lesions show dense dermal eosinophilic infiltrates in an edematous dermis. Subacute lesions characterized by giant cells, histiocytic, eosinophils and flame figures in the dermis and the last stage with micro granulomas consisting of foreign bodytype giant cells (8, 10).Here, we report a case of idiopathic Wells’ syndrome in a 75-year-old female, who presented with an exceptionally large size pruritic, erythematous and annular plaque on her abdomen extending to her flanks. Despite the large size of the lesion _not previously reported in other literature_ there was neither associated systemic involvement nor triggering factors. Furthermore, considering the outstanding large size of the lesion we performed a complete malignancy work up for the patient which reveals negative results.
The most confusing clinical differential diagnosis of wells syndrome is acute infectious cellulitis. Other clinical differential diagnosis includes erythema chronicum migrans, arthropod bites, hypereosinophilc syndrome, granoloma annular and chronic idiopathic urticarial (3, 8).Although, flame figures are not pathognomonic for wells syndrome and may be detected in other dermatoses such as bullous pemphigoid, tinea pedis, bite reactions, severe prurigo, eczema and follicular mucinoses (3, 10). We regarded this case as a case of eosinophilic cellulitis because its additional histopathologic features together with the clinical presentation as well as peripheral blood eosinophilia was more consistent with wells syndrome rather than other disease in the differential diagnosis of flame figure.
Treatment options include topical corticosteroids, antihistamines, anti-microbial agents, dapsone, griseofulvin, colchicine, minocycline, nicotinamide ,cyclosporine ,azathioprine, interferon alpha, and ultraviolet light and systemic corticosteroids_ which usually considered as the first-line treatment in Wells’ syndrome_ but many cases resolve spontaneously without therapy (5,8) as what actually happened in our case who didn’t response to topical and systemic corticosteroid while stating disappearance of the lesions unexpectedly after a period of three weeks following discontinuation of prednisolone.To our knowledge, this is the first case of wells syndrome with such a large size of single lesion (80m*35cm) extending from abdomen to flanks bilaterally. Interestingly despite the large size we didn’t find any associated systemic symptom or underlying disease, and surprisingly it resolved spontaneously without any additional treatment. References:1. Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp DermatolSoc 1971; 57: 46-56.
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Erythematous, well-defined, annular indurated plaque affecting abdomen and flank skin approximately 80cm* 35 cm in size.2. Patchy infiltration of lymphocytes and eosinophils extending from superficial to deep dermis.(H *40)3.
There was also mild vasculopathy and scattered flame figures, which represented eosinophilic granules encrusted on dermal collagen. (H *100) 1