Wells' syndrome: vesiculobullous presentation and possible role of ectoparasites

A 21-year-old woman with recurrent skin lesions present since December 1992 was seen at the University of Alberta hospitals in 1994. The skin lesions consisted of pruritic, painful, erythematous, “beefy” plaques on the trunk and extremities and also of vesicles, bullae, and pustules on the ankles and hands. She experienced numerous flare-ups of her eruption that required hospital admission on five separate occasions. Each episode was associated with sweats, chills, and dizziness. Headaches, arthralgias, myalgias, and generalized fatigue occurred on occasion. Previous skin biopsies suggested Wells' syndrome. Detailed investigations did not reveal any underlying etiology. The patient was a single woman who had recently moved from New Brunswick to live with her aunt and two cousins in Edmonton and to find employment. She had previously been a healthy young woman until her episodes first began in December 1992. There was a personal history of childhood atopic dermatitis, but she denied any other manifestations of atopy. Her mother had recently passed away at the age of 37 from Hodgkin's lymphoma, otherwise the family history was unremarkable. At the time of this admission she was not taking any medications; however, during the previous months in New Brunswick she had been using numerous medications including prednisone, dapsone, colchicine, and an oral contraceptive for a functional ovarian cyst. Prednisone, up to 60 mg/day, had initially appeared beneficial for her skin lesions, but later her symptoms became resistant. In addition, she suffered from many side-effects induced by chronic corticosteroid usage, inciuding biurred vision, weight gain, striae, and irreguiar menstruai periods. She had not been on corticosteroids for the past 6 weeks, and none of the other medications were of benefit. In fact, she had suffered a severe idiosyncratic reaction to dapsone consisting of high fever, abdominai pain, and abnormal liver parameters. There was no significant travel history and she denied any drug abuse or HIV risk factors, the patient was an animal lover and often played for hours at a time with her aunt's dog and two cats. By coincidence, in New Brunswick she had also owned a dog and two cats. A review of systems was unremarkable. On physical examination, she was a moderately obese young woman who appeared cushingoid, but otherwise well. She was afebrile. Examination of the skin revealed multiple vesicles, bullae, and pustules on her hands and fingers, involving both dorsal and palmar surfaces and extending to her wrists (Fig. 1). Some of the lesions had broken open forming overlying crusts. A few bullae had become hemorrhagic. There was an obvious foul-smelling odor emanating from the lesions. Multiple small excoriations were present bilaterally on the shins and two erythematous, edematous papules were seen on the abdomen. A live flea was found in her suprapubic area. No other fleas were found. The rest of the general examination was unremarkable. Pertinent laboratory investigations revealed an absolute eosinophilia of 2700/mm3 (24% eosinophilia) with a white blood count (WBC) of 11,400/mm”. Swabs of hand lesions were culture-positive for multiple aerobes and anaerobes: 3+ growth of Staphylococcus aureus, 3+ growth of Haemophilus parainfluenza, 1 + growth of Coxiella oxytoca, 2+ growth of Streptococcus viridans, and 4-i- growth of mixed anaerobes. Virologic tests were negative. Examination of a skin biopsy specimen (Fig. 2) revealed multiple flame figures in the dermis with surrounding and interspersed eosinophils. Numerous eosinophils, / lymphocytes, and histiocytes were also present around small blood vessels. There were degenerative changes of the upper epidermis with spongiosis, suggestive of early intraepidermal vesicle formation. Direct immunofluorescence on skin specimens was negative. Skin prick testing with flea antigen was positive for immediate hypersensitivity, but negative for a delayed reaction after 48 h. On further questioning, the patient admitted that she had played with a cat just prior to her admission, which, unbeknownst to her at the time, was flea-infested. Her aunt strongly denied any fleas in the home environment, despite her pets. No other household members complained (5f insect bites or skin symptoms. The patient's skin improved remarkably with hospitalization and treatment with antibiotics; no new lesions developed while in hospital and she was discharged 12 days later. She continued to experience recurrent, sporadic eruptions that resolved without corticosteroids, but was later lost to follow-up when she returned home to New Brunswick.