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- W2000354585 abstract "A 22-year-old black woman presented with a 3-month history of recurrent, pruritic plaques on her arms and trunk beginning during her seventh month of pregnancy. After a prodrome of pruritus, large, red, indurated plaques would erupt, persist for days, and then clear. She had an otherwise uncomplicated pregnancy with vaginal delivery of a healthy newborn 6 weeks before presentation. During her postnatal course, the eruptions persisted unchanged in intensity or frequency. A review of systems, including gastrointestinal upset, headaches, or recent illnesses, was negative. She denied over the counter or prescribed medications other than prenatal vitamins. Her family history was significant for hypertension and diabetes. The physical examination revealed numerous large, indurated, edematous, erythematous, and violaceous plaques on her bilateral upper arms, back, and torso (Fig 8). Laboratory values were normal, including a complete blood cell count and liver function tests. Two 4-mm punch biopsy specimens of her right arm were obtained (Fig 9, Fig 10).14.What is the most likely diagnosis?a.Fixed drug eruptionb.Tumid lupusc.Wells syndromed.Sweet syndromee.Allergic contact dermatitis15.What is the most likely laboratory abnormality in these patients?a.Leukopeniab.Neutrophiliac.Eosinophiliad.Positive antinuclear antibody titere.Positive antistreptolysin titer16.What is the first-line treatment for this condition?a.Prednisoneb.Potassium iodidec.Hydroxychloroquined.Azathioprinee.Dapsone17.Which of the following upregulates eosinophil function?a.Tumor necrosis factor–alfab.Interleukin-5c.Interleukin-6d.Transforming growth factor–betae.Interleukin-1218.Flame figures may be seen in which of the following conditions?a.Bullous pemphigoidb.Eczemac.Arthropod bitesd.Dermatophyte infectionse.All of the aboveFig 9View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 10View Large Image Figure ViewerDownload Hi-res image Download (PPT) First described in 1971 by Wells, eosinophilic cellulitis, also called Wells syndrome, is a rare inflammatory skin condition of unknown etiology that most commonly presents as pruritic, cellulitic-like plaques. More than 100 cases of Wells syndrome have been reported in the literature in patients ranging from neonates to the elderly. It is often preceded by a prodrome of itching or stinging followed by the development of solitary or multiple lesions that resolve over the course of several weeks. The lesions are often bright red, annular or arcuate plaques or nodules, sometimes with a violaceous border. On occasion, residual atrophy and hyperpigmentation may develop as the lesions fade from red to bright pink to slate-gray. Recurrences are common. Most frequently affected regions include the trunk, arms, face, and neck. Rarely do patients report systemic symptoms of malaise, fevers, or arthralgias. Unusual bullous and urticarial presentations have been reported. The lesions are frequently mistaken for infectious cellulitis; however, skin biopsy specimens reveal an eosinophilic infiltrate. The differential diagnosis includes arthropod bites, urticarial vasculitis, pressure urticaria, and allergic contact dermatitis. The pathogenesis is unknown; however, excess production of interleukin-5, the main cytokine responsible for eosinophilic migration in local TH2 responses, has been shown in several cases. Eosinophilic cellulitis may represent a hypersensitivity reaction to circulating antigens or occur as a result of hypereosinophilia in the blood. Wells initially postulated that lesions start as an urticarial reaction that persists when the patient has an abnormal response to eosinophils. Indeed, the syndrome has been associated with arthropod bites, underlying viral or intestinal infections, and medications such as penicillin G, bleomycin, local anesthetics, and tumor necrosis factor inhibitors. Several reports have also detailed a connection to an underlying cancer. Authors have hypothesized that these infectious, parasitic, neoplastic, and drug triggers may indicate evidence for an allergic hypersensitivity reaction responsible for the clinical and histologic findings. In many reports, however, no inciting event is determined, making it difficult to draw conclusions regarding the etiology of eosinophilic cellulitis. No other cases have described a link of eosinophilic cellulitis to pregnancy; the immune alterations during pregnancy, however, may have been a triggering factor in this case. The laboratory work-up of patients suspected of having Wells syndrome involves a comprehensive metabolic panel, a complete blood cell count, and a punch biopsy specimen of the lesion. When a parasitic disorder is suspected, a stool examination and specific antibody titers are indicated. Laboratory studies in Wells syndrome can reveal a peripheral eosinophilia in 50% of cases. Histology reveals an infiltrate of eosinophils with degranulation and the formation of flame figures, which are not pathognomonic and can also be seen in other eosinophil-mediated dermatoses, such as eczema, bullous pemphigoid, and arthropod bites. Flame figures are formed when degranulating eosinophils coat collagen bundles with eosinophilic major basic protein. Vasculitis is not seen, and direct immunofluorescence studies are usually negative. Wells syndrome must be differentiated from hypereosinophilic syndrome (HES), a multisystem disease that can be fatal if untreated. HES consists of several diagnostic criteria, including peripheral blood eosinophil counts >1500 per μL for a duration of at least 3 months with evidence or organ damage, no evidence of parasitic, allergic, or other causes of eosinophilia, and symptoms and signs of organ system involvement. In HES, patients often present with symptoms of weight loss, malaise, and fever, and show signs of internal organ involvement. Skin findings are seen in more than half of cases of HES ranging from pruritic, erythematous papules, plaques, or nodules to urticaria and angioedema. While skin findings may be similar between the two entities, patients with Wells syndrome should not have the signs of internal organ involvement nor meet the above criteria for HES. The initial treatment of choice for Wells syndrome is oral steroids at doses of 10 to 60 mg daily with slow tapering over several weeks. Recurrences will necessitate retreatment with oral steroids or topical steroids in milder cases. In situations where steroids are contraindicated, other therapy options include antihistamines such as certrizine, oral antibiotics such as minocycline, or antifungals such as griseofulvin. Immunosuppressants such as azathioprine, dapsone, cyclosporine, and tacrolimus have been reported effective in the literature in several patients. Cases can resolve spontaneously, making it difficult to determine the effectiveness of treatments. Though rare, eosinophilic cellulitis is important to keep in the differential of bullous or cellulitic-appearing eruptions that do not respond to antimicrobial therapy and are recurrent in nature. For this series, the recommended choices are: 14, c; 15, c; 16, a; 17, b; 18, e." @default.
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- W2000354585 title "Pruritic, recurrent, erythematous plaques" @default.
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