Recurrent Granulomatous Dermatitis Research Articles

Wells Syndrome in Children: Case Study and Differential Diagnostics

Background . Wells syndrome (eosinophilic cellulitis) is recurrent granulomatous dermatitis with peripheral blood eosinophilia. This is extremely rare pathology, therefore, there are no reliable epidemiological data on its prevalence. Only about 200 cases were recorded worldwide and 30 of them among children according to the meta-analysis (2012). The disease is mostly sporadic, there are rare family cases, according to the results of little number of scientific publications. Clinical Case Description . The clinical case of Wells syndrome in female 4 years old patient is presented. Clinical findings included symmetrical skin lesions, nodes and large irregular edematous plaques of red-purple color with clear fluid vesicles on its surface. The disease had wavy course: rashes have recovered spontaneously over 7–10 days, new elements appeared alongside with feeling unwell, fever up to 37,8°C and abdominal pain. Similar clinical findings of rashes were observed in paternal relatives of the child. Conclusion . Differential diagnostics of Wells syndrome should be carried out with skin granulomatous diseases and hypereosinophilic syndrome that may be characterized by similar clinical findings. Verification of Wells syndrome diagnosis is complicated due to its rareness, low awareness of dermatologists and pediatricians about this pathology, as well as ignoring the need to carry out histological tests during the disease exacerbation.

Steroid Dependent Recalcitrant Well's Syndrome in a Patient with Chronic Lymphocytic Leukemia (CLL) Responding to Hydroxychloroquine (HCQS)

IntroductionWell's syndrome is an uncommon skin condition with cosmetically unpleasant outcomes. It was initially described by Dr. GC wells in 1971 as recurrent granulomatous dermatitis with eosinophilia(1).CaseA 69-years-old woman with well controlled CLL presented with pruritic, papular eruptions & ulcerations over extremities and back (Figure 1). She denied asthma or atopy, insect bite, or new medications. Skin biopsy (Figure 2) revealed perivascular & interstitial infiltrate composed of lymphocytes, histiocytes, neutrophils and eosinophils.Antibiotics, antihistamines, dapsone, steroid injection failed to control her symptoms. She responded to oral prednisone. On attempted steroid taper she experienced disease flares. She was transitioned eventually to HCQS. Prednisone was discontinued. She remained in remission on HCQS.DiscussionWell's syndrome is often described as exaggerated response to insect bite; it may occur without an inciting event, commonly involves extremities, may precede or follow the diagnosis of CLL and has no association with disease course (2). The lesions range from itchy papules, plaques to edematous vesiculo-bullous lesions and panniculitis or ulcerations (3) and can result in disfiguring skin atrophy (4). Pathology reveals eosinophilic or lymphohistiocytic infiltrates (5). Use of glucocorticoids, antihistamines, immunoglobulins, dapsone, ultra violet-B therapy for treatment has been described (2, 5) and may require prolonged treatment (6). Multiple recurrences are often seen in recalcitrant cases (7).Well's syndrome should be considered in differential diagnosis of skin lesions in patients with CLL. HCQS offers an alternative to long term steroid use in its management.

Successful Treatment of Steroid-Dependent Eosinophilic Cellulitis With Cyclosporine

Eosinophilic cellulitis (EC) is a rare idiopathic disorder, first described as a "recurrent granulomatous dermatitis with eosinophilia", that mimics cellulitis of infectious origin. We describe here a previously healthy 11-year-old girl who experienced fever and tender erythematous patch lesions after trauma to her knees. Because of the relapsing cellulitis-like skin lesions, skin biopsies were taken, resulting in a diagnosis of EC. The patient responded well to oral prednisolone but experienced side effects and relapse during dose tapering. She was switched from prednisolone to cyclosporine. Her EC remained under control, and she showed no evidence of relapse after discontinuation of cyclosporine.

Wells' Syndrome Associated with Churg-Strauss Syndrome

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features.

Eosinophilic cellulitis or Wells’ syndrome in a 6-year-old child

Wells’ syndrome or eosinophilic cellulitis is a rare, recurrent inflammatory dermatosis. Wells described this syndrome in 1971 as “recurrent granulomatous dermatitis with eosinophilia” [8], and in 1979, Wells and Smith proposed the term ‘eosinophilic cellulitis’ [9]. The clinical presentation can be variable, but the histopathologic features are characteristic. The etiology is unknown. Most cases occur in adults, and only a few cases have been reported in children. We present a case of eosinophilic cellulitis on both feet in a 6-year-old boy. Two weeks prior to the eruption he had a high fever and complained of pain in his legs. The fever resolved after 5 days, but toe walking was observed. One week thereafter he developed itchy, painful plaques on the back of his feet, starting at the toes and progressing in a few days to above the ankles. Edema and small blisters developed rapidly. His past medical history included cow’s milk allergy and reactive airway disease. There was no history of recent vaccination. The differential diagnosis included cellulitis, erythema nodosum, glove and sock syndrome (parvovirus B19 infection), first dermal presentation of inflammatory arthritis or inflammatory bowel disease, febrile neutrophilic dermatosis, hypereosinophilic syndrome and lymphoproliferative disease. Rest and ibuprofen were prescribed. During the first 2 days, the lesions became more demarcated, indurated and violaceous at their borders, then slow regression began. A complete blood count showed leukocytosis (21.5×10 cells/l) with eosinophilia (4.19×10 cells/l); hemoglobin and platelet count were normal. The erythrocyte’s sedimentation rate was slightly elevated (38 mm/h). Other laboratory investigations included antibody levels (Mycoplasma, Borrelia, Parvovirus B-19, Bartonella henselae, Yersinia), PCR Parvovirus B-19 and detailed immunologic testing, all of which were normal. Stool culture was negative. Ultrasound examination of the ankles revealed subcutaneous swelling, but no signs of arthritis. The diagnosis of eosinophilic cellulitis was established after a skin biopsy was performed. The epidermis was unremarkable. Extending through the full dermal thickness and hypoderm was a dense mixed inflammatory infiltrate that consisted mostly of eosinophils, neutrophils, lymphocytes and histiocytes. Flame figures (Fig. 1) and foci of degenerated, necrotic collagen fibers were diffusely scattered. No signs of vasculitis, granulomas or parasites were noted. Two weeks after discharge there was complete recovery of the skin. After 6 months, there was a short, less severe relapse with edema and erythema on both feet. Diagnosis of Wells’ syndrome is made by the clinical appearance, course and characteristic histopathologic findings. Lesions can begin with itching or tender burning sensations, subsequent cellulitis-like eruption, well-demarcated nodules or plaques and the development of vesicles or bullae. The lesions progress rapidly over 2–3 days and resolve gradually over 2–8 weeks. They pass through a blue-greyish discoloration phase to form atrophic plaques, sometimes with hyperpigmentation, and they heal generS. Van der Straaten . M. Wojciechowski (*) . M. Hagendorens Department of Pediatrics, University Hospital Antwerp, Wilrijkstraat 10, 2650 Edegem, Belgium e-mail: Marek.Wojciechowski@uza.be Tel.: +32-38-213810 Fax: +32-38-291194

Eosinophilic cellulitis (Wells’ syndrome): a new case description

Background Wells’ syndrome was first described by Wells in 1971 as a recurrent granulomatous dermatitis with eosinophilia and was later named eosinophilic cellulitis. It is defined by the following criteria: (i) sudden onset of annular or circinate erythematous-edematous patches that rapidly evolve to morphea-like blue-slate-colored plaques; (ii) a histological picture usually characterized by the presence of ‘flame figures’; (iii) non-constant blood hypereosinophilia.Methods We describe the case of a 49-year-old woman who reported the sudden appearance of a few hard, pasty, oval-shaped, reddish-violet, moderately itchy, erythematous-edematous patches on both arms about 2 months before our observation.Results The remote pathologic history showed that the woman have been submitted to quadrantectomy with lymph node dissection followed by cobalt therapy for breast cancer. Based on the pharmacological history, intake of drugs was excluded. Routine blood chemistry and instrumental tests did not show any alteration, nor did assays of the main autoantibodies, complement, circulating immunocomplexes, or tumor markers.Conclusions Histologically the picture was compatible with the diagnosis of Eosinophilic cellulitis. Following topical corticosteroid therapy the lesion healed rapidly. It relapsed 2 months later, and was again cured with the same topical treatment. No further relapses were observed in a 1 year follow-up.

Eosinophilic cellulitis (Wells' syndrome): a new case description

Wells' syndrome was first described by Wells in 1971 as a recurrent granulomatous dermatitis with eosinophilia and was later named eosinophilic cellulitis. It is defined by the following criteria: (i) sudden onset of annular or circinate erythematous-edematous patches that rapidly evolve to morphea-like blue-slate-colored plaques; (ii) a histological picture usually characterized by the presence of 'flame figures'; (iii) non-constant blood hypereosinophilia. We describe the case of a 49-year-old woman who reported the sudden appearance of a few hard, pasty, oval-shaped, reddish-violet, moderately itchy, erythematous-edematous patches on both arms about 2 months before our observation. The remote pathologic history showed that the woman have been submitted to quadrantectomy with lymph node dissection followed by cobalt therapy for breast cancer. Based on the pharmacological history, intake of drugs was excluded. Routine blood chemistry and instrumental tests did not show any alteration, nor did assays of the main autoantibodies, complement, circulating immunocomplexes, or tumor markers. Histologically the picture was compatible with the diagnosis of Eosinophilic cellulitis. Following topical corticosteroid therapy the lesion healed rapidly. It relapsed 2 months later, and was again cured with the same topical treatment. No further relapses were observed in a 1 year follow-up.

Fiberoptic laryngoscopy as a guide to tracheal extubation in acute epiglottitis

atrophic skin changes? There are no diagnostic laboratory features of eosinophilic fasciitis, although peripheral blood eosinophilia is usually present, and most patients have modest elevations of the serum immunoglobulin concentrations and erythrocyte sedimentation rates. 7-8 Histologically, eosinophilic fasciitis is characterized by inflammation and thickening of the collagen bundles of the deep fascia, with some extension into the muscle and subcutaneous tissue. Eosinophils are rarely prominent in the tissue infiltrate; the major infiltrative cells are lymphocytes and plasma cells. 7~9 However, biopsy during the acute stage of eosinophilic fasciitis may show prominent eosinophilic infiltrate of the dermis and subcutis, 9 thus blurring the histologic distinction between it and eosinophilie eellulitis. Signs and symptoms in our patient would most closely fit the syndrome of eosinophilic cellulitis. Exact categorization of difficult, however, because eosinophilic cellulitis and eosinophilic fasciitis share a number of features in common. Further characterization will await precise definition of the cause(s) of these two conditions. REFERENCES 1. Wells GC: Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 57:46, 1971. 2. Shulman LE: Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: A new syndrome? (abst). J Rheumatol (SuppI) 1:82, 1974. 3. WeIIs GC, Smith NP: Eosinophilic ceIlulitis. Br J Dermatol 100:10t, 1979. 4. Nielsen T, Schmidt H, Sogaard H: Eosinophilic cellulitis (Well's syndrome) in a child. Arch Dermatol 117:427, 1981. 5. Spigel GT, Winkelmann RK: Well's syndrome: Recurrent granulomatous dermatitis with eosinophilia. Arch Dermatol 115:611, 1979. 6. Rosenthal J, Benson MD: Diffuse fasciitis and eosinophilia with symmetric polyarthritis. Ann Intern Med 92:507, 1980. 7. Michet C J, Doyle JA, Ginsburg WW: Eosinophilic fasciitis: Report of 15 cases. Mayo Clin Proc 56:27, I981. 8. Moutsopoulos HM, Webber BL, Pavlidis NA, Fostiropoulos G, Goules D, Shulman LE: Diffuse fasciitis with eosinophilia: A clinicopathologic study. Am J Med 68:701, 1980. 9. Barnes L, Rodman GP, Medsger TA, Short D: Eosinophilic fasciitis: A pathologic study of twenty cases. Am J Pathol 96:493, 1979.

Wells' syndrome: recurrent granulomatous dermatitis with eosinophilia

Wells' syndrome: recurrent granulomatous dermatitis with eosinophilia

Wells' syndrome. Recurrent granulomatous dermatitis with eosinophilia

Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.