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Hendra Gunawan, Zulfan Zulfan Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran, Dr. Hasan Sadikin Hospital, Bandung, IndonesiaCorrespondence: Hendra Gunawan, Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Jl. Pasteur 38, Bandung, West Java, 40161, Indonesia, Tel +6281221111215, Email h.gunawan2016@unpad.ac.idAbstract: Wells’ syndrome is a rare inflammatory skin disease characterized by pruritic erythematous lesions and cutaneous edema, often accompanied by eosinophilia. Parasitic infestations, such as toxocariasis and strongyloidiasis, can serve as triggers. However, Wells’ syndrome associated with toxocariasis and strongyloidiasis has not been reported previously in Indonesia. Herein, we present a case of a 27-year-old male with a chief complaint of recurrent, pruritic, and painful erythematous rash on the right lower leg for 6 months, accompanied by fever and diarrhea. Physical examination showed cutaneous edema with erythematous macules and bullae on the affected leg. Peripheral blood eosinophilia was noted, and the histopathological analysis demonstrated flame figures, confirming the diagnosis of Wells’ syndrome. A stool culture identified Strongyloides stercoralis, confirming strongyloidiasis, and serological testing was positive for toxocariasis immunoglobulin G antibodies. The patient was treated with albendazole 400 mg twice daily for 3 weeks resulted in clinical improvement observed by the 14th day. The diverse clinical features of Wells’ syndrome present a challenge to clinicians in making an accurate diagnosis, which typically hinges on histopathological assessment and identifying flame figures. Therefore, clinicopathological correlation is important to establish an accurate diagnosis.Keywords: strongyloidiasis, toxocariasis, Wells’ syndrome

Introduction: Eosinophilic skin diseases encompass a broad spectrum, including Wells syndrome, which is characterized by histological findings of eosinophilic infiltration in the dermis. The etiology and pathogenesis of Wells syndrome are still unknown, but it has been reported that insect bites, infections, and medications can act as triggers. Fungal infections are common in clinical practice; however, when they are recurrent, have atypical characteristics, or are resistant to treatments, they must be recognized as potential triggers for hypersensitivity reactions such as Wells syndrome. Case Presentation: We present the clinical and histopathological characteristics of a case in which Wells syndrome was associated with a fungal infection. Conclusions: Wells syndrome is a rare skin disorder with polymorphic manifestations, making diagnosis challenging in clinical practice. Therefore, a high index of suspicion is required, and blood tests and skin biopsy are necessary to confirm the diagnosis. [ABSTRACT FROM AUTHOR]

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Introduction: Many conditions included under figurate erythemas have similar clinical appearance which makes diagnosis difficult. Among these, eosinophilic annular erythema (EAE) is a rare, uncommon, underreported entity in the pediatric population. Case Report: Herein, we report an 11-year-old boy who presented with asymptomatic recurrent, erythematous-edematous annular plaques with central hyperpigmentation. Discussion: Review of the literature shows scarce reporting of such cases globally and none from India. Clinicopathological findings of our case, suggest that eosinophilic annular erythema is a distinct entity rather than a type of Wells' syndrome.

A 55-year-old woman presented with a 3-year history of recurrent episodes of pruritic cellulitis-like erythematous plaques, mostly located on the limbs. Simultaneously, fever, malaise and peripheral eosinophilia were noted. The clinical diagnosis of eosinophilic cellulitis (also known as Well’s syndrome) was supported by the histopathological finding of typical “flame figures”. Treatment with dapsone was initiated at a dose of 50 mg per day. After one year of follow-up the patient was relapse-free. Eosinophilic cellulitis is an uncommon, recurrent inflammatory skin disease. The management is often a challenge, due to the frequent need for long-term therapy. Dapsone is an effective and safe treatment option.

Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge., (Sociedad Argentina de Pediatría.)

Introduction: Many conditions included under figurate erythemas have similar clinical appearance which makes diagnosis difficult. Among these, eosinophilic annular erythema (EAE) is a rare, uncommon, underreported entity in the pediatric population. Case Report: Herein, we report an 11-year-old boy who presented with asymptomatic recurrent, erythematous-edematous annular plaques with central hyperpigmentation. Discussion: Review of the literature shows scarce reporting of such cases globally and none from India. Clinicopathological findings of our case, suggest that eosinophilic annular erythema is a distinct entity rather than a type of Wells' syndrome. [ABSTRACT FROM AUTHOR]

Wells’ syndrome, also called eosinophilic cellulitis, is a rare eosinophilic dermatosis characterized by an unspecific inflammatory erythematous eruption often associated with systemic symptoms. Here we report the case of a 57-year-old female with bilateral painful pitting and pruritic feet progressive for two weeks despite one week of oral antibiotics. Skin biopsy was performed showing dermal eosinophilic infiltration. The patient showed a spontaneous progressive improvement of the condition. The presented case demonstrates both clinical and histologic presence of lesions of Wells’ syndrome in the course of the disease. A careful diagnostic approach is needed because of the lack of specific signs. The global outcome is favorable and spontaneous resolution is possible.

AIM: In this study, we aimed to review the diagnostic approach to eosinophilic cell renal neoplasms by light microscopy and immunohistochemical techniques. METHOD: In this study 23 of these tumors were eosinophilic variant classic RCC, 15 eosinophilic variant papillary RCC, 13 eosinophilic variant chromophobe RCC and 13 oncocytoma cases. These tumors were immunohistochemically treated with CK7, CD117, EpCAM, Vimentin, RCCm (Renal cell carcinoma marker) and GST-a. RESULTS: In our study, contrary to the general literature on Vimentin, 65.2% negativity was found in our patients with eosinophilic variant classic RCC. However, when compared with other tumor types in our study, vimentin expression was highest in eosinophilic variant classical RCC with 34.8%. Statistically; RCCm, GST-a, EpCAM, CD117, CK7 were found to be significantly associated with tumor types, while no significant relationship was found between Vimentin and tumor types. RCCm positivity and CK7 and CD117 negativity were in favour of eosinophilic variant classical RCC, EpCAM, CK7 and CD117 positivity and Vimentin, GST-a and RCCm negativity supported eosinophilic variant chromophobe RCC, CK7 and RCCm positivity and CD117 and GST-a negativity were found in favour of eosinophilic variant papillary RCC. CD117 positivity and Vimentin, CK7 and GST-a negativity were found to support oncocytoma. CONCLUSIONS: The panel with RCCm, GST-a, EpCAM, CD117, CK7 will contribute to the differentiation of eosinophilic cytoplasm renal tumors that cannot be determined by morphological fi ndings and to reach the correct diagnosis (Tab. 3, Fig. 4, Ref. 54). Text in PDF www.elis.sk. [ABSTRACT FROM AUTHOR]

Eosinophilic dermatoses encompass a broad spectrum of diseases of different etiologies hallmarked by eosinophilic infiltration of the skin and/or mucous membranes, with or without associated blood eosinophilia. The wide range of dermatological manifestations of this spectrum, including nodules and plaques, pustules, blisters, ulcers, and urticarial lesions, is reflected in a non-univocal classification system. We identified six groups of eosinophilic dermatoses based on the predominant anatomic level of involvement: (1) epidermal; (2) of the dermal–epidermal junction; (3) dermal; (4) of the hypodermis and muscle fascia; (5) of the pilosebaceous unit; and (6) vascular/perivascular. We review clinicopathologic features and management of diseases belonging to each group, particularly: (1) pemphigus herpetiformis and atopic dermatitis as prototypes of the epidermal group; (2) bullous pemphigoid as prototypic eosinophilic dermatosis of the dermal–epidermal junction; (3) eosinophilic cellulitis (Wells syndrome), hypereosinophilic syndromes, Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome, eosinophilic dermatosis of hematologic malignancy and chronic spontaneous urticaria as paradigmatic dermal eosinophilic dermatoses; (4) eosinophilic fasciitis as an eosinophilic dermatosis with predominant involvement of the hypodermis and muscle fascia; (5) eosinophilic pustular folliculitis as a model of the pilosebaceous unit involvement; and (6) granuloma faciale, angiolymphoid hyperplasia with eosinophilia, and eosinophilic granulomatosis with polyangiitis, belonging to the vascular/perivascular group. [ABSTRACT FROM AUTHOR]

Eosinophilic cellulitis is an uncommon, inflammatory and chronic disorder of unknown etiology. Corticosteroids are currently considered as the first-line treatment but they are not without significant disadvantages such as contraindications in steroid-resistant cases and patients with frequent recurrences. We report a patient suffering from Wells’ syndrome with a 24-year history of symptomatic and generalized skin lesions. After consultation in our department, treatment with colchicine 1 mg/day was prescribed resulting in large clinical improvement. No side effects have been recorded. To our knowledge, this is an original disease approach. Although small, our clinical experience supports the inclusion of colchicine in the drug armamentarium when treating patients suffering from Wells’ syndrome. Indeed, its excellent safety profile makes it very attractive for patients with frequent recurrent episodes who need secure options for the medium- and long-term disease control.

A 5-year-old boy was referred because of urticarial flares with angioedema. Antihistamines and systemic steroids were not useful to prevent flares. At examination, at distance of a flare, chronic red-brownish plaques were found on the legs, trunk, and back, resembling granuloma annulare. The skin biopsy showed epithelioid and giant-cell granulomas, elastophagia, and “flames figures” surrounded with many eosinophils. A diagnosis of disseminated granuloma annulare-like variant of Wells' syndrome as described earlier by Caputo was made. A treatment with dapsone was initiated and enabled to stop flares. Wells' syndrome is a rare clinicopathologic entity with a chronic benign course and often misleading clinical presentation. Our case illustrates the clinical polymorphism and benefit of dapsone therapy.

Le syndrome de Wells secondaire à une piqûre d'abeille est peu fréquent. Nous en rapportons un cas, survenu chez un nourrisson de 18 mois ayant présenté un œdème de la main gauche surmonté de lésions bulleuses après une piqûre d'abeille. Le patient a été traité initialement par des antibiotiques, mais sans amélioration. Un traitement par corticothérapie systémique a été instauré avec une bonne évolution. Le diagnostic de la cellulite de Wells a été retenu devant l'aspect clinique, les données biologiques et histopathologiques. Wells' syndrome caused by a honeybee sting is not common. We report the case of a 18-month-old infant who was stung by a honeybee, and whose left hand showed swelling with bullous lesions. The lesions showed no response to antibiotic treatment, but the lesion gradually resolved with subsequent systemic corticosteroid treatment. The diagnosis of Wells' syndrome was made according to clinical features, biological and histopathological findings. [ABSTRACT FROM AUTHOR]

The article describes the case of a 5-year-old boy who experienced Wells' syndrome associated with Mycoplasma pneumoniae. The patient had history of a pruritic, erythematous and edematous lesion on both ankles and feet. Laboratory investigations led to the diagnosis of M. pneumoniae infection. Gradual clinical improvement was observed after administration of oral clarithromycin and oral prednisolone.

The article discusses the various aspects of the dermatology related periodical "International Journal of Dermatology." It talks about the management of the skin diseases and the different updates it offers to the dermatologists worldwide. It discusses the various topics discussed in the issue including stigmata, dermatitis, and Wells syndrome.

Eosinophilic annular erythema (EAE) is a rare entity of unknown etiology that is possibly related to a hypersensitivity reaction and presents as annular erythematous plaques with tissue eosinophilia. It is classified as a figurate erythema with a controversial relationship to Wells syndrome (WS) in the literature, where it is generally considered a separate entity or subset based on clinical and histopathologic differences. EAE typically presents with recurrent, erythematous, arcuate, and annular plaques on the trunk and proximal extremities. The course of the disease is often chronic, recurrent, and relapsing. Responses to treatment are variable but are typically best with systemic steroids and antimalarials. We report a patient refractory to other therapies who had a striking response to dapsone. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To describe the clinical and histologic features of acute erythroderma in dogs with gastrointestinal disease. DESIGN: Retrospective case series. ANIMALS: 18 dogs with erythroderma and gastrointestinal disease. PROCEDURES: Medical records and biopsy specimens were reviewed. Information collected from medical records included signalment, clinical signs, physical examination and diagnostic test results, treatment, and outcome. The Naranjo algorithm was used to estimate the probability of an adverse drug reaction for each dog. RESULTS: All dogs had an acute onset of erythematous macules or generalized erythroderma. Histologic features of skin biopsy specimens had 3 patterns representing a progressive spectrum of inflammation. Most dogs had vomiting (n = 17) and hematochezia (10). Signs of gastrointestinal disease became evident before, after, or concurrent with the onset of skin lesions in 10, 3, and 5 dogs, respectively. Inflammatory bowel disease, pancreatitis, and adverse food reaction were diagnosed in 5, 3, and 3 dogs, respectively. The cause of the gastrointestinal signs was not identified for 8 dogs. Eight dogs had a Naranjo score consistent with a possible adverse drug reaction. Treatment of skin lesions included drug withdrawal (n = 15), antihistamines (16), and corticosteroids (14). Signs of gastrointestinal disease and skin lesions resolved at a mean of 4.6 days and 20.8 days, respectively, after onset. CONCLUSIONS AND CLINICAL RELEVANCE: Results indicated acute erythroderma may be associated with > 1 gastrointestinal disease or an adverse drug reaction in some dogs. Recognition of the clinical and histologic features of this syndrome is essential for accurate diagnosis. [ABSTRACT FROM AUTHOR]

Eosinophilic cellulitis is an uncommon, inflammatory and chronic disorder of unknown etiology. Corticosteroids are currently considered as the first-line treatment but they are not without significant disadvantages such as contraindications in steroid-resistant cases and patients with frequent recurrences. We report a patient suffering from Wells' syndrome with a 24-year history of symptomatic and generalized skin lesions. After consultation in our department, treatment with colchicine 1 mg/day was prescribed resulting in large clinical improvement. No side effects have been recorded. To our knowledge, this is an original disease approach. Although small, our clinical experience supports the inclusion of colchicine in the drug armamentarium when treating patients suffering from Wells' syndrome. Indeed, its excellent safety profile makes it very attractive for patients with frequent recurrent episodes who need secure options for the medium- and long-term disease control. [ABSTRACT FROM AUTHOR]

Mott cells are a variant form of plasma cells in humans and laboratory animals. This report describes the morphological characteristics of Mott cells observed in a 33-week-old female CB6F1-Tg rasH2 mouse. Microscopically, a large number of round cells with abundant eosinophilic globules, which were variable in size, were observed in the spleen and were densely distributed in the red pulp adjacent to the marginal zone. A few similar cells were present in the submandibular lymph node and bone marrow. Neither systemic nor local chronic inflammatory changes were seen in this animal. These cells were positive for mouse immunoglobulins. Ultrastructurally, the dilated rough endoplasmic reticulum had a homogenous substances with an intermediate electron density. On the basis of the above findings, these cells were identified as Mott cells. The present lesion is thought to be a spontaneous lesion, an unusual appearance of Mott cells without any associated pathological conditions. [ABSTRACT FROM AUTHOR]

Eosinophilic cellulitis (Wells syndrome) is a rare inflammatory skin disease defined by erythematous, tender, sometimes urticarial plaques, possibly with vesicles and bullae, and granulomatous eosinophilic infiltrates in the dermis. Usually the disease has a benign course with spontaneous remission within a few weeks. Nevertheless, recurrences are quite frequent and may occur for several years. The objective of this study was to review the so far reported treatment options for Wells syndrome in a systematic manner. This systematic review is based on a search on Medline, Embase and Cochrane Central Register for English and German articles from 1970 to 2015. Advices on the treatment of Wells syndrome are limited predominately to case reports or to small case series. There are no randomized controlled trials, and control groups are missing. A variety of treatment options for Wells syndrome were reported including topical and systemic corticosteroids, antihistamines, cyclosporine, dapsone, azathioprine, griseofulvin, doxycycline, minocycline, antimalarial medications, oral tacrolimus/topical tacrolimus, sulfasalazine, interferon alpha and gamma, TNF alpha inhibitors, colchicine and PUVA therapy. As well-designed, randomized controlled trials are missing, no guidelines for the treatment of this disease can be given. Due to the small number of patients and the frequent misdiagnosis of this clinical entity, the aim of this systematic overview is to call attention to this rare condition and to help clinicians to diagnose and treat Wells syndrome effectively. Due to the good prognosis and tendency to resolve, systemic treatment should be limited to cases resistant to local therapy or with widespread lesions. [ABSTRACT FROM AUTHOR]

Mosquito bites are skin irritating reactions, which usually resolve spontaneously without intensive medical care. However, in certain situations, mosquito bites may form a more vicious reaction, sometimes accompanying fever and systemic symptoms. In such cases, the presence of rare hematological disorders, abnormalities in eosinophils and/or association with Epstein-Barr virus (EBV) may underlie. Importantly, hypersensitivity to mosquito bites (HMB), which is characterized by necrotic skin reactions to mosquito bites with various systemic symptoms, is often observed in association with EBV infection and natural killer (NK) cell lymphoproliferative disorder. Exaggerated skin reaction to mosquito bites is also seen in Wells’ syndrome. While strong Th2-skewing immune dysregulation is apparent in the patients, they also show robust CD4 + T cell proliferation in response to mosquito salivary gland extracts, indicating close association between Wells’ syndrome and mosquito bites. Similar skin reaction to mosquito bites is also noticed in certain types of B cell neoplasm, although the role of B cells in this peculiar reaction to mosquito bites is yet to be elucidated. In this review, we will discuss the current knowledge of exaggerated reaction toward mosquito bites seen in conjunction with these unique hematological disorders, and examine the scientific studies and observations reported in previous literatures to organize our current understanding of the pathogenesis of this distinct disorder. [ABSTRACT FROM AUTHOR]

Abstract: A 4‐year‐old boy presented with erythematous vesicular plaques, ulceration, edema, and pruritus on the left foot and ankle 10 days after receiving the tetanus, diphtheria, pertussis, and polio; measles, mumps, rubella, and varicella; and hepatitis A/B vaccines. Biopsy showed eosinophilic infiltrates and flame figures, suggesting Wells syndrome. Patch testing showed a 1+ reaction to neomycin and aluminum hydroxide, with a recall reaction of Wells syndrome of the feet bilaterally. We report a rare case of pediatric Wells syndrome triggered by nonthimerosal vaccine components confirmed by patch testing. [ABSTRACT FROM AUTHOR]

Wells syndrome, part of eosinophilic dermatosis, is characterized by infiltrated plaques with edema and erythema, which resolve without scarring and are accompanied by peripheral blood eosinophilia. Familial cases have been described.We report a case of this group of rare dermatosis, pathological studies are essential for diagnosing as clinically encountered numerous differential diagnoses. [ABSTRACT FROM AUTHOR]

Canine urolithiasis is a common disorder of the urinary tract, characterized by stones located anywhere within the urinary tract, which is mostly encountered in middle-aged to older dogs. Urolithiasis is influenced by familial, congenital and pathophysiological factors including urinary pH, dehydration, urinary infection, anatomical abnormalities and drug administration. A 3 weeks old mixed German Shepherd male puppy with no antecedent clinical signs (sudden death) was submitted to the Pathology Department for necropsy. The animal was suspected of bronchopneumonia ab ingestis due to milk aspiration. Necropsy, cytological and histological exams were performed. Grossly, a large amount of urine was found within the peritoneal cavity (uroperitoneum) secondary to urinary bladder rupture, severe bilateral hydronephrosis and hydroureter, and urethral obstruction with numerous large white to gray calculi varying in size from 2-10mm were identified. The cytological exam showed several large, white to yellow spheroids with radial concentric laminations consistent with leucine crystals. Histologically, the renal tubules were diffusely dilated and contained pale eosinophilic hyaline casts, sloughed necrotic epithelial cells and lamellated concretions of amphophilic radiating structures. A diagnosis of urethral obstruction due to leucine urolithiasis was made, and it was associated with hydronephrosis, hydroureter and urinary bladder rupture. To the best of the authors' knowledge this is the first report of leucine urolithiasis in a dog in Romania. [ABSTRACT FROM AUTHOR]

Eosinophilic/T-cell chorionic vasculitis (ETCV) is an inflammatory lesion of placental fetal vessels. In contrast to acute chorionic vasculitis, inflammation in ETCV is seen in chorionic vessel walls opposite the amnionic surface. It is not known whether inflammation in ETCV consists of maternal cells from the intervillous space or fetal cells migrating from the vessel. We used fluorescent in situ hybridization (FISH) to differentiate fetal versus maternal cells in ETCV. Placentas with ETCV, previously identified for a published study, were used. Infant sex in each case was identified using the electronic medical record. For male infants, 3-pm sections were cut from archived tissue blocks from placentas involving ETCV and stained with fluorescent X- and Y-chromosome centromeric probes. A consecutive hematoxylin/eosinstained section was used for correlation. FISH analysis was performed on 400 interphase nuclei at the site of ETCV to determine the proportion of XX, XY, X, and Y cells. Of 31 ETCV cases, 20 were female and 10 were male (1 sex not recorded). Six of 10 cases with male infants had recuts with visible ETCV. In these 6 cases the average percentages (ranges) of XY cells, X-only cells, and Y-only cells in the region of inflammation were 81 (70-90), 11 (6-17), and 8 (2-14), respectively. There was a 2:1 female:male infant ratio in ETCV. Similar to acute chorionic vasculitis, the inflammation in ETCV is of fetal origin. It is still unknown, however, whether the stimulus for ETCV is of fetal or maternal origin. [ABSTRACT FROM AUTHOR]

Wells' syndrome (WS), or eosinophilic cellulitis, is an uncommon inflammatory dermatosis of unknown etiology that typically presents with pruritic cellulitis-like plaques on the extremities. Therefore, WS is often misdiagnosed as bacterial cellulitis due to its similarity in presentation. Here, we report two cases of WS that masqueraded as bacterial facial cellulitis. Under treatment with oral prednisone and/or a combination therapy with levocetirizine and hydroxyzine, both patients showed a dramatic improvement of the skin lesions. These cases highlight the need for clinicians to consider WS in the differential diagnosis when evaluating a patient with facial cellulitis that does not respond to an initial antimicrobial regimen. In addition, our cases suggest that combination therapy with levocetirizine and hydroxyzine may be successfully used as corticosteroid-sparing treatment or to prevent relapse after the discontinuation of corticosteroid treatment. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Stings by honeybees are not uncommon and most cases cause pain but no significant medical problems. Some patients, however, have lethal complications such as acute anaphylactic shock. Cellulitis caused by honeybee sting is very rare and can be a late complication in some patients. We report a 45-year-old female patient who was stung by a honeybee, and whose right forearm showed progressive swelling with bullous formation after the sting. She was sent to our emergency department with the diagnosis of right hand cellulitis. After treatment with antibiotics for 5 days, the lesions showed no response. Then, systemic steroid was used and the lesion gradually resolved. Diagnosis of Wells' syndrome was made according to clinical appearance, course and characteristic histopathological findings.

A 5-year-old boy was referred because of urticarial flares with angioedema. Antihistamines and systemic steroids were not useful to prevent flares. At examination, at distance of a flare, chronic red-brownish plaques were found on the legs, trunk, and back, resembling granuloma annulare. The skin biopsy showed epithelioid and giant-cell granulomas, elastophagia, and "flames figures" surrounded with many eosinophils. A diagnosis of disseminated granuloma annulare-like variant of Wells' syndrome as described earlier by Caputo was made. A treatment with dapsone was initiated and enabled to stop flares. Wells' syndrome is a rare clinicopathologic entity with a chronic benign course and often misleading clinical presentation. Our case illustrates the clinical polymorphism and benefit of dapsone therapy. [ABSTRACT FROM AUTHOR]

Background We report a case of a 33-year-old man who presented with immunoglobulin (Ig)G4-related disease (IgG4-RD) forming a pseudotumor in the left paratesticular region during oral administration of corticosteroid for Wells syndrome, which involves cellulitis with eosinophilia. Case presentation The patient was introduced to our institution from a private hospital with a 3-month history of asymptomatic left scrotal mass. A 5-cm diameter nodule was palpable in the left scrotum. Tumor lesion in the left paratestis involving the epididymis and spermatic cord was observed on computed tomography and magnetic resonance imaging. Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels. Urine examination likewise revealed no significant findings. Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor. The tumor was pathologically identified as IgG4-RD of the left paratestis involving the epididymis and spermatic cord. Conclusions We present a first description of IgG4-RD in a patient with Wells syndrome and the ninth case of IgG4-RD in a scrotal organ, and discuss this very rare entity with reference to the literature. Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_225 [ABSTRACT FROM AUTHOR]

Background: Viliuisk encephalomyelitis (VE) is an endemic neurological disease in Northeast Siberia and generally considered to be a chronic encephalomyelitis of unknown origin actually spreading in the Sakha (Yakutian) Republic. Methodology and Principle Findings: In search for the pathophysiology and causative agent of VE, we performed a cross-sectional study on clinical, serological and neuroimaging data on chronic VE patients during two medical expeditions to three villages within the Viliuiski river basin in the Republic of Sakha in 2000 and to the capital Yakutsk in 2006. The severity of the core clinical picture with predominant sensory ataxia, gait apraxia, lower limb spasticity, cognitive impairment and bladder dysfunction correlated with the degree of MRI findings showing enlargement of inner ventricular spaces as in communicating hydrocephalus. Laboratory studies revealed transient eosinophilia during the preceding acute meningitis-like phase, but no ongoing inflammatory process in the CSF. We found immune reactions against Toxocara canis in the majority of chronic VE patients but rarely in controls (P = 0.025; Fisher's exact test). Histological analysis of subacute to subchronic VE brain samples showed eosinophilic infiltrations with no signs of persistent Toxocara canis infection. Conclusions and Significance: Our data showed that pressure by the communicating hydrocephalus as a mechanical factor is the major pathogenic mechanism in chronic VE, most likely triggered by eosinophilic meningitis. There are no signs for an ongoing inflammatory process in chronic VE. The past eosinophilic reaction in VE might be caused by Toxocara ssp. infection and might therefore represent the first hint for an initial cause leading to the development of chronic VE. Our data provide a framework for future studies and potential therapeutic interventions for this enigmatic epidemic neurological disease potentially spreading in Sakha Republic. [ABSTRACT FROM AUTHOR]

The "Wells syndrome" is a rare inflammatory dermatosis of unknown pathogenesis, clinical polymorph, both the location and the extent of the lesions. This is a skin disease of inflammatory origin, which usually appears in children and adolescents. Has a polymorphic clinical picture, both the location and the extent of the lesions, and for the correct interpretation of diagnostic histopathological lesions required. This entity should be considered in the differential diagnosis of any atypical presentation of cellulite with peripheral eosinophilia, that does not respond to antibiotics. [ABSTRACT FROM AUTHOR]

Background: Wells syndrome is an uncommon inflammatory dermatosis first described in 1971 by Wells. The clinical eruption is characterized by varying morphology and severity and usually follows a relapsing remitting course. The majority of the reported cases are of unknown etiology, drug induced Wells syndrome has rarely been reported. A literature search using MEDLINE was performed. We recorded the features of our case and of the additional cases of drug induced Wells syndrome in the literature. Main observations: Including our case there are 25 cases of drug-induced Wells syndrome reported. Causative drugs include antibiotics, anticholinergic agents, anaesthetics, non-steroidal anti-inflammatory agents, thyroid medications, chemotherapeutic agents, thiomersal containing vaccinations, anti-tumor necrosis factor agents and thiazide diuretics. Conclusions: To the authors knowledge this is the first reported case of drug-induced Wells syndrome from thiazide diuretics. The diagnosis of Wells syndrome is often controversial and we propose a set of diagnostic criteria. [ABSTRACT FROM AUTHOR]

A 10-week old preterm infant boy presented with a pustular eruption of the face and blood eosinophilia. Histology demonstrated eosinophils without predominant follicular involvement. The lesions healed without intervention within 2 months. The presentation was consistent with neonatal eosinophilic pustulosis. We discuss the similarities between cases described in the literature and suggest that this entity affects a unique patient population of preterm infants. [ABSTRACT FROM AUTHOR]

Objective and design: Asthma is associated with eosinophilic airway inflammation and characterized by enhanced airway sensitivity. Interleukin (IL)-5 plays an important role in the pathogenesis of asthma. The involvement of IL-5 receptor-mediated cellular signals in the pathogenesis of a mite antigen-induced chronic asthma model was investigated. Subjects: In this study, 48 female C57BL/6J (WT) mice and IL-5 receptor-deficient (IL-5RKO) mice were used. Treatment: Mite antigen (50 μl) was intranasally administered 13 times to WT and IL-5RKO mice. Methods: Airway hypersensitivity (Mch PC) and specific antigen exposure tests were performed, and lung tissue, bronchoalveolar lavage fluid (BALF), and blood were collected to investigate the asthma pathology and differences in the local pulmonary levels of cytokines and chemokines. Results: Airway sensitivity was enhanced and antigen-specific airway resistance was increased in WT mice. In addition, the number of eosinophils and Th2 cytokine levels in the BALF were increased. In contrast, IL-5RKO mice did not acquire the asthma pathology, such as antigen-specific airway resistance and eosinophilic airway inflammation. Mch PC was significantly correlated with cysteinyl leukotriene levels in WT mice. Conclusion: These findings suggested that both IL-5 induced eosinophils and cysteinyl leukotrienes are involved in the pathology of this mite antigen-induced chronic asthma model. [ABSTRACT FROM AUTHOR]

Background/Objective Eosinophilic annular erythema (EAE) was proposed to describe annular skin lesions associated with tissue eosinophilia, however, its relation to Well's syndrome (WS) remains a source of controversy. We studied a series of patients to increase awareness of this entity and to clarify its relation to WS. Methods A multicentre study in which the clinical records, histological findings, laboratory results, therapeutic responses and follow-up of 10 patients were demonstrated. Results The study included seven women and three men with age ranging from 31 to 54 years. The duration of the disease ranged from 3 to 28 months. All patients showed involvement of trunk and extremities. Early lesions were manifested as erythematous plaques, which progressed to well-developed figurate lesions and ended as large annular lesions with pigmented centre and elevated border. Flame figures were only observed in well-developed and long-standing lesions. Blood eosinophilia was found in all patients with different grades. Associated disorders included chronic gastritis, diabetes mellitus, chronic hepatitis-C virus infection and chronic kidney disease. The disease showed chronic course with high relapse rate and resistance to various therapeutic modalities including systemic steroid alone and in combination with hydroxychloroquine and cyclosporine. Conclusion We believe that EAE is a peculiar clinical variant in the spectrum of WS, which is characterized by a chronic course, resistance to treatment and high relapse rate. The diagnosis and evaluation of this condition need a close monitoring with repeated clinical, histological and laboratory assessment. [ABSTRACT FROM AUTHOR]

Eosinophilic fasciitis is a systemic autoimmune disease that manifests with fibrosing inflammation of the fascia and the surrounding skin, sometimes even muscle tissue is involved. Clinically, the disease manifests as rigid indurations or sclerotization of the skin that may resemble systemic sclerosis. In laboratory tests, elevated levels of ESR, increased C-reactive protein in serum and marked eosinophilia dominate. Autoantibodies are usually negative. Inflammation of the fascia can be best viewed by magnetic resonance imaging, and the diagnosis is confirmed by histological examination. The therapy includes the administration of glucocorticoids. In refractory forms, the combination of glucocorticoids with immunosuppressive agents (methotrexate, etc.) is used. A beneficial effect of infliximab has been described as well. [ABSTRACT FROM AUTHOR]

Wells' syndrome is a rare eosinophilic dermatitis first described 40 years ago, with a wide clinical spectrum and varied histology according to the stage of the cutaneous lesions, associated with numerous endogenous and exogenous factors, including infectious and gastrointestinal diseases. We present the case of a woman with a 10-day history of edematous, itching and recurrent plaques in limbs with pustules in the right cheek, chest, abdomen and hips. Wells' syndrome was diagnosed and the following findings were documented: flame figures in the histological sections, negative immunofluorescence for immune complexes and complement deposits, peripheral blood eosinophilia, and Helicobacter pylori infection confirmed by endoscopy and gastric biopsy. Possible physiopathological mechanisms of a new association with H. pylori are discussed. It may be a coincidence or a possible etiology that should be further investigated. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 47 years old male patient, who was admitted for the evaluation of multiple pruritic erythematous papules on the lower extremities and diagnosed with wells' syndrome with a clinicopathological correlation associated with mantle-cell lymphoma. Topics include clinical examination stated to be unremarkable except for the presence of splenomegaly, and the inflammatory process involved the entire thickness of the dermis as well as the subcutis.

Eosinophilic Cellulitis (Wells Syndrome) is an uncommon inflammatory disease with clinical polymorphism. A thirty-five-year-old female presented with 2 weeks history of gradually progressive and painful erythematous plaque on the right foot. She had already received several types of antibiotic for the cellulitis without improvement. A diagnosis of Wells syndrome was established. Significant improvement was noted after one month of treatment with prednisolone. Awareness of this condition is necessary for early identification and management. [ABSTRACT FROM AUTHOR]

Well's syndrome, in many circumstances, is determined by intensive eosinophilic infiltration into dermal layer with the clinical presentation of erythematous and edematous plaques which can easily be remitted by current corticosteroid therapies. Up to now, different etiologies have been proposed governing Well's pathophysiological procedure. Of note, no evidence of seasonal association has been already reported. A 70-years-old afebrile male presented with a chief complaint of 30-years history of cutaneous manifestations along with non-ulcerated edematous, erythematous plaques and patches, and extensive pruritus confined on both dorsal and palmar surface of upper extremities occurred annually in early summer. We hereby report a case in season-dependent of Well's syndrome occurrence. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Chronic relapsing eosinophilic cellulitis associated, although independent in severity, with chronic lymphocytic leukemia," in the previous issue.

Wells' syndrome (WS) is an uncommon inflammatory skin disease which typically presents single or multiple erythematous and edematous urticarial plaques similar to cellulitis. The lesions may evolve into blue-grey morphea-like lesions and may persist for weeks or months. They ultimately heal without scar. Other clinical presentations reported in literature include papular and nodular and, rarely, bullous eruptions. Previously, bullous Wells' syndrome was rarely reported in the literature. Herein, we describe a case of a female patient with bullous Wells' syndrome localized to the upper limbs without any associated disorders. [ABSTRACT FROM AUTHOR]

Eosinophilic cellulitis (Wells' syndrome) is an uncommon inflammatory disease with clinical polymorphism. It is often associated with infectious, allergic or myeloproliferative diseases; however, the exact aetiology is unknown. This report describes a rare case of eosinophilic cellulitis in association with angioimmunoblastic lymphadenopathy. The typical skin findings of Wells' syndrome disappeared completely following chemotherapy and autologous stem cell transplantation. [ABSTRACT FROM AUTHOR]

A 69-year-old woman with chronic lymphocytic leukaemia presented with an 18-month history of pruritic, tender, erythematous plaques. The recurrent lesions simulated insect bites and cellulitis, but failed to respond to appropriate treatments. A recent severe flare was associated with markedly elevated inflammatory markers and swelling of the left leg. The swelling settled rapidly with ibuprofen, leaving cellulitis around a small ulcer infected with Staphylococcus aureus and Streptococcus pyogenes. The cellulitis responded to oral flucloxacillin. Subsequent multiple small exaggerated insect bite reaction lesions settled with oral prednisolone. Eosinophilic cellulitis (Wells' syndrome) was considered unlikely based on the lack of a morphoea-like resolution phase, histological lack of flame figures and presence of vasculitis; however, the distinction is probably artificial. [ABSTRACT FROM AUTHOR]

Mantle cell lymphoma (MCL) is an aggressive type of malignant lymphoma with short median survival despite conventional therapy. We describe the unusual case of a 66-year-old man with a chronic skin rash which preceded the occurrence of an indolent MCL by 2 years. Repeated skin biopsies did not show involvement by the lymphoma. Short therapy with rituximab resulted in the complete and lasting resolution of the cutaneous lesions. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Well's syndrome is an uncommon condition of unknown etiology, clinically characterized by pruritic or tender cellulitis like eruption and histologically by flame figures in the dermis. We report a case of Well's syndrome in an 18-month-old boy who presented with urticaria like eruptions. [ABSTRACT FROM AUTHOR]