Eosinophilic Annular Erythema Research Articles

Neutrophilic and eosinophilic dermatoses associated with hematological malignancy.

Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the knowledge on individual neoplasms. Indeed, a growing body of evidence supports the concept of recurrent molecular defects associating with specific clinical features, as best exemplified by VEXAS. Herein neutrophilic and eosinophilic dermatoses of potential interest for both hematologists and dermatologists will be reviewed, including subcorneal pustular dermatosis-type IgA pemphigus, neutrophilic eccrine hidradenitis, Sweet's syndrome as well as myelodysplasia cutis and VEXAS, pyoderma gangrenosum, eosinophilic annular erythema, eosinophilic dermatosis of hematological malignancy, Wells syndrome and cutaneous involvement in hypereosinophilic syndromes. Possible management approaches are discussed for each, emphasizing scenarios that require treatment of the underlying condition to achieve remission at the skin level.

Diagnostic difficulties in pediatric annular dermatoses.

The polymorphic presentation of annular dermatoses in the pediatric population renders them adiagnostic challenge to the clinician. They include various distinct disease entities that can be vaguely categorized according to the age of onset. Herein, we report on ayoung girl with clinical characteristics of Wells' syndrome, while histological findings favored the diagnosis of annular erythema of infancy (AEI). Although morphological and histological similarities do exist, AEI and eosinophilic annular erythema (EAE) of childhood are considered as distinct entities in the literature. Wells' syndrome (WS) is an eosinophilic dermatosis and histologically characterized by eosinophilic dermal infiltration with the hallmark feature of "flame figures." Based on this case, we discuss and review the differential diagnoses of annular dermatoses in children.

Figurate annulare erythemas.

T. Colcott Fox (1849-1916) first introduced in 1889 the term "figurate erythemas." According to the clinical pattern, figurate erythemas are annular, circinate, concentric, polycyclic, or arciform. The most important figurate annulare erythemas are erythema annulare centrifugum, erythema marginatum, erythema gyratum repens, erythema migrans, erythema chronicum migrans, and the pediatric annular erythemas. Erythema annulare centrifugum might be due to fungal, bacterial, or viral infections or drugs. It tends to spread centrifugally while developing central clearing. The most common locations are the trunk and the proximal extremities. Individual lesions last from several days to weeks and may resolve spontaneously. Erythema marginatum is one of the criteria for the diagnosis of acute rheumatic fever, but it also might be seen as a symptom of other diseases such as hereditary angioedema with C1-inhibitor deficiency and psittacosis. The typical clinical picture is presented by serpiginous erythematous macules and plaques with central clearing and accentuated borders. Erythema gyratum repens is a figurate erythema associated with internal malignancy. It has been linked especially to lung, esophageal, and breast cancers. Erythema gyratum repens is characterized by multiple erythematous, rounded macules or papules, rapidly progressing and forming concentric bands with an unique wood-grained appearance with desquamation on the edges of the erythema. Erythema chronicum migrans is the most common sign of infection with Borrelia burgdorferi and other Borrelia species. It is characterized by a round or oval erythematous or livid macule with a central depressed or raised area on the spot of a previous tick bite. Erythema migrans grows centrifugally and slowly in a matter of days or weeks. Central clearing is observed in 60% of patients, thus forming a targetoid appearance of the lesion. Many other figurate erythemas can be observed in infancy (pediatric annular erythemas). To this group belong neonatal lupus, erythema gyratum atrophicans transiens neonatale, annular centrifugal erythema, familial annular erythema, annular erythema of infancy, eosinophilic annular erythema, and figurate neutrophilic erythema of infancy. The treatment of the various types of figurate erythemas should be etiologic, and when the underlying condition is addressed, the therapy usually is successful.

Eosinophilic Annular Erythema as a Subset of Wells Syndrome or a Distinct Entity: Report of Two Cases

Eosinophilic annular erythema (EAE) is an uncommon acute eosinophilic dermatosis of unknown etiology. Urticarial papules and plaques as pruritic lesions appear in annular and arcuate forms on the trunk and proximal limbs and rarely affect the face. In histopathological diagnosis, EAE has typical features, including acute dermal inflammatory infiltrate with abundant eosinophils and lymphocytes around arteries. In spite of no clear treatment, the findings indicated the positive effects of systemic corticosteroid, hydroxychloroquine, and Ultraviolet B therapy along with recurrences. Two patients have been elected in the present study. The first patient was a middle-aged diabetic female with urticarial papules and plaques around the pelvis and proximal thighs that had active borders with improved centers. We started with 400 mg of hydroxychloroquine and 25 mg of prednisone for the patient whose lesions were subsided during the first week. The second consulted patient was a 41-year-old female without a history of previous illness with four months of painful, pruritic, and violaceous plaques on the back of the trunk. In histopathology, dermal inflammatory infiltrate with abundant eosinophils was observed in the dermis. Corticosteroids and hydroxychloroquine were prescribed with the diagnosis of EAE for this patient as well.

Eosinophilic annular erythema successfully treated with cyclosporine.

Eosinophilic annular erythema successfully treated with cyclosporine.

Vesiculobullous eosinophilic annular erythema: A case report

Eosinophilic annular erythema is a rare eosinophilic dermatosis, characterized by arcuate erythematous urticarial plaques of unclear etiology. Vesiculobullous forms are even rarer, with only few cases described in the English literature. We report a case of vesiculobullous eosinophilic annular erythema with extensive cutaneous involvement poorly responsive to prednisone but showing complete remission with dapsone.

Eosinophilic Annular Erythema of Childhood: A Rare Case

Eosinophilic annular erythema (EAE) of childhood is a rare and recurrent skin condition, with only a few cases described in the literature. The etiology of EAE in children remains unclear. Clinical presentation shows a persistent, non-pruritic, urticarial annular lesions that enlarge in a centrifugal pattern. Biopsy is required for diagnosis which characteristically shows a perivascular eosinophilic infiltrate concentrated in the dermis. Although there is considerable overlap between EAE and Annular Erythema of Infancy (AEI), some proposed differences include age of onset, duration of lesions, presence of mucin deposition of histopathology, and time to resolution. This case report describes a rare case of EAE of childhood in a newborn with associated neonatal eosinophilic pustulosis and laboratory abnormalities.

Uncommon non-infectious annular dermatoses

Several cutaneous diseases can present with annular lesions, making a distinction by physical appearance alone challenging. They can be distinguished into infectious and non-infectious, and common and uncommon annular dermatoses. Common non-infectious diseases include granuloma annulare, urticaria, and subacute lupus erythematosus. In addition, there are rare non-infectious non-neoplastic annular dermatoses whose nosographic attribution is established, including annually recurring erythema annulare centrifugum (EAC) and annular erythema in Sjögren syndrome and others whose nosographic positioning is still debated. They are neutrophilic figurate erythema, palpable migratory arciform erythema, eosinophilic annular erythema, and annular lichenoid dermatitis of youth. Their etiopathogenesis is largely unknown, although immune-mediated mechanisms are likely involved. It is difficult to establish if they are variants of reaction patterns or separate clinic-pathological entities. In fact, EAC and annually recurring EAC may represent different aspects of the same disease. Palpable migratory arciform erythema is hardly distinguishable from EAC deep type, Jessner-Kanof disease, and lupus tumidus. Neutrophilic figurate erythema and eosinophilic figurate erythema are clinically very similar and differing only in the relative proportion of eosinophils and neutrophils.

Treatment of Eosinophilic Annular Erythema: Retrospective multicenter study and literature review

BackgroundEosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse. MethodsThe aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE. To this end, we conducted a retrospective multicenter study and a systematic review of the MEDLINE database. ResultsWe included 18 patients with EAE followed in 8 centers. The MEDLINE database search yielded 37 relevant publications reporting 55 cases of EAE with 106 treatment sequences. The most common and efficient treatments included topical or systemic corticosteroids, hydroxychloroquine and dapsone. In refractory patients, a combination of systemic corticosteroids with hydroxychloroquine was associated with 88% of complete clinical response. DiscussionTo improve the management of EAE patients, we discuss the following treatment strategy: in topical steroid-resistant patients, hydroxychloroquine can be given as first-line systemic treatment. Dapsone, hydroxychloroquine or systemic corticosteroids are second-line options to consider. Last, monoclonal antibodies or JAK inhibitors targeting type 2 inflammation could represent promising last-resort options in refractory patients.

Eosinophilic annular erythema associated with eosinophilic pneumonia and asthma

Eosinophilic annular erythema associated with eosinophilic pneumonia and asthma

Eosinophilic annular erythema in childhood: A distinct entity

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.

Mepolizumab Therapy for Recalcitrant Eosinophilic Annular Erythema in an Adult: A Case Report and Review of Treatment Options.

Eosinophilic annular erythema (EAE) is a rare condition with a chronic relapsing and remitting course, characterized by the presence of annular or polycyclic erythematous and plaque lesions and prominent tissue eosinophilia on histopathology. There is an ongoing discussion on whether EAE is a subset of Wells syndrome (eosinophilic cellulitis) or a separate entity. To date, few cases of EAE have been reported in the literature; of these, about 40 cases were in adults and fewer than ten cases were in children. Given the rarity of this condition, there are no clear recommendations for its management. Systemic corticosteroids and antimalarials are the most commonly used medications used to treat EAE, but many cases have been reported in the literature that are resistant to treatment with these medications. Here, we present a 65-year-old female with EAE refractory to numerous systemic therapies (corticosteroids, hydroxychloroquine, dapsone, doxycycline, methotrexate) who showed a good response to mepolizumab, a humanized monoclonal antibody that blocks interleukin-5. To the best of our knowledge, this is the first reported case of mepolizumab therapy in a patient with EAE. We also review other treatment strategies that have been used to manage this condition to date. Targeting cytokines crucial for the functioning of eosinophils may be a novel direction in the management of EAE, but prospective, double-blinded and placebo-controlled studies are needed to provide further evidence.

Eosinophilic Annular Erythema- Successful Treatment with Dupilumab

Eosinophilic annular erythema (EAE) is a rare eosinophilic dermatosis with few documented cases in the literature. It is considered either a distinct entity or subtype of Well’s syndrome (eosinophilic cellulitis), but EAE, in contrast to Well’s syndrome, is characterized by a chronic course and resistance to treatment. Therapies with reported efficacy include anti-malarial medications, suplatast tosilate, and dapsone. We report the first adult case of EAE to respond to dupilumab, an IL-4 receptor antagonist, which targets the type 2 inflammatory response associated with tissue eosinophilia.

Eosinophilic annular erythema in an adolescent treated with dupilumab

Eosinophilic annular erythema in an adolescent treated with dupilumab

A clinicopathologic study of ten cases of eosinophilic annular erythema

A clinicopathologic study of ten cases of eosinophilic annular erythema

Dapsone for Recalcitrant Eosinophilic Annular Erythema: A Case Report and Literature Review.

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.

Eosinophilic Annular Erythema: A Rare Dermatologic Manifestation of Autoimmune Hepatitis

Autoimmune hepatitis (AIH) is defined as an immune regulated chronic inflammation of the liver. Several extrahepatic cutaneous disorders associated with AIH have been described. The most common are a maculopapular rash, psoriasis,vitiligo, impetigo, lichen planus, erythema nodosum, pyoderma gangrenosum, and vasculitis. Eosinophilic annular erythema (EAE) is a figurate erythema which has been associated with several chronic conditions and malignancies. We report the first case of EAE developing in association with AIH. A 22-year-old male with history of acne was referred for evaluation liver enzymes. Six months prior to his initial visit, he developed a pruritic eruption of demarcated plaques on his scalp, face, neck, and extremities for which he saw dermatology. At that time his liver associated tests were normal. Labs were remarkable for eosinophilia (13%) and biopsies of the lesions showed superficial to mild perivascular lymphohistiocytic cell infiltrate with scattered eosinophils in the dermis. He was diagnosed with eosinophilic annular erythema. A trial of hydroxychloroquine was ineffective. Methotrexate caused leukocytosis and worsening eosinophilia, resulting in discontinuation after 2 weeks of therapy. While on methotrexate his liver tests remained normal. Over the next 10 weeks he was treated with mycophenolate mofetil. However, the AST, ALT, and alkaline phosphatase increased to 107 IU/L, 91 IU/L, and 199 IU/L, respectively. Despite moderate improvement in his skin, discontinuation of mycophenolate mofetil was advised and he was referred for evaluation of abnormal liver enzymes. Subsequent evaluation revealed a positive anti-smooth muscle antibody (65 units) elevation of serum IgG (2037 mg/dL) and a liver biopsy showing chronic nonspecific hepatitis with mild interface hepatitis. A diagnosis of autoimmune hepatitis was made based on labs and histology. The patient was started on high dose prednisone with a decrease in liver enzymes. Imuran was added with tapering of the prednisone resulting in both resolution of his rash and normalization of the liver tests (AST 22 IU/L, ALT 23 IU/L, alkaline phosphatase 79 IU/L) Many cutaneous manifestations of AIH have been described. To our knowledge, this is a unique case of AIH associated with EAE. Furthermore, the correlation between adequate treatment of the patient's AIH and resolution of his rash suggests the importance for awareness of EAE as a presenting sign for AIH.

Eritema anular eosinofílico en un adulto Eosinophilic anular erythema in an adult

Eosinophilic annular erythema (EAE) is an uncommon eosinophilic dermatosis. Clinically it is characterized by recurrent episodes of annular or figurative plaques. The histopathological study shows a perivascular inflammatory infiltrate in the superficial and deep dermis, composed of lymphocytes and eosinophils. It was originally described in children. We report an adult woman who presented with recurrent erythematous annular plaques on the trunk and extremities. A biopsy showed a mainly perivascular lymphocytic infiltrate with numerous eosinophils in the dermis. Laboratory examinations revealed subclinical hypothyroidism. The lesions resolved with topical corticosteroid spontaneously after 3 months.

Eosinophilic Annular Erythema Localized to the Palms and the Soles.

Eosinophilic Annular Erythema Localized to the Palms and the Soles.

Spontaneously resolving eosinophilic annular erythema

To the Editor: Eosinophilic annular erythema is a rare and relatively newly described condition, initially reported in children.1 To our knowledge, there have been only 4 adult cases published in the medical literature.2-5