Search

Background Eosinophilic 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. Methods The 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. Results We 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. Discussion To 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.

In neurofibromatosis type 1 (NF-1), a Ras-pathway mutation disorder both celiac disease and juvenile myelomonocytic leukemia (JMML) have been found in association. A 3-year-old male child presented to us with annular erythema and cafe-au-lait macules (>6 in number). On investigation, he was found to have JMML and celiac disease. A child with early manifestation of NF1 in association with JMML and celiac disease is an even rarer presentation.

ZusammenfassungWir berichten über ein 20 Monate altes Mädchen mit teils anulären, urtikariellen Hautveränderungen am gesamten Integument, die bis zu 1 Woche persistierten und dann an neuen Stellen auftraten. Die histologische Aufarbeitung zeigte ein urtikarielles Entzündungsmuster mit interstitiellem Ödem und einem Infiltrat mit Lymphozyten, Neutrophilen und vielen eosinophilen Granulozyten ohne Flammenfiguren. Das Labor war unauffällig. In Zusammenschau stellten wir die Diagnose eines eosinophilen anulären Erythems (EAE) im Kindesalter, das zu den eosinophilen Dermatosen zählt.

Erythema annulare centrifugum (EAC) is a figurate erythema presenting with annular, erythematous plaques with a trailing scale. It is considered a hypersensitivity reaction to a variety of antigens and is associated with multiple underlying causes including malignancy. Malignancy-associated EAC is termed paraneoplastic erythema annulare centrifugum eruption (PEACE). Although a specific etiology has yet to be elucidated, this form of EAC is likely to occur due to cytokine and/or antigen stimulation from an underlying malignancy. PEACE is primarily associated with lymphoproliferative disorders and rarely solid tumors. Our report discusses a case of PEACE associated with metastatic colorectal adenocarcinoma. On presentation to our clinic, the patient had developed a migratory annular eruption over a year. His review of symptoms was positive for signs of underlying malignancy, including weight loss and recent lower vertebral fracture. A biopsy of his annular lesion revealed a non-specific pityriasiform dermatitis. A vertebral biopsy uncovered a diagnosis of metastatic colorectal cancer. At that time, clinicopathologic correlation allowed us to reach the diagnosis of PEACE.

A 4 year old boy presented with annular erythema on his nose. The lesion had begun as a small itchy macule seven days earlier. Initially, discoid eczema was diagnosed and he was treated with hydrocortisone butyrate ointment for three days. Although the pruritus improved, the skin rash became more extensive. The patient had no history of preceding injury or insect bite. The household had a cat. On physical examination, an annular erythematous lesion (1 cm ×1 cm) without scaling was observed on the boy’s nose (fig 1). Fig 1 1 cm × 1 cm infiltrated annular erythema without scaling on …

Eosinophilic annular erythema is an idiopathic acute eosinophilic dermatosis. It is a rare condition, with approximately 30 cases reported in the English literature. It features annular, figurate urticarial edematous plaques primarily affecting the trunk and proximal limbs. During evaluation of a patient, secondary causes of eosinophilic inflammation such as allergy-related conditions (eczema, drug, urticaria, contact dermatitis), parasitic infestations, and autoimmune dermatoses will need to be excluded. We present an unusual case of a 47-year-old patient who developed this condition.

Eosinophilic annular erythema (EAE) is a rare eosinophilic dermatosis characterized by annular, erythematous papules and plaques commonly found on the trunk and the extremities. There is continued debate on whether EAE is a distinct entity or a clinical polymorphism of Well's syndrome, but it is generally considered a separate entity based on clinical and histopathological differences. We present a case of EAE and discuss the histopathological findings.

Annular erythema is one of the cutaneous manifestations of Sjogren's syndrome (SS). Topical corticosteroids and tacrolimus, and oral corticosteroids, have been used as treatments for this condition. However, the safety and efficacy of these treatments remains unsatisfactory, and further development of therapies are desired. In this study, we performed a retrospective analysis of 16 annular erythema associated with SS (AESS) patients treated with hydroxychloroquine (HCQ). Disease activity was assessed using a modified version of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI), which we termed the modified CLASI (m-CLASI). HCQ treatment improved AESS lesions in all 16 patients. The mean m-CLASI score was reduced by 85.6% at the 12-week follow-up relative to baseline (p < 0.01). Notably, 60% (6/10 cases) of patients with AESS lesions limited to the facial area achieved complete remission within 4 weeks. In the analysis of six patients who had taken oral prednisolone before starting HCQ, all were able to reduce the dose within 52 weeks without relapse. Particularly, 75% (3/4 cases) of patients with prednisolone dose of more than 5 mg/day could reduce their dose to less than 5 mg/day in combination with HCQ. For the safety concerns, two patients experienced grade 1 diarrhea during the 52-week observation period. However, neither serious adverse events nor adverse events requiring discontinuation of treatment occurred. The results of the present study suggest that HCQ may not only be highly effective as a single agent, but may also be useful as a steroid-sparing agent in refractory case requiring long-term steroid administration, making it a good treatment option for AESS.

Several skin diseases can present with annular lesions, including granuloma annulare, urticaria, tinea, erythema chronicum migrans, and lupus erythematosus. In addition, there are rare annular diseases whose nosographic identity is accepted including erythema annulare centrifugum (EAC) and erythema gyratum repens. In this chapter, rare variants of annular erythema whose nosographic positioning is still debated are discussed. They are annually recurring EAC, neutrophilic figurate erythema, palpable migratory arciform erythema, eosinophilic annular erythema, and annular lichenoid dermatitis of youth.

Annular erythema as a clinical manifestation of locoregional recurrence of breast cancer is extremely rare. Only three papers with the description of five cases have been published in the current literature to our knowledge. Herein, we describe two additional cases of annular erythema as a cutaneous sign of recurrent ductal breast carcinoma; both were misdiagnosed as erythema chronicum migrans.

Erythema annulare centrifugum (EAC) is an unusual skin condition appearing as recurrent erythematous annular eruptions associated with autoimmune disorders, infections, and various neoplastic conditions. We present a very rarely reported association of EAC with Hashimoto thyroiditis (HT) in a young male. A 26-year-old male recently diagnosed as case of HT presented in the dermatology clinic with nine-month history of non-itchy persistent annular lesions on the body. The morphology and biopsy of lesions confirmed the diagnosis of EAC. HT is a part of the spectrum of autoimmune thyroid diseases with its own specific cutaneous manifestations. Our case also depicts the impact of antigen-antibody related immunological reaction, which might be involved in the development of both HT and EAC, and it could be the stages of the same pathological condition of two different clinical presentations.

Lyme disease is a tick-borne illness, which is typically caused by Borrelia Burgdoferi. Over time, a typical Borreliosis skin reaction takes shape, i.e. the formation of an annular erythema that tends to expand centrifugally with erythematous edges whose diameter can reach up to 20 cm. The symptoms of Lyme disease are not only cutaneous but there may be a systemic involvement. Obviously, this disease can also affect pregnant women and for this reason this review aims to summarize the main ways of treatment to avoid worsening of the clinical condition in the mother and an eventual, albeit rare, involvement of the fetus.

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.

The etiology of purpura in Sjögren syndrome (SS) includes cryoglobulinemia, hypergammaglobulinemia, and leukocytoclastic vasculitis (LCV). The clinical symptoms of LCV associated with SS comprise palpable or nonpalpable purpura and urticarial vasculitis. Here, we report a case of LCV presenting as erythema gyratum repens (EGR)-like purpura in a 62-year-old woman with rheumatoid arthritis and SS. EGR-like skin lesions, characterized by annular lesions with expanding concentric pattern and coalescing to form a zebra-like pattern or grain of wood pattern, can appear in various autoimmune conditions; however, EGR-like eruption in SS is extremely rare. On the basis of the expansion pattern, we considered that the EGR-like purpura in this case was elicited by urticarial vasculitis accompanied by SS.

Erythema papulatum centrifugum (EPC), also known as erythema papulosa semicircularis recidivans (EPSR), is distinct from eczema and other well-described figurate erythemas characterised by annular erythematous lesions. We report 7 cases of EPC and propose new diagnostic criteria including the following: (i) EPC is characterised by single or multiple recurrent expanding annular or semi annular erythema with central regression, surrounded by tiny red papules; (ii) the lesions regularly relapse and resolve; (iii) the histopathologic feature shows superficial perivascular inflammation with or without mild inflammation around sweat glands in the mid dermis and (iv) patients lack other associated cutaneous or internal abnormalities.

A middle-aged man displayed annular erythematous plaques with crusted borders and tiny vesicles distributed on the face, neck, trunk, and upper extremities. The histological picture and serum indirect immunofluorescence supported the diagnosis of an extremely rare condition.

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.

Annular erythema with Sjögren's syndrome (AESS) is occasionally found, especially in Asian patients, which is classified into three types. We present a case of Sjögren's syndrome showing various types of AESS with anti-signal recognition particle antibody-positive polymyositis. We successfully treated the eruption and myositis with a low dose of prednisolone. Every onset of annular erythema coincided with elevation of serum creatine kinase levels, which suggests the correlation between the activities of annular erythema and polymyositis.

Eosinophilic annular erythema (EAE) is a rare and relatively newly described eosinophil-rich dermatosis. Debate still exists as to whether it represent a subtype of Well syndrome or a separate disease entity. We report an 8-year-old boy with a 4-year history of recurrent, asymptomatic annular lesions, which were diagnosed after clincopathological correlation as EAE. This condition usually runs a relapsing and remitting course with resistance to multiple treatments. Prednisolone and hydroxychloroquine have been reported as successful but the response to these was limited in this case. Complete resolution occurred after treatment with ultraviolet B (UVB) therapy. To our knowledge, this is the first report of a favourable response of EAE to such therapy.

Eosinophilic annular erythema is a rare, benign, recurrent disease, clinically characterized by persistent, annular, erythematous lesions, revealing histopathologically perivascular infiltrates with abundant eosinophils. This report describes an unusual case of eosinophilic annular erythema in a 3-year-old female, requiring sustained doses of hydroxychloroquine to be adequately controlled.

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. El eritema anular eosinofílico (EAE) es una dermatosis eosinofílica poco frecuente. Clínicamente se caracteriza por episodios recurrentes de placas anulares o figuradas. El estudio histopatológico muestra un infiltrado inflamatorio en dermis superficial y profunda, de localización perivascular y compuesto por linfocitos y eosinófilos. Se describió originariamente en niños. Presentamos una mujer adulta con episodios recurrentes de placas anulares o figuradas en el tronco y extremidades. La biopsia mostró un infiltrado linfocítico perivascular con numerosos eosinófilos en la dermis. La analítica reveló la presencia de hipotiroidismo subclínico. Las lesiones se resolvieron después de tres meses de tratamiento con una crema de corticoesteriodes.

• A novel missense mutation in the lactate dehydrogenase A gene (LDHA) was identified in a Chinese family with glycogenosis type XI.

Eosinophilic annular erythema is a rare, benign, recurrent condition characterized by annular skin lesions, tissue eosinophilia, and resistance to a variety of treatments. There are fewer than 30 cases reported in the English literature, 7 of which are in children. We present a case of recurrent eosinophilic annular erythema in an adolescent that was successfully treated with dupilumab, an interleukin-4 receptor alpha antagonist.

Objective The objective of this paper is to evaluate the prevalence and characterize the main epidemiological, clinical and immunological features of annular erythema (AE) in non-Asian patients with primary Sjögren’s syndrome (SS). Methods We carried out a retrospective study searching for AE in 377 Spanish patients with primary SS fulfilling the 2002 American-European criteria. In addition, we searched PubMed (1994–2012) using the MeSH terms “annular erythema” and “primary Sjögren’s syndrome” for additional cases. All cases with AE reported in patients with SS associated with systemic lupus erythematosus were excluded. Results In our Spanish cohort, we found 35 (9%) patients diagnosed with AE. All were white females, with a mean age of 47 years at diagnosis of AE. AE preceded diagnosis of SS in 27 (77%) patients. Cutaneous AE lesions involved principally the face and upper extremities. All patients reported photosensitivity, with cutaneous flares being reported during the warmest months in 93% of patients. Immunological markers consisted of anti-Ro/La antibodies in 31 (89%) patients. In the literature search, we identified eight additional non-Asian patients with primary SS diagnosed with AE. In comparison with 52 Asian patients, the 43 non-Asian patients with AE related to primary SS were more frequently women (100% vs 78%, p = 0.008), and cutaneous lesions were less frequently reported in the face (55% vs 81%, p = 0.045) and more frequently in the neck (40% vs 14%, p = 0.041). Immunologically, non-Asian patients had a lower frequency of anti-Ro antibodies and a higher frequency of negative Ro/La antibodies, although the differences were not statistically significant. Conclusion AE is not an exclusive cutaneous feature of Asian patients with primary SS. In addition to the characteristic cutaneous expression, AE has a very specific clinical and immunological profile: often presenting before the fulfillment of SS criteria, overwhelmingly associated with anti-Ro antibodies but weakly associated with other immunological markers and the main systemic SS-related features.

Classification of the wide variety of autoimmune diseases that can occur before or after the onset of Sjögren’s syndrome (SS) is currently debated within the conventional SS criteria or as primary SS ( pSS) developing autoimmune disease or as ‘associated-overlap’ with other systemic autoimmune diseases. There is also debate on whether or not to consider annular polycyclic subacute cutaneous lupus erythematosus (SCLE) and annular erythema associated with Sjögren's syndrome (AESS) as a spectrum linked to Ro-SSA and/or La-SSB auto-antibodies (SSA/SSB auto-ab). We present the case of a 55-year-old female patient, with pSS positive for SSA and SSB auto-ab, who developed chronic relapsing polymyositis and atypical annular non-polycyclic SCLE lesions resembling AESS, which seemed to suggest a common spectrum. While a chronic-progressive polymyositis may be generally accepted as a relatively rare myositis complicating pSS, interpretation of annular lesions of non-systemic SCLE in SS patients might actually be underestimated as pSS skin manifestation likely related to SSA/SSB auto-ab.

We herein report two cases of eosinophilic annular erythema in adults, which is rare. In both patients, lesions developed rapidly in few days and were located mainly on the trunk, buttocks, and extremities. Diagnosis was histopathological, with typical features including acute dermal inflammatory infiltrate with abundant eosinophils. One of the patients recurred after treatment on three occasions and finally cured spontaneously. The second patient recurred once and was then successfully treated with topical clobetasol. Clinical and histopathological features of eosinophilic annular erythema in adults have rarely been reported. A review of the literature and discussion of relationship with Wells syndrome are also included.