Annular Leukocytoclastic Vasculitis: A New Feature of IgA Vasculitis.

Background: Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases. Case description: A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse. Conclusion: An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis. LEARNING POINTS: Annular erythema may reveal an IgA vasculitis.Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.This presentation is treated with topical steroids.

A case of leukemia cutis showing annular erythema during the course of Philadelphia chromosome-positive acute B-lymphoblastic leukemia.

We report a case of leukemia cutis showing annular erythema during the course of Philadelphia chromosome-positive acute B-lymphoblastic leukemia. The annular appearance may be developed by immunomodulatory effects of blinatumomab.

Centrifugally Spreading Annular Erythema as a Dermatological Indicator of Metastatic Breast Carcinoma.

Breast cancer is the leading cause of skin metastasis in women with internal malignancies. This report highlights an atypical case of cutaneous metastasis of breast cancer (CMBC) in a 66-year-old woman. Starting four months before her dermatology consultation, the patient underwent a chemotherapy regimen comprising pertuzumab, trastuzumab, and vinorelbine for right breast cancer, right axillary lymph node enlargement, and bone metastases. After commencing chemotherapy, erythematous macules appeared around her right nipple. Subsequently, the cutaneous lesions developed into annular erythematous patches around her right nipple and began to coalesce and expand to the contralateral breast. A skin biopsy revealed dysplastic cells indicative of metastasis from invasive ductal carcinoma. In addition, lymphovascular tumor cell invasion was noted in the reticular dermis. Based on these clinical progressions and histopathologic findings, a diagnosis of CMBC was made, specifically considering the possibility of inflammatory breast cancer (IBC). The patient continued the same chemotherapy regimen for 17 cycles, which improved the skin lesions, but she succumbed to breast cancer two years later. This case emphasizes the importance of considering CMBC in breast cancer patients with expanding, treatment-resistant thoracic cutaneous lesions, especially in aggressive subtypes like IBC. The diverse presentations of CMBC require thorough histopathological evaluation.

Figurate erythema in 11 Eastern European sphynx cats.

BACKGROUND: In humans, figurate erythema (FE) represents a heterogenous group of dermatoses with circular or serpiginous erythematous skin lesions; FE has not been reported in cats. OBJECTIVES: To report clinical and histological characteristics and outcomes of FE in sphynx cats from Baltic sea-bordering countries. ANIMALS: Eleven client-owned sphynx cats with FE. MATERIALS AND METHODS: We recruited cases meeting the following criteria: (i) a sphynx breed, (ii) FE with or without scaling, (iii) a chronic, waxing-and-waning course lasting longer than a month and (iv) an absence of other skin diseases. RESULTS: Of 11 cats, there were seven Donskoys, one Peterbald, one Ukrainian Levkoy and two presumed Canadian sphynxes; all except one were males, and the age of onset was <12 months in eight cats. Skin lesions lasted between 1.2 and 56 months, and they consisted of erythematous plaques with a linear-to-serpiginous, annular, gyrate or iris configuration predominating on the trunk and extremities. Scaling was often seen trailing the edge of the centrifugally expanding erythema. All cats were otherwise asymptomatic or mildly pruritic. Dermatophytosis was ruled out by special stains and/or fungal cultures in eight cats. Microscopic lesions revealed focal, mild-to-moderate epidermal hyperplasia and hyperkeratosis, minimal-to-mild dysplasia and subepidermal collagen smudging. Special stains were negative for dermatophytes. The clinical remission of FE was not achieved with diet changes or medical interventions; yet, a spontaneous, transient, partial or complete improvement occurred in most cats. CONCLUSION AND CLINICAL RELEVANCE: This is the first report of FE in sphynx cats from Eastern Europe.

Eosinophilic Annular Erythema Responding to Doxycycline.

Eosinophilic annular erythema (EAE) is a rare skin disease characterized by relapsing and remitting pruritic, annular erythematous plaques and tissue eosinophilia. A 39-year-old male presented with a mildly pruritic, relapsing, and remitting urticarial rash. A biopsy revealed superficial and deep perivascular dermatitis with numerous eosinophils and some neutrophils, with an absence of flame figures. Based on clinical and histopathologic findings, the patient was given a diagnosis of eosinophilic annular erythema. Treatment was initiated with doxycycline 100 mg twice daily. The patient reported substantial improvement at three months and sustained clearance at one year, remaining on doxycycline well tolerated throughout. To our knowledge, no cases of EAE improving with doxycycline have been reported in the literature and, thus, our findings highlight a potential new therapy to consider in a patient with EAE.

Diagnostic difficulties in pediatric annular dermatoses.

The polymorphic presentation of annular dermatoses in the pediatric population renders them a diagnostic 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 a young 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.

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.

Annular erythema of infancy: A rare and challenging diagnosis.

Annular erythema of infancy is a rare, benign disease characterized by enlarging annular patches and plaques that resolve spontaneously. Histopathology typically demonstrates a perivascular mixed lymphohistiocytic infiltrate with increased eosinophils. We present two cases of annular erythema of infancy, at ages 2-4 weeks, and review the literature on annular erythema of infancy. It is important to differentiate this distinct, benign disease from serious autoimmune or infectious processes, such as neonatal lupus erythematosus and syphilis, which may present with similar annular lesions in infancy.

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.

Sjögren's Syndrome with Lichen Sclerosus: A Case Report.

Sjögren's syndrome (SJS) is a systemic disease in which the accumulated skin can include xeroderma, eyelid dermatitis, and annular erythema (AE). To the best of our knowledge, there are few reports on cases of SJS with concomitant lichen sclerosus (LS). Herein, we report the case of a 69-year-old woman with these two diseases. The patient's skin showed atrophic leukoplakia and AE. Based on the comprehensive evaluation of general condition, the patient was diagnosed and actively received treatment. After systematic treatment, her symptoms were controlled. However, the patient's condition requires long-term management.

A case of annular erythema of infancy accompanied by elevated tryptase.

Annular erythema of infancy (AEI) is characterized by self-limited eruptions of erythematous, annular to polycyclic patches and plaques, the etiology of which is thought to involve a hypersensitivity reaction to an unknown antigen. We present a case of AEI mistaken for systemic mastocytosis due to elevated serum tryptase. We were unable to find prior reports of an association between AEI and elevated tryptase in the literature.

Eosinophilic Annular Erythema: A New Entity of Eosinophilic Dermatosis.

Eosinophilic annular erythema (EAE) is a rare dermatosis. Its relationship with Wells syndrome (WS) is debated. We report a case treated with hydroxychloroquine.  A 31-year-old patient presented with a mildly pruritic rash that had been evolving by flares for two weeks. Clinical examination revealed inflammatory erythematous-annular plaques on the trunk and limbs. The blood count was normal. Skin histology showed an eosinophilic-rich inflammatory infiltrate. After local steroid treatment, the patient was treated with oral steroids with a momentary improvement. The course with new relapses is treated by synthetic antimalarial drugs with the complete disappearance of the lesions at a six-month follow-up. Although some authors consider EAE to be a variant of WS, we believe that there are subtle differences that differentiate them despite their clinical similarity.

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

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.

Paraneoplastic Erythema Annulare Centrifugum Eruption (PEACE) Associated With Metastatic Colorectal Adenocarcinoma.

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.

Eosinophilic annular erythema.

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.

Efficacy of hydroxychloroquine for treating annular erythema associated with Sjögren's syndrome.

Annular erythema is one of the cutaneous manifestations of Sjögren'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.