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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) which preferentially infiltrates into subcutaneous adipose tissue is rare, however may mimic autoimmune diseases from the aspect of clinical manifestations. Here, we describe a 16-year-old young man, who initially presented with eyelid erythema and swelling, accompanied by fever and muscle and bone marrow involvement. He was preliminarily considered as a patient with classical dermatomyositis (DM), but finally diagnosed as SPTCL concomitant with paraneoplastic inflammatory myositis, confirmed by in total 8 times repeated biopsies at different sites. After systematically reviewing the literatures, we summarized the main features of SPTCL mimicking DM with eyelid edema as the presenting manifestation. The cautionary tale reminds rheumatologists of considering mimickers in patients with atypical autoimmune-like manifestations. Suitable biopsy is critical for diagnosis and improving prognosis., (© 2021. International League of Associations for Rheumatology (ILAR).)

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.

Rosacea is a disease resulting from dysregulation of innate, adaptive, and neurovascular immune systems. Inflammatory pathways activated in rosacea can explain many of its signs and symptoms. Current treatments address some of these inflammatory processes, alleviating erythema and decreasing papules and pustules. However, for the majority of patients, complete clearance of these features is not currently achievable even with combination therapy. There is a need to address the spectrum of inflammatory processes involved in rosacea and for more efficacious agents with the goal of providing complete clearance for patients. J Drugs Dermatol. 2020;19(6): doi:10.36849/JDD.2020.5187.

Anti-small ubiquitin-like modifier-1 activating enzyme (anti-SAE) antibodies have been recently discovered especially for myosin and identified as dermatomyositis (DM) marker. The frequency of anti-SAE antibodies in DM patients is extremely low. Diffuse pruritic erythema may be one kind of clinical manifestations of DM with anti-SAE antibodies. In this report, a 48-year-old female patient with amyopathic dermatomyositis (ADM) carrying anti-SAE antibodies presented diffuse pruritic erythema for 5 months. Diffuse pruritic erythema improved after treatment with prednisolone, cyclosporine, and thalidomide. The clinical characteristics of 75 previously reported cases with anti-SAE antibody-positive DM were reviewed, and the manifestations of the Asian and Western cohorts were compared. It was revealed that the Asian patients were more susceptible to diffuse erythema (17/34 vs. 3/41, P = 0.000), dysphagia (16/34 vs. 10/41, P = 0.040), and interstitial lung disease (ILD) (21/34 vs. 5/41, P = 0.000) compared with the Western patients. The frequency of malignancy in the Asian cohort was significantly higher than that in the Western cohort (10/34 vs. 4/41, P = 0.030).

Background: The occurrence of chemotherapy-related adverse cutaneous reactions in the setting of capillary leak syndrome (CLS) is quite rare. Our objective was to identify the type of skin reactions associated with CLS., Methods: Leukemia or hematopoietic stem cell transplant patients between January 2010 and December 2017 were identified, and medical records were reviewed for a dermatology consultation occurring concomitantly with CLS., Results: Five patients were identified, two with a diagnosis of toxic erythema of chemotherapy (TEC) and three others with a skin diagnosis of toxic epidermal necrolysis (TEN). Pathology of all patients was available for clinical-pathologic confirmation., Conclusions: Although TEC is generally self-limited, both TEC and TEN can present with severe adverse skin manifestations during CLS secondary to toxicity from chemotherapy., (© 2019 The International Society of Dermatology.)

Competing Interests: None

Competing Interests: None

Introduction: Kawasaki disease (KD) is a challenging diagnosis. Erythema and induration of the Bacillus Calmette-Guérin (BCG) site is increasingly recognised as a significant clinical clue. However, there is little data to support its specificity for KD as compared to other febrile illnesses. We aimed to evaluate BCG reaction or induration as a diagnostic tool for KD., Methods: A retrospective case-controlled study of patients discharged with a diagnosis of KD from 2007 to 2010 was conducted. Another group of patients admitted over the same period for possible KD, but later found not to have KD, served as control., Results: Significantly more infants with KD (69.7%) had BCG site changes than older children (27.8%; p < 0.001). It also presented earlier in the course of KD; < 5 days (53.3%) compared to ≥ 5 days of fever (30.0%; p < 0.001). Positive predictive value of BCG site reaction or induration for KD was 90.8% (95% confidence interval [CI] 0.819-0.962) for infants and 96.2% (95% CI 0.868-0.995) for older children. The prevalence rate of changes at the BCG site was 9.9% among patients with non-KD febrile illnesses and 42.6% among patients with KD., Conclusion: BCG site reaction or induration is a useful clinical clue for the diagnosis of KD in both infants and older children, with a higher prevalence in infants. Physicians should consider KD in children with febrile illness and redness or crust formation at the BCG site, especially in view of low rates of BCG reaction or induration in non-KD febrile illnesses., (Copyright: © Singapore Medical Association.)

We report here one case of Zika virus (ZIKV) infection associated with auto-immunity directed against the central nervous system in a Brazilian woman who developed acute transverse myelitis 9 days after recovery from an acute episode of fever with generalized erythema. Imaging of the spinal cord showed an elongated area on the T1-T10 level with gadolinium uptake. The diagnostic of the ZIKV infection was confirmed by cerebrospinal fluid and serum analysis. This patient had serum positivity for autoantibodies against myelin oligodendrocyte glycoprotein (MOG), a specific antibody against the myelin sheath. We propose that a direct central nervous system infection by ZIKV could lead to a specific auto-immunity against MOG protein.

Objectives: Eosinophilic esophagitis (EoE) is an inflammatory, atopic disease of the esophagus without a clear etiology. Our objective was to identify exposures and conditions in early infancy associated with the development of EoE., Methods: A case-control study was performed using the Military Health System Database. Subjects diagnosed with EoE from October 2008 to September 2015 were matched 1:2 on age and sex. Early infant risk factors from the first 6 months of life were investigated., Results: A total of 1410 cases with EoE were matched to 2820 controls. The median (interquartile range) age at diagnosis of EoE was 4.2 years (2.1-7.2) and 68.7% were boys. Proton pump inhibitors (adjusted odds ratio [aOR], 2.73; 95% confidence interval [CI] 1.93-3.88), histamine-2 receptor antagonists (aOR, 1.64; 95% CI 1.27-2.13), and antibiotics (aOR, 1.31; 95% CI 1.10-1.56) were associated with EoE. Prematurity (aOR, 1.46; 95% CI 1.12-1.89) and early manifestations of atopic disease such as milk protein allergy (aOR, 2.37; 95% CI 1.26-4.44) and eczema (aOR, 1.97; 95% CI 1.64-2.36) were related to increased odds for EoE. Erythema toxicum in infancy was strongly associated with a diagnosis of EoE (aOR 3.52; 95% CI 1.03-12.04). Infants with feeding difficulty (aOR, 1.45; 95% CI 1.18-1.77) and gastroesophageal reflux disease (aOR, 1.79; 96% CI 1.43-2.26) were also at increased risk for EoE., Conclusions: Acid-blocking medications and antibiotics during infancy were associated with later diagnosis of EoE. Erythema toxicum neonatorum, an eosinophilic immune phenomenon, was strongly associated with EoE. Identifying early infant risk factors for EoE may help to risk stratify the need for endoscopy.

Background/objectives: Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined., Methods: We performed a retrospective review of patients who clinically presented with annular erythema between 2000 and 2016. Altogether, 30 patients with a histopathological presentation of isolated lymphocytic hidradenitis were identified. Their following characteristics were recorded: clinical features, number and localisation of lesions, serology and other associated medical conditions., Results: Isolated lymphocytic hidradenitis was found most frequently in middle-aged women. Most patients (n = 28, 93%) presented with many annular erythematous patches and plaques with mild pruritus; 22 (73%) had the SS-A antibody and 17 (57%) met the diagnostic criteria of Sjögren syndrome. Among these patients, 11 had primary and six had secondary Sjögren syndrome associated with systemic lupus erythematosus. Altogether 15 (50%) patients tested positive for a high titre of the antinuclear autoantibody. Other underlying diseases identified during the follow-up period include cryoglobulinaemia, angioimmunoblastic T-cell lymphoma, autoimmune hepatitis, hepatitis C infection and toxic thyroid goitre., Conclusions: Lymphocytic hidradenitis is a microscopic finding associated with annular erythemas of Sjögren syndrome. Systemic survey for sicca symptoms and work up for autoimmune diseases, including antinuclear antibodies, SS-A, SS-B antibodies, cryoglobulin, lymphoma, viral and autoimmune hepatitis should be performed to facilitate the correct diagnosis., (© 2017 The Australasian College of Dermatologists.)

The purpose of this study is to evaluate the outcome of a series of patients with erythematotelangiectatic rosacea (ETR) affected by persistent erythema and varying degree of telangiectasias being treated with brimonidine alone or combined with a vascular laser based on the type of vascular components preliminarily evaluated by clinical and instrumental observation. Ten patients affected by ETR were enrolled in a pilot, open study. Instrumental evaluation included erythema-directed digital photography by VISIA-CR™ system and X10 dermoscopy. Those patients showing marked background erythema and minimal telangiectasias (group A) were treated with a single application of brimonidine 0.33% gel, while patients showing both marked background erythema and marked telangiectasias (group B) were treated with a session of Nd:YAG laser and reevaluated 1 month later after a single application of brimonidine. An Investigator Global Assessment (IGA) of treatment outcome was performed at the end of treatment in both groups. In group A, 6 h after brimonidine application, a marked reduction of the background erythema was observed in all patients, and IGA was rated as excellent. In group B, 6 h following the application of brimonidine, a marked reduction of the background erythema was observed in all cases, while telangiectasias remained unchanged. A further treatment with brimonidine 1 month after the Nd:YAG laser session determined complete clearing of facial erythema, and IGA was rated as excellent. In conclusion, a preliminary evaluation of the vascular component by erythema-directed digital photography and dermoscopy in ETR may be helpful to select the most appropriate therapeutic strategy.

Competing Interests: There are no conflicts of interest

Necrotising infection of the lower limb is a rare presentation for colorectal malignancy. We report a case of a perforated caecal adenocarcinoma presenting with right leg erythema, pain and swelling in the presence of a right lower lobe lung opacity. Following initial debridement and washout, CT imaging demonstrated a thickened terminal ileum, caecum and appendix, in keeping with primary malignancy. This fed the right-sided lower limb sepsis tracking down from the medial aspect of the psoas muscle to give rise to the multiloculated collection seen in the adductor compartment. The lung lesion measured 16 mm and was metastatic. The patient was successfully managed with a subtotal colectomy and an end ileostomy. The biopsy confirmed an adenocarcinoma (T4N1M1). We highlight the importance of perforated colonic carcinoma as a leading differential for lower limb abscesses. Suspicions should be raised further if accompanied by rounded opacifications on plain film radiography of the lungs., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Serum sickness is a hypersensitivity reaction to proteins in antiserum derived from nonhuman animal sources and can be seen in patients being treated with antiserum to prevent transplant rejection. Serum sickness may display variable clinical presentations. Because cutaneous findings may be the initial symptom in some cases, it is important for dermatologists to be able to recognize this condition given its potentially life-threatening symptoms. We present a case of a 35-year-old man with presumed serum sickness after receiving thymoglobulin for the treatment of acute cellular rejection of a heart transplant. The clinical presentation, laboratory findings, and treatment options are reviewed.

Background: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The characteristic episodes of subcutaneous/submucosal edema formation may be preceded by erythema marginatum (EM) - the occurrence of a 'map-like' pattern on the skin. EM can occur as an isolated finding or accompanying a hereditary angiooedema (HAE) attack as well. Nevertheless, it is unknown whether a HAE attack can be prevented by the proper prophylactic treatment during EM., Objectives: Our aim was to assess the prevalence of EM in the Hungarian C1-INH-HAE population and to introduce a safe and effective novel prophylactic treatment during EM in patients who's HAE attacks are preceded by EM in a considerable proportion of the cases., Results: Based on the data of the Hungarian HAE Registry, 49 among 173 C1-INH-HAE patients (28.3%) from 32 families had EM in their life. According to the clinical data and the patient's HAE diaries, two patients (Patient #1, Patient #2) were selected who frequently had EM as a prodromal symptom. Both patients were instructed to administer plasma-derived C1-inhibitor concentrate (pdC1-INH) as soon as possible after the onset of EM, in order to prevent the development of HAE attack. Interestingly, HAE attacks never developed if pdC1-INH was administered within 6h from the occurrence of EM in both patients. In contrast, without pdC1-INH treatment, in Patient 1 and Patient 2, 97.0% and 44.3% of the EM were followed by a HAE attack, respectively (p<0.0001, Fisher's exact test)., Conclusions: As a novel prophylaxis in C1-INH-HAE, intravenous administration of pdC1-INH concentrate during EM might be an effective, individual therapeutic strategy in those patients who's HAE attacks are often preceded by EM. Besides it can improve the quality of life of these selected patients, pdC1-INH administration during EM provides the lowest effective dose for the prophylaxis of their HAE attacks., (Copyright © 2017 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.)

A 69-year-old woman with a 12-month history of asymptomatic erythematous, non-scaly, annular and arciform plaques on her face, trunk and extremities is presented. The skin lesions had been unresponsive to treatment with systemic corticosteroids and antihistamines. Skin biopsy demonstrated superficial and deep dermal inflammatory infiltration consisting mainly of eosinophils and a few neutrophils. Dermal interstitial mucin deposition was also detected in the absence of vasculitis, flame figures or granulomatous reaction. The patient was diagnosed as having eosinophilic annular erythema and treated with hydroxychloroquine (2 × 200 mg/day, p.o.). Response to treatment was observed after 7 weeks and full recovery was achieved after 10 weeks. Eosinophilic annular erythema is rarely reported in the literature. Although hydroxychloroquine is a good choice for treatment, response time can vary between patients., (© 2016 The Australasian College of Dermatologists.)

BACKGROUND: There are few studies on the natural history of acne lesions including the antecedents of atrophic scars.

STUDY DESIGN: Prospective study of relationship between primary (papules, pustules, comedones) and secondary lesions (atrophic scars, macular erythema, and hyperpigmentation) over 6 months. Subjects (n=32) had moderate facial acne including 10 or more atrophic acne scars and were their own control via randomized split-face design. Lesions were mapped 2x/week for 2 months and every 2 weeks thereafter until month 6 to track pathogenic progression.

RESULTS: Clinical assessment showed acne scars continuously forming throughout the 6-month study period. While the majority (66.2%) of these scars did not resolve by study endpoint, the remainder were transient. The likelihood of a scar developing from a primary acne lesion was 5.7%, and almost all scars arose from erythematous macules or hyperpigmentation (83%) and some (16%) developed directly from papules and pustules. Duration of papules was a key factor in the risk of scarring. The majority (81.7%) of the scars remaining at 6 months were still present at 2-year follow-up.

CONCLUSIONS: Atrophic acne scars continuously form, some resolve, and evolve primarily from inflammatory and post-inflammatory lesions. Clinicians should closely monitor patients with macular erythema for scarring.

J Drugs Dermatol. 2017;16(6):566-572.

Gyrate erythema, which also is known as erythemaannulare centrifugum (EAC), is a reactive dermatitisthat is thought to occur in response to an underlyingtrigger. The superficial form is characterized bythe typical, centrifugally-expanding, annular,erythematous patches or plaques with a distincttrailing scale. The deep form also is a centrifugallyexpanding,erythematous plaque but with induratedborders and absence of scale. These cutaneousfindings are thought to be reactive, most often inresponse to infections or drugs and, less likely, tounderlying malignant conditions.

Erythema dyschromicum perstans is a rare, chronic, pigmentary disorder with unknown etiology. It clinically presents with oval to round, gray, blue, or brown macules of various sizes. The etiology remains unknown; however, cobalt allergy, radio contrast media, intestinal parasites, human immunodeficiency virus, and hypothyroidism have been proposed as causative factors. In addition, vitiligo is characterized by depigmented macules and patches that are widely andsymmetrically distributed. It has been suggested that autoimmune mechanisms play an important role in the etiopathogenesis of vitiligo. Physical and emotional stress can trigger vitiligo in genetically predisposed patients. However, coexistence of erythema dyschromicum perstans and vitiligo is extremely rare, and similar immune mechanisms have been implicated in the pathogenesis of these cutaneous pigmentary disorders.

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., (© 2016 Japanese Dermatological Association.)