Differential diagnosis between urticarial vasculitis and chronic spontaneous urticaria: An international Delphi survey.

BACKGROUND: Urticarial vasculitis (UV) should be differentiated from chronic spontaneous urticaria (CSU) in patients initially presenting with recurrent wheals, although criteria for differential diagnosis remain ill-defined. OBJECTIVES: To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases. METHODS: Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round. RESULTS: The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients. CONCLUSIONS: The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum.

Anxiety in heart failure patients and its association with socio-demographic and clinical characteristics: a cross-sectional study.

Anxiety is one of the most frequent psychiatric disorders in heart failure (HF) patients. However, it is often neglected in clinical practice and studies about the particular relationship with the New York Heart Association (NYHA) classes for HF are scarce. In this context, this study aims to analyze the presence of anxiety symptoms in HF outpatients and also its association with sociodemographic and clinical characteristics of these patients. Methods: This cross-sectional study is part of the longitudinal Deus Ex-Machina project (NORTE-01-0145-FEDER-000026). HF patients were recruited at an outpatient clinic at a University Hospital. Patients with inability to communicate, severe visual/hearing impairment, or NYHA class IV were excluded. Sociodemographic data and NYHA class were registered. Anxiety was assessed with the 7-item Generalized Anxiety Disorders Scale (with a score ≥10 clinically relevant anxiety). Patients with and without anxiety were compared regarding socio-demographic and clinical variables. Results: The sample (n = 136) had a median age of 59years (Q1: 49; Q3: 68), 66.2% were male and 31.6% presented clinically relevant anxiety. A higher percentage of HF patients with anxiety had psychiatric disorders (58.1% vs 26.9%; P = .001), psychotropic medication (62.8% vs 30.1%; P = .001), and depression (60.5% vs 9.7%; P< .001). No significant differences were found regarding the remaining variables, including NYHA classes. Conclusions: A substantial proportion of HF patients present clinically relevant anxiety, particularly those with psychiatric history, depressive symptoms, or under psychotropic medication. Therefore, integrating routine screening and treatment of this comorbidity in clinical practice is of utmost importance. Further studies are needed to clarify the association of anxiety with HF.

Histopathology of chronic spontaneous urticaria with occasional bruising lesions is not significantly different from urticaria with typical wheals.

BACKGROUND: Chronic spontaneous urticaria (CSU) may occasionally exhibit long-lasting lesions with bruising, usually considered a hallmark of urticarial vasculitis (UV). Histopathology of these chronic urticarial lesions has not been extensively studied. METHODS: Skin biopsies from patients with anti-H1 resistant CSU were evaluated for several parameters (edema, location, intensity, and cell composition of the inflammatory infiltrate, and abnormalities in the blood vessels). RESULTS: We studied 45 patients (37 female/8 male, mean age 49.3 years) with CSU, 60% of whom with occasional bruising lesions and 3 patients with hypocomplementemic UV. Histopathology in CSU showed mainly perivascular and interstitial inflammatory infiltrate (91.1%), including eosinophils (80%), neutrophils (77.8%), and lymphocytes (71.1%), vasodilatation (88.9%), intravascular neutrophils (95.6%), dermal edema (51.1%), swelling of endothelial cells (51.1%), and minor and rare fibrinoid necrosis and karyorrhexis (6.7%). Significant karyorrhexis and frank fibrinoid necrosis were observed, respectively, in two and three cases of UV. In patients with occasional bruising, mast cells occurred in fewer cases whereas eosinophils were more frequent, but no statistically significant difference was found for other parameters. CONCLUSIONS: Histopathological findings were not significantly different between CSU with or without bruising lesions. Bruising may be associated with more severe forms of CSU with no histopathological signature, although UV cannot be completely excluded based on histopathology.

Atypical vascular proliferations of the neck.

Atypical vascular proliferations (AVP) are a late complication after radiotherapy. Most cases have been reported in female breast cancer patients on the chest wall. These lesions are mostly of the lymphatic type. Herein, we report a blood vascular-type AVP in a male on the neck 60 years after radiotherapy for a benign hemangioma, which makes this case exceptional. We removed the whole chronic radiodermatitis surgically. Histopathology excluded vascular malignancies but confirmed AVP. We discuss the differential diagnoses and treatment.

Histopathology of the Exanthema in DRESS Is Not Specific but May Indicate Severity of Systemic Involvement.

OBJECTIVE: Exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) has no specific clinical diagnostic hallmark and there are few histopathologic studies. The aim of this study was to describe dermal-epidermal histopathologic features in DRESS and correlate them with the culprit drug, viral reactivation, or systemic organ involvement. METHODS: Skin biopsies were independently evaluated by 2 dermatopathologists who characterized the main histological patterns and scored dermal and epidermal changes, which were further correlated with clinical and laboratorial data. RESULTS: In 15 DRESS patients (9 male/6 female patients, mean age 53.3 years), the main observation was lymphocyte exocytosis (1.87 ± 1.25), spongiosis (0.93 ± 0.94), scattered keratinocyte necrosis (1.70 ± 1.44), basal cell vacuolization (2.13 ± 1.42), lymphocyte infiltration around dermal vessels (2.93 ± 0.92) or at the dermal-epidermal junction (2.07 ± 1.12), often with eosinophils and extravasated erythrocytes, swollen endothelial cells, and intravascular neutrophils but no vasculitis. Histopathologic patterns were classified mainly as spongiotic (5), erythema multiforme-like (3), or lichenoid (2). There was a significant positive correlation between the intensity of lymphocyte infiltration and the severity of hepatic cytolysis (r = 0.51; P < 0.05) and eosinophilia (r = 0.51; P < 0.05). No correlation was observed between the intensity and type of dermal inflammation and the degree of epidermal damage or the culprit drug. Human herpes virus type 6-positive patients had a pseudolymphomatous reaction or a perifollicular localization of the infiltrate. CONCLUSIONS: Histopathology in DRESS is variable with no specific diagnostic aspect, but there is a possible correlation between the intensity of the lymphocyte infiltrate and DRESS severity, namely, liver cytolysis.

Malignant sweat gland tumours: an update.

Cutaneous adnexal tumours can be a diagnostic challenge for the pathologist. This is particularly true in the case of tumours with sweat gland differentiation, due to a large number of rare entities, a multiplicity of names to designate the same neoplasms and consequent lack of consensus regarding their classification and nomenclature. In the traditional view, sweat gland tumours were divided into eccrine and apocrine. However, this has been challenged in recent years, and in fact many of these tumours may have both eccrine and apocrine variants. Some display more complex features and defy classification, due to the presence of other lines of differentiation, namely follicular and/or sebaceous (in the case of apocrine tumours, due to the close embryological relationship between apocrine glands, hair follicles and sebaceous glands). The present paper reviews and updates the basic concepts regarding the following malignant sweat gland tumours: apocrine carcinoma, porocarcinoma, hidradenocarcinoma, spiradenocarcinoma, cylindrocarcinoma, microcystic adnexal carcinoma and related entities, squamoid eccrine ductal carcinoma, digital papillary adenocarcinoma, primary cutaneous mucinous carcinoma, endocrine mucin-producing sweat gland carcinoma and primary cutaneous signet ring cell carcinoma. Particular emphasis is put in recent findings that may have implications in the diagnosis and management of these tumours.