64-year-old woman • hot flashes, facial flushing, excessive sweating, and palpitations • daily headaches • history of hypertension • Dx?

► hot flashes, facial flushing, excessive sweating, and palpitations ► daily headaches ► history of hypertension.

Differential diagnosis of a thyroid mass, facial malar rash and ptosis on the flora in the primavera by Sandro Botticelli (1445-1510).

PURPOSE: The Primavera is considered amongst the greatest and controversial artistic masterpieces worldwide painted by renaissance artist Sandro Botticelli. The aim was to identify any underlying medical foundations for the painting. METHODS: Observational study. RESULTS: The painting reveals, a 'butterfly' malar rash, bilateral ptosis and a clear neck swelling consistent with a goitre in the figure of Flora. This could be explained by concomitant Graves' disease and systemic lupus erythematosus, or other presentations of multiple autoimmune syndrome. CONCLUSION: These findings highlight the likely presentation of the earliest pictorial depictions of thyroid disease with systemic lupus erythematosus and emphasize the exactitude of depiction demonstrated by Botticelli in renaissance era.

Intravenous ferric derisomaltose for the treatment of iron deficiency anemia.

Intravenous (IV) iron is the therapy of choice when oral iron is ineffective or poorly tolerated, yet use has been limited by fears of hypersensitivity reactions (HSRs). Newer formulations that bind iron more tightly and release it more slowly have made the risk of serious or severe HSRs very low. One such formulation, ferric derisomaltose, has been approved in the United States for delivery of 1000 mg iron in a single IV infusion. Ferric derisomaltose rapidly repletes iron parameters with low rates of serious or severe HSRs. Single-infusion iron repletion offers convenience, eliminates adherence concerns, and reduces healthcare resource utilization.

Harlequin syndrome in a pediatric population: a case series.

Harlequin syndrome is a rare condition, presenting with unilateral facial flushing and hyperhidrosis in response to physical exercise, heat or emotional stressors and has scarcely been reported in pediatric patients. It is caused by a dysfunction of vasomotor and sudomotor sympathetic fiber activity inhibiting the ability to flush on the affected side, causing the neurologically intact side to appear red. We present three pediatric cases of this uncommon syndrome, each of them of different origin and displaying distinct associated (neurological) symptoms, and review medical literature. Insight into the anatomical structure of the thoracocervical and facial sympathetic nervous system is pivotal as it dictates symptomatology. About half of Harlequin syndrome cases are complicated with ocular symptoms and a minority may be part of more extensive partial dysautonomias affecting facial sudomotor, vasomotor and pupillary responses, such as Holmes-Adie syndrome and Ross syndrome. Etiology is generally idiopathic, however, cases secondary to surgery, trauma or infection have been described. Considering its predominantly self-limiting nature, treatment is usually unnecessary and should be restricted to incapacitating cases.

Harlequin syndrome: a case report / Síndrome de arlequín: reporte de un caso

El síndrome de Arlequín es una condición neurológica poco común, la mayoría de las veces benigna. Por lo general la mayor parte de los casos son de causa idiopática (aunque existen causas secundarias). Esta afección consiste en la aparición de eritema e hiperhidrosis facial unilateral desencadenado por el ejercicio, calor, emociones o estrés, la que se produce debido a una disfunción contralateral de la vía simpática vasodilatadora y sudomotora. Se presenta el caso de un paciente de sexo masculino de 49 años, que relata presentar un cuadro de 15 años de evolución caracterizado por la aparición de eritema y sudoración excesiva de la hemicara derecha, la cual termina abruptamente en la línea media de la cara, gatillado por el ejercicio y el calor en verano, mientras que la hemicara izquierda presenta anhidrosis sin eritema.

The Harlequin syndrome is a rare neurological condition, most of the time benign. In general, most cases are idiopathic (although there are secondary causes). This condition consists in the appearance of unilateral facial erythema and hyperhidrosis triggered by exercise, heat, emotions or stress, which occurs due to a contralateral dysfunction of the sympathetic vasodilator and sudomotor pathway. We present the case of a 49-year-old male patient, with no relevant morbid history, who reports a medical history of 15 years of evolution, characterized by the appearance of erythema and excessive sweating of the right side of his face, which ends abruptly in the middle line of the face, triggered by exercise and heat during the summer. While the left side presents anhidrosis without erythema.

Facial flushing and erythema of rosacea improved by carvedilol.

Flushing and erythema are the most common symptoms of rosacea; however, management of these symptoms remains challenging. Recent case studies suggest that treatment with carvedilol may reduce facial flushing and persistent erythema in the pathogenesis of rosacea. To find the effect of carvedilol in the treatment of facial flushing and erythema in rosacea. Twenty-four rosacea patients treated with carvedilol for facial flushing and erythema were retrospectively reviewed. All patients were prescribed carvedilol 6.25 mg either once or twice per day, and the daily dose was gradually titrated up to 12.5 mg. Clinical erythema severity was assessed by the Clinician's Erythema Assessment (CEA) and Patient's Self-Assessment (PSA) scales. Improvement of CEA and PSA scores compared to the baseline were assessed. The proportion of patients with improvement of two or more points from baseline in CEA score was analyzed by sex, previous treatment exposure, disease duration, and subtypes. The mean change of -1.6 in the CEA score and of -1.8 in the PSA score showed significant improvement from baseline. Erythematotelangiectatic rosacea (ETR) patients achieved more than 2-points improvement in the CEA score, compared with non-ETR patients (53.8% vs 16.7% [P = .035]). No statistically significant differences were observed by sex, disease duration, or previous treatment exposure. No serious adverse event was observed. Carvedilol can be an effective and safe treatment option for rosacea patients suffering from facial flushing and erythema.

Pulsed dye laser followed by intradermal botulinum toxin type-A in the treatment of rosacea-associated erythema and flushing.

Rosacea is a common inflammatory skin disease characterized by erythema, episodes of flushing, and inflammatory lesions. It typically affects the face and is more prevalent among fair skin individuals affecting women more than men. Various treatments are available for rosacea with light-based therapies commonly used in the management of erythema. The use of intradermal botulinum toxin type-A has been reported to be beneficial in the treatment of rosacea-associated erythema and flushing with good results and a low side-effect profile. In this article, we present our experience on the successful combination of both pulsed dye laser and intradermal botulinum toxin type-A in erythema and flushing in 20 rosacea patients. In addition to subjective improvement, we measured the degree of erythema using a 3D Antera camera in order to quantify our results. We demonstrated high efficacy and satisfaction rate with this combined approach and a low side-effect profile. To our knowledge, the combination of laser and intradermal botulinum toxin in the management of rosacea has not been previously reported.

Flushing as atypical initial presentation of functional gallbladder neuroendocrine carcinoma: A case report.

BACKGROUND: Neuroendocrine neoplasms are rarely located in the gallbladder (GB), and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms. CASE SUMMARY: We report a case of GB neuroendocrine carcinoma (GB-NEC) in a 65-year-old man, who presented with flushing for 2 mo. Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis. Computed tomography and magnetic resonance imaging indicated neoplasm in the GB with liver invasion and enlarged lymph nodes in the portacaval space. High fluorodeoxyglucose uptake was detected in lymph nodes in the portacaval space, but distant metastasis was not seen by positron emission tomography. Ultrasound-guided needle biopsy of the GB neoplasm was suggestive of high-grade NEC. Because of the functional characteristics of poorly differentiated NEC, en bloc cholecystectomy, resection of hepatic segments IVb and V, pancreaticoduodenectomy, and regional lymphadenectomy were performed. A diagnosis of poorly differentiated NEC was made by pathological findings and immunohistochemical staining data. Ki-67 index was > 80%. The patient refused adjuvant therapy and passed away in the 7th month. CONCLUSION: Distinctive manifestation combined with imaging helps make correct preoperative diagnosis. Radical surgery and adjuvant chemotherapy might improve prognosis.

[Harlequin syndrome after scoliosis surgery]. / Harlekinsyndrom nach Skoliosechirurgie.

Harlequin syndrome is a rare combination of symptoms, characterized by unilateral facial anhidrosis and paleness on the affected side, becoming obvious by contralateral flushing mainly during sports activity. The syndrome is mostly idiopathic, however it is also described as a complication of thoracic surgery, i.e. superior lobectomy. Here, we report on two cases of Harlequin syndrome following scoliosis surgery at the cervicothoracic junction.

Enfoque diagnóstico del paciente con rubor facial transitorio en atención primaria / A Diagnostic Approach of Patient with Flushing in Primary Care

El rubor facial transitorio se define como una sensación de calor, acompañada de enrojecimiento de la piel, causada por una respuesta vasomotora con el resultante incremento en el flujo sanguíneo regional, debido a la acción directa de diferentes mediadores liberados por los nervios. Los cuadros pueden ser episódicos o constantes y se relacionan con respuestas fisiológicas y con patologías de origen benigno y maligno. El objetivo de la revisión es realizar un abordaje racional de esta condición en el escenario de atención primaria, con el fin de llegar a un diagnóstico precoz y completo, abordando los principales diagnósticos diferenciales asociados con esta condición.

The definición of flushing involves a sensation of heat along with redness of the skin, caused by vasomotor influx resulting in an increased of the regional vascular blood flow due to the direct action of different mediators through the vasomotor nerves. These symptoms can be episodio or repetitive and they' can be related to benign or malignant conditions. The objective of this review is to develope a racional approach of the condition at primary care, with the purpose of an early and complete diagnosis, covering the main differential diagnosis related to this condition.

Do you know this syndrome? Harlequin syndrome

Abstract: Harlequin syndrome is a rare condition in which one half of the face fails to flush and sweat due to damage of the sympathetic fibers on the ipsilateral side. The majority of cases are idiopathic, but may be iatrogenic or caused by space-occupying lesions or brainstem infarction. We report a case of idiopathic harlequin syndrome in a 34-year-old man with a 5-month history of unilateral facial flushing and sweating after exercise. Despite the rarity of this syndrome, dermatologists should be aware of this condition in order to diagnose properly and provide multidisciplinary assistance.

Harlequin syndrome following upper lobectomy.

A 58-year-old women developed unilateral facial flushing and sweating on the left side of her face immediately after a right thoracotomy upper lobectomy and paravertebral block. She was diagnosed with Harlequin syndrome in the absence of any other neurological signs or symptoms. She had recovered completely from this episode on follow-up.

MANAGEMENT OF ENDOCRINE DISEASE: Flushing: current concepts.

OBJECTIVE: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions. DESIGN AND METHODS: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'. European and American guidelines relating to neuroendocrine tumours, mastocytosis and menopause were reviewed. RESULTS: In this review, we discuss the main causes of flushing and propose an algorithm based on pathogenesis, which can be used to guide the clinical evaluation process. We also review recent significant developments in the assessment and treatment of the carcinoid syndrome and menopausal hot flushes, which should guide the clinical practice regarding this common but sometimes confusing condition. CONCLUSIONS: When evaluating flushing, a precise systematic approach is needed to exclude potentially serious underlying causes, although despite this, the cause of the disorder is not always found. If symptoms are not progressive, the patient should be advised about its apparently benign nature in order to avoid unnecessary studies or initiating treatments of minimal benefit.

Three Extremely Rare Findings in the Same Patient: Harlequin Syndrome, Thyrocervical Trunk Aneurysm, and Systemic-Pulmonary Arterio-Arterial Fistula.

Harlequin syndrome is a rare autonomic disorder characterized by unilateral diminished sweating and flushing of the face in response to heat or exercise. Extrinsic ganglion compressions, most of the times by neoplasms, can induce the syndrome. During investigation of a 27-year-old woman presenting Harlequin syndrome with diminished sweating on the left side and flushing of the right half of her face, a left thyrocervical trunk aneurysm was detected by angio-magnetic resonance imaging. Thyrocervical trunk aneurysms are rare, and only a few cases have been reported. Treatment is advised due to possible rupture and airway compression by the resulting hematoma. Endovascular treatment was scheduled. Angiographies revealed bronchial arteries arising from a common trunk at the right thyrocervical trunk and the left bronchial artery was feeding an arterio-arterial fistula to the left pulmonary artery; the left thyrocervical trunk aneurysm was confirmed and an anomalous artery arising from the aneurysm converged to the left pulmonary artery arterio-arterial fistula; the fistula was also supplied by a branch of the left internal mammary artery. The young age and lack of clinical antecedents suggest a congenital etiology for this fistula. The treatment was postponed until cardiopulmonary repercussions of the arterio-arterial fistula could be assessed. Coil embolization of the aneurysm was performed in a second procedure. The fistula was not treated. The patient has been followed up for 12 months without complications.

Pharmacologic treatments for rosacea.

Rosacea represents a common and chronic inflammatory skin disorder. Clinical features include transient and permanent erythema, inflammatory papules and pustules, phymatous changes, and ocular signs and symptoms. Rosacea is generally classified into four subtypes and one variant. Subtype 1, erythematotelangiectatic rosacea, includes clinical features of flushing and persistent central facial erythema with or without telangiectasia. Subtype 2, papulopustular rosacea, is characterized by persistent central facial erythema with transient papules or pustules or both on the central face. Subtype 3, phymatous rosacea, includes thickening of the skin with irregular surface nodularities and enlargement. Subtype 4, ocular rosacea, includes inflammation of different parts of the eye and eyelid. A variant, granulomatous rosacea, is noninflammatory and is characterized by hard, brown, yellow, or red cutaneous papules or nodules of uniform size. Patients may present with more than one subtype, and each individual characteristic may fluctuate. There is debate whether rosacea progresses from one subtype over time or subtypes represent discreet entities. Defining clinical presentation and improved understanding of pathophysiology has resulted in identification of novel treatment approaches. This contribution outlines a rationale for treatment, highlights an evidence-based approach with approved treatments, and considers novel developments and off-license therapy available.