Histiocytoid Giant Cellulitis-Like Sweet Syndrome At The Site of Sternal Aspiration: A Case Report

Background: Sweet syndrome with both histiocytoid pathology and giant cellulitis-like lesion feature is extremely rare and has only been reported once. Our case is different from the previous report because the cutaneous lesion was caused by local invasive irritation, which made it much more difficult to distinguish from cellulitis.Case presentation: A 52-year-old male was diagnosed with myelodysplastic syndrome with multilineage dysplasia (MDS-MLD) associated with myelofibrosis (MDS-F) as well as angioneurotic edema of the tongue and floor of the mouth. Seven days after sternal aspiration, a cellulitis-like lesion was formed at the puncture site. Since he had neutropenia, history of glucocorticoid use and didn't keep the site dry and clean after aspiration, cellulitis was diagnosed, followed by broad-spectrum antibiotics and debridement. However, the lesion continued to expand, associated with blisters formation, accompanied by chills and fever. Blood cultures and blister smears didn't detect any pathogens. Biopsy of the lesion was performed and histiocytoid Sweet syndrome was diagnosed. He received prednisone treatment, and the fever relieved within 24 hours and the cutaneous lesion resolved within one week. He has had no recurrence during two-month follow-up.Conclusions: This case can provide help for timely diagnosis and treatment and a reference for further summarizing the characteristics of this rare variant.

Current aspects in the differential diagnosis of angioneurotic edema

Angioedema is a disease characterized by various triggers and an increasing prevalence. The great Greek physician Hippocrates (377-460 BC), considered one of the most outstanding figures in the history of medicine and "Father of the Western Medicine," already used the term oídēma to describe „swelling of organs“. It took many centuries later until the first description of angioedema as a distinct medical entity was minted by Quinсke in 1882. The contemporary perception, defines аngioedema as a transient swelling of the skin or submucosal surface due to increased vascular permeability of small venules. The overlying skin may be normal or mildly erythematous. Over time, many causes and factors that cause or trigger angioedema have been discussed. There are two main types of angioedema without urticarial (histaminergic and nonhistaminergic) as the role of histamine in the pathogenesis of the disease is crucial. Angioedema is a frequent clinical condition that sometimes can be life-threatening. Different types of angioedema can be challenging to distinguish clinically. However, establishing a correct diagnosis is critical as different forms of angioedema require distinct treatment approaches. Implementation of contemporary differential diagnostic approaches could be a prerequisite for an accurate diagnosis, which secures appropriate management strategies are possible.

Quincke’s disease: a case report

Background Isolated angioneurotic edema of the uvula is termed Quincke’s disease. It is a rare clinical disorder of acute onset with few known causes. It may be encountered in any emergency setup and must be dealt with rapidly and with utmost vigilance for prevention of progression and complications. Case presentation A young adult, 3 months post Frey’s procedure for chronic pancreatitis, presented with acute onset throat discomfort and gagging progressive over 8 h. Examination showed isolated edematous hyperemic uvula with normal oropharyngeal structures and adequate airway. Prompt antihistaminic and corticosteroid therapy caused relief of symptoms over 2 h with no recurrences. Conclusion Any symptom suggesting orofacial edema must not be trivialized. Awareness about this rare but acute condition, even in the background of unknown etiology, in all medical personnel is essential. Early diagnosis with appropriate management can prevent life-threatening airway obstruction and hypoxemia.

CURRENT TRENDS IN THE PREVALENCE OF FOOD HYPERSENSITIVITY IN CHILDREN OF THE KYIV REGION

The aim of the study. Due to the growing prevalence of food hypersensitivity worldwide and the number of dangerous food reactions, the aim was to determine the current prevalence of food hypersensitivity symptoms among children in the Kyiv region, Ukraine. Methods. Total of 7106 children were surveyed: Group I (4 months – 5 years) had 1787 children, Group II (6–7 years) – 2080 children, Group ІІI (13–14 years) – 1909 children, Group ІV (15–17 years) – 1330 children. Results. The prevalence of food hypersensitivity among the surveyed children is 26.5 % (95 % CI: 25.5–27.6) – this is 1888 children out of 7106. Highest prevalence of food hypersensitivity (FHS) is observed in the Group I, which is 32 % (95 % CI: 29.8–34.2) – 571/1787, Groups II – 27.2 % (95 % CI: 25.7–29.8) – 566/2080, Groups III – 27.7 % (95 % CI: 14.7–18.0) – 529/1909, in the Group IV – 16.7 % (95 % CI: 25.3-29.2) – 222/1330). FHS in children is most often manifested by skin symptoms, followed by gastrointestinal manifestations, and acute urticaria/angioneurotic edema is at the third place. Respiratory manifestations possess 5 % in the structure of FHS symptoms. An anaphylactic reaction was observed in 4 children out of 7106 respondents. Conclusions. The prevalence of food hypersensitivity among Ukrainian children is high. There is an increase in the prevalence of urticaria/angioedema in preschool children.

DECRIPTION OF A HEREDITARY ANGIONEUROTIC EDEMA FORM IN A CHILD

Congenital deficiency of the C1-component inhibitor of the complement cascade, or hereditary angioneurotic edema, is a rare autosomal dominant disease due to a mutation in the human C1-esterase inhibitor. Caused by C1 deficiency unregulated cleavage of high molecular weight plasma kininogen results in excess production of a mediator with vasodilating action – bradykinin. Hereditary type 1 angioedema develops as a result of C1 inhibitor deficiency, while type 2 is caused by decreased C1 inhibitor activity. The disease manifests itself in childhood or adolescence as recurrent episodes of edema in the skin, subcutaneous fiber and mucous membranes. Localization of edemas in the submucous layer of the larynx represents a threat to life, which can lead to the development of laryngostenosis and acute respiratory failure. The article describes a case of hereditary angioneurotic edema in a girl, which manifested in early childhood. There were no great difficulties in diagnosing the disease, as patients with this pathology were already identified earlier in the family. A significant reduction in C1-inhibitor content was found in the patient, which made it possible to clarify the type of hereditary angioneurotic edema and to categorize it to type 1. A synthetic antifibrinolytic agent with the ability to block kinins and angioneurotic edemas was successfully used in the treatment and prevention of swelling attacks in the patient. The analysis of the case shows that hereditary angioneurotic edema remains a problem difficult enough for a practical doctor requiring careful history taking, assessment of disease development dynamics and performing a laboratory – genetic examination. In most cases, only differential diagnostics can give the opportunity to suspect the dangerous pathology in the patient in a timely manner which requires immediate hospitalization and providing aid adequate to the disease.

Allergic Contact Dermatitis, Angioneurotic Edema and Conjunctivitis in a Patient with Autoimmune Thrombocytopenia – A Clinical Case

Allergic contact dermatitis (ACD) is common in clinical practice, but the etiology of this disease is quite varied. A leading pathogenetic mechanism is cell-mediated immunity. The combinations of ACD with other allergic and systemic autoimmune diseases are relatively rare, but these conditions are undoubtedly a professional challenge for practitioners. We present a case of ACD combined with other immune-allergic conditions. Etiology and pathogenesis in these cases are not well understood.