Generalized eruptive keratoacanthomas of Grzybowski (case report)

В статье представлен краткий обзор литературы о клинических проявлениях, современных методах диагностики и лечения чрезвычайно редкого заболевания – множественной эруптивной кератоакантомы типа Гржебовски. Заболевание характеризуется возникновением сотен или тысяч генерализованных зудящих узелков на коже лица, туловища, конечностей, половых органов, слизистой полости рта и гортани в течение короткого времени. Заболевание развивается в возрасте старше 40 лет, имеет рецидивирующее течение, возможна спонтанная инволюция отдельных элементов с формированием участков депигментации или атрофических рубцов. Авторы приводят случай собственного клинического наблюдения множественной эруптивной кератоакантомы типа Гржебовски у пациента 50 лет, который обратился на консультацию к дерматовенерологу в клинику Уральского НИИ дерматовенерологии и иммунопатологии. Длительность болезни на момент обращения составляла 2,5 года, ее возникновению предшествовало неоднократное посещение больным стран с избыточной инсоляцией, что может рассматриваться в качестве этиологического фактора развития заболевания. Диагноз впервые был заподозрен на консилиуме дерматовенерологов на основании клинической картины заболевания и в дальнейшем верифицирован данными патоморфологического исследования биоптата пораженной кожи. Показана эффективность применения ароматических ретиноидов в лечении множественной эруптивной кератоакантомы типа Гржебовски и приведены побочные эффекты, которые развились у больного при самостоятельном увеличении дозы ацитретина до 70 мг/сутки. В статье констатируется чрезвычайно редкая встречаемость данного заболевания, о чем свидетельствует приведенный клинический случай, диагностируемый впервые более чем за 90-летнюю историю существования института. Подчеркивается важность консолидации клинического опыта нескольких ведущих дерматовенерологов для диагностики множественной эруптивной кератоакантомы типа Гржебовски. The article presents a brief review of the literature about clinical manifestations, modern methods of diagnosticsand treatment of an extremely rare disease – generalized eruptive keratoacanthomas of Grzybowski. The disease is characterized by the appearance of hundreds or thousands of generalized itchy nodules on the skin of the face, trunk, limbs, genitals, oral mucosa and larynx within a short time. The disease develops at the age of over 40, has a recurrent course, spontaneous involution of individual elements with the formation of areas of depigmentation or atrophic scars is possible. The authors describe a case of their own clinical care of generalized eruptive keratoacanthomas of Grzybowski in a 50-year-old patient who consulted a dermatovenerologist at the clinic of the Ural Research Institute of Dermatovenerology and Immunopathology.The duration of the disease at the time of visit to a doctor was 2,5 years, its occurrence was preceded by repeated visits to countries with excessive insolation, which can be considered as an etiological factor in the development of the disease. The diagnosis was first suspected at a boarddermatovenerologists based on the clinical picture of the disease and subsequently verified by the data of a pathomorphological examination of the biopsy of the affected skin.The effectiveness of the use of aromatic retinoids in the treatment of generalized eruptive keratoacanthomas of Grzybowski is shown and the side effects that developed in the patient with an independent increase in the dose of acitretin to 70 mg/day are given. The article states the extremely rare occurrence of this disease, as evidenced by the above clinical case, diagnosed for the first time in more than 90 years of the institute's existence. The importance of consolidating the clinical experience of several leading dermatovenerologists for the diagnostics of generalized eruptive keratoacanthomas of Grzybowski is emphasized.

Hepatic hemangioma: A case of spontaneous involution

Hepatic hemangiomas are benign, well-circumscribed tumors often found incidentally on imaging. They are estimated to be prevalent in 0.4-20% of the population. While usually solitary lesions, these tumors tend to not cause any issues for patients. However, when they grow to be larger than 5 cm, there can be associated symptoms including pain, nausea, or early satiety from compression on adjacent structures. There can be more rare, serious complications including hemobilia, rupture, coagulopathy, or high output heart failure seen in tumors that are typically larger than 10 cm. While small hepatic hemangiomas (<5 cm) that do not cause symptoms do not need to be followed, ones that are greater than 5 cm should be followed at least once to ensure that they are stable in size, or more regularly if they are growing. Symptomatic large hepatic hemangiomas can be intervened on with a number of procedures if needed. These include hepatectomy, enucleation, transcatheter arterial embolization, radiofrequency ablation, and in rare cases, liver transplant.

Infantile haemangioma: 5-year experience at the vascular anomaly clinic

Background Infantile haemangiomas (IH) represent a common benign vascular tumour affecting the paediatric population. Infantile haemangiomas are characterised by a natural history differentiating it from other vascular anomalies. After a transient proliferative phase in early infancy, the tumour passes through a plateau phase before going into spontaneous involution. In this report, we tried to share our experience over the last 5 years in managing cases presenting with IH at a specialised vascular anomaly clinic. Main body of abstract This report included cases of IH who were attending the vascular anomaly clinic during the period 2015 through 2019. Data of all patients attending the clinic were retrospectively examined. Files of 103 cases with IH were available for review. The diagnosis of IH was usually straight forward owing to the typical history and characteristic findings at clinical examination. A significant female predominance was noticed. Generally, IH were more common in the head and neck region (70%). Active intervention was necessary in specific situations (eye occlusion, airway involvement, large lesions with skin ulcerations). Whenever intervention proved to be necessary, propranolol was chosen as the first line of treatment with a favourable response detected in about 90% of cases. Surgery was still a valid option (6%) for lesions amenable to resection; however, we must put in consideration that most lesions will spontaneously regress. Conclusion Infantile haemangiomas are common benign vascular tumours of infancy with relatively few complications. Cosmesis is a major concern especially for lesions affecting the face. Propranolol can induce tumour regression in most cases, and generally, a favourable outcome can be anticipated.

Imaging findings of spontaneous thymic hemorrhage in infancy: Case report and literature review

The authors report imaging findings of spontaneous thymic hemorrhage, which is a rare entity in infancy, in a 4-month-old boy with congenital factor VII deficiency presented with acute respiratory distress and anemia. Widening of superior mediastinum and left pleural effusion were detected on the chest radiograph. Ultrasound and computed tomography of the chest revealed diffusely enlarged thymus with heterogeneous parenchyma from poorly-defined areas of altered echogenicity or attenuation. Vascular flow was depicted within the abnormal thymus in color-mode sonography. The spontaneous involution of thymic abnormality seen on serial ultrasonography confirmed the diagnosis of thymic hemorrhage, resulting in avoidance of further unnecessary imaging or invasive procedure.

Nonlife-Threatening Sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology. The outcome is quite variable and is mainly related to persistent inflammatory processes and the development of fibrosis. Many prognostic factors have been described, but the disease evolution is not yet entirely known. The nonthreatening course is characterized by spontaneous involution or stability after treatment withdrawal. Löfgren's syndrome is a subset within the spectrum of sarcoidosis phenotypes, composed of acute onset of fever, bilateral hilar lymphadenopathy, erythema nodosum and/or bilateral ankle periarticular inflammation/arthritis, specifically characterized by a self-limiting disease course. In contrast, advanced fibrotic sarcoidosis with pulmonary hypertension phenotype is correlated with a poor prognosis. Further studies are necessary to detail phenotypes to better understand the mechanisms of the disease and plan future clinical therapeutic studies.

Is There More Than One Road to Nevus-Associated Melanoma?

The association of melanoma with a preexisting nevus is still a debated subject. Histopathological data support an associated nevus in approximately 30% of all excised melanomas. The annual risk of an individual melanocytic nevus becoming malignant is extremely low and has been estimated to be approximately 0.0005% (or less than 1 in 200,000) before the age of 40 years, to 0.003% (1 in 33,000) in patients older than 60 years. Current understanding, based on the noticeable, small, truly congenital nevi and nevi acquired early in life, is that the first develops before puberty, presents with a dermoscopic globular pattern, and persists for the lifetime, becoming later a dermal nevus in the adult. In contrast, acquired melanocytic nevi develop mostly at puberty and usually undergo spontaneous involution after the fifth decade of life. The purpose of this review is to analyze the data of the literature and to propose, on the basis of epidemiological and clinical-dermoscopic characteristics, a new model of melanogenesis of nevus-associated melanoma.

Optic nerve head retinal artery macroaneurysm: Report of a case

A 61-year-old elderly male, hypertensive patient presented to the retina clinic with sudden drop in vision in the left eye for 6 days. His best-corrected visual acuity at presentation was counting fingers close to face. Fundus examination of the left eye revealed the presence of subretinal and preretinal haemorrhage at the macula along with hypertensive retinopathy changes in both eyes. Fluorescein angiography was done, which showed a retinal artery macroaneurysm at the optic nerve head. Optical coherence tomogram through the optic nerve head also confirmed the presence of retinal artery macroaneurysm. The patient was treated with injection of 0.4 cc of 100% C3F8 to displace the blood off the macula. At final follow-up visit at 2 months post treatment, his vision improved to 6/12, N8. Fundus examination showed a small residual altered blood nasal to the fovea. No treatment was however done to the retinal artery macroaneurysm due to its atypical location and chance of spontaneous involution. In conclusion, retinal artery macroaneurysm at the optic disc is extremely uncommon. Identification of the retinal artery macroaneurysm lesion is more difficult in glaucoma patients due to the large and deep optic cup. Fluorescein angiography remains the main investigative modality to confirm the diagnosis. Spontaneous involution still remains the mainstay of treatment in optic disc retinal artery macroaneurysm.