Bullous mastocytosis in a 3-month-old infant

Asati, Dinesh P; Tiwari, Anurag

doi:10.4103/2229-5178.142520

Cited by 7 publications

(19 citation statements)

References 14 publications

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“…1,12,13 Treatment options are symptomatic and aim to reduce mast cell degranulation. 2,14 This patient took a noncompetitive H1-antihistamine and mast cell stabilizer (ketotifen), a histamine-1 receptor antagonist (cetirizine), and a histamine-2 receptor antagonist (ranitidine). The dose of these medications was increased in this patient because of persistent pruritus and flushing symptoms.…”

Section: Discussionmentioning

confidence: 99%

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

Hudson

Finlayson

2016

J Cutan Med Surg

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Section: Discussionmentioning

confidence: 99%

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

Hudson

Finlayson

2016

J Cutan Med Surg

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“…Согласно обобщенным статистическим данным, на каждые 10 тыс. пациентов, обратившихся к дерматологу, приходится 2-3 случая мастоцитоза [4,5]. В России кожные формы мастоцитоза составляют, по разным данным, от 0,1 до 0,8% всех дерматологических диагнозов [6].…”

Section: обоснованиеunclassified

“…Различные патологические варианты гена c-KIT, в т. ч. изменения нуклеотидной последовательности в кодоне 816 (D816V), были выявлены у 86% детей с мастоцитозом [9]. Результаты исследований подтверждают также и клональную при-роду детского мастоцитоза, несмотря на возможность спонтанного регресса [5]. Считается, что наличие активирующих вариантов гена KIT необходимо для развития мастоцитоза, а фенотипическое разнообразие заболевания может быть обусловлено их комбинацией с другими генетическими вариантами [5,8,9].…”

Section: обоснованиеunclassified

“…В 70-75% случаев кожный мастоцитоз дебютирует у детей в возрасте от 1 мес до 1 года, но может начинаться и в более старшем возрасте [5,12,13]. Полагают, что благоприятный исход заболевания характерен для детей с типичным течением болезни и при возникновении его в возрасте до 2 лет [3,7].…”

Section: обоснованиеunclassified

“…Вместе с тем существуют указания на трансформацию кожных форм мастоцитоза в системные при отсутствии спонтанного регресса высыпаний, предположительно, связанные с усовершенствованием и доступностью методов диагностики мастоцитоза, появлением новых триггерных факторов по причине изменения экологической обстановки в мире [1,13]. В настоящий момент имеются единичные работы, касающиеся описания клинических случаев мастоцитоза у детей без анализа динамики течения заболевания [5,7,14]. В зарубежной литературе преимущественно отражены данные о структуре мастоцитоза кожи у детей, оценке тяжести течения и трансформации в системный мастоцитоз [2,3,13].…”

Section: обоснованиеunclassified

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Analysis of Risk Factors Affecting Skin Mastocytosis in Children: Cross-Sectional Study

Касихина

Круглова

Ващенко

et al. 2020

Vopr. sovr. pediatr.

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Background. Skin mastocytosis is rare disease that is diagnosed in most children under the age of 2 years. The date on rash regression dynamics and disease symptoms is not fully presented in the literature.Objective. The aim of the study was to analyze risk factors associated with clinical manifestations and regression time of skin mastocytosis in children.Methods. The study includes data on 28 children aged from 3 months to 12 years who has undergone outpatient care and observation in Moscow Scientific and Research Center of Dermatovenerology and Cosmetology of Moscow City Health Department in the period between January 2016 and November 2019. The data about diagnosis was obtained from medical records.Results. Maculopapular skin mastocytosis (MPSM) was diagnosed in 28.6% of children, solitary mastocytoma — in 71.4%. The analysis of clinical course of skin mastocytosis has shown constantly relapsing process and slow spontaneous rash regression in more than 50% of children. Diffuse skin rash, flushing reactions, persistent skin itching or its combination with hepatomegaly or neurological symptoms were prevalent among MPSM manifestations. Risk factors affecting delayed regression of skin mastocytosis in children with MPSM are: late onset, area of skin lesions, comorbidities, severity of reticular vascular pattern at dermatoscopy. Severity of skin lesions did not affect the tryptase activity. The major risk factor affecting the delayed regression of solitary mastocytoma is rash injury (OR 6.10, 95% CI 3.66–16.73). The severity of reticular vascular pattern in skin mastocytosis foci has varied depending on the severity of skin lesions.Conclusion. Half of all children with skin forms of mastocytosis have delayed rash regression. This causes high concern among parents and violates social adaptation of children. Timely assessment of risk factors alongside with dynamic assessment of the dermatoscopy patterns and tryptase activity are important for implementation of correct follow-up monitoring and management for children. Rash (of any localization) injuries should be avoided to prevent delayed regression of the disease in children with skin forms of mastocytosis.

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Solitary mastocytoma in children: a retrospective analysis of the clinical course and timing of regression

2021

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Introduction. Solitary mastocytoma is a rare clinical variant of cutaneous mastocytosis that makes its debut in infancy and early childhood. Сhanges in clinical manifestations at different ages and the rate of regression of the disease are insufficiently covered in the literature.Aim. To study the clinical manifestations and timing of regression of solitary mastocytoma in children, through retrospective analysis.Materials and methods. We retrospectively reviewed data from 32 children aged 3 months to 9 years who were on outpatient treatment and observation at the State Budgetary Healthcare Institution “Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology of Moscow Health Department” in the period from 2016 to 2020 inclusive. Diagnosis information obtained from medical records.Results and discussion. The average age of disease onset was 2.5 ± 0.7 months. Solitary mastocytoma was observed more often in boys than in girls (1.4:1). In 81.2% of children, solitary mastocytoma clinically regressed before the age of 6 years. Delayed regression of rashes in the age range from 6 to 9 years was observed in 18.8% of children. Late correct diagnosis, lack of timely recommendations for care and treatment, traumatization of elements are factors that slow down the regression of the disease. Dermatoscopic examination can be used for dynamic observation of mastocytoma, since the patterns in the foci of regression differ from mature mastocytomas in the absence of yellow-orange areas. A pronounced brown pigment network on a yellow background can be a sign of the activity of the process and a reason for the appointment of symptomatic therapy.Conclusion. The clinical features of the modern course of solitary mastocytoma are the tendency to multiple rashes, the variety of localization and the long-lasting positivity of the Darier sign. Obviously, clinical monitoring using dermatoscopy and laboratory research methods allows to follow-up the activity of the process and promptly adjust drug treatment. To prevent delayed regression of solitary mastocytoma, traumatization of rashes of any localization should be excluded.

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Bullous mastocytosis in a 3-month-old infant

Cited by 7 publications

References 14 publications

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

Analysis of Risk Factors Affecting Skin Mastocytosis in Children: Cross-Sectional Study

Solitary mastocytoma in children: a retrospective analysis of the clinical course and timing of regression

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