Solitary cutaneous hemorrhagic bullous mastocytoma: A rare entity

Solitary cutaneous mastocytoma, a clinical type of cutaneous mastocytosis, may present from birth itself as a macule, plaque, nodule, or bulla. It may be associated with pruritus, flushing attacks, or convulsions. The diagnosis of a solitary mastocytoma is made by the presence of a characteristic skin lesion and confirmed by typical histopathological features of mast cell clusters in dermis and subcutaneous tissues, metachromatic staining of mast cell granules with toluidine blue or giemsa staining, and immunohistochemical mast cell marker c-kit/ CD-117 staining. We report a 4-month-old child who presented with a hemorrhagic bulla of left knee. Skin biopsy and immunohistochemistry confirmed the clinical diagnosis of solitary cutaneous mastocytoma. We did not come across any previous report of solitary cutaneous mastocytoma presenting as hemorrhagic bulla.

Solitary Mastocytoma in Children: Case Report

Solitary mastocytoma is the most common form of mastocytosis in children. The main symptom is pruritus. The treatment is symptomatic and it’s based on Topical corticosteroids. The prognosis is good with possibility of spontaneous regression at adulthood. This case report is about a 7-month-old infant that presented with an erythromatous papular lesion on the left temple. The patient responded well to topical steroids and antihistamines.

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

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.

Primary Solitary Mastocytoma of the Lung: A Case Report

Background Extracutaneous mastocytoma is really rare. Although only four cases of extracutaneous mastocytoma in lung have been reported in the literature to date, the cellular composition of mast cell tumors has not yet been confirmed. Here we report a new case of primary solitary mastocytoma and try to discuss its cellular composition. Case report: A 46-year-old woman, who has been having bronchial asthma for 3 years, had been found a nodules in the left lower lobe of lung by computed tomography (CT) scan because of chest pain, distress, and pain in the left thoracic region of the back for 10 days. Then the left inferior pulmonectomy and lymphadenectomy were performed. We confirmed the presence of mast cells in the resected tumor by immunohistology (i.e., strong CD117 and CD45 staining and positivity for tryptase) and toluidine blue staining. Additionally, our findings suggested that solitary mast cell tumors was consisted of mature mast cells and immature mast cells with features of epithelial cells. A diagnosis of solitary mastocytoma of the lung was suggested. By now, the patient’s overall health was normal. No signs of recurrence was found. Symptoms of bronchial asthma were significantly alleviated as well. Conclusion Solitary mastocytoma of the lung is rare, but operative and relative drug therapy can release symptoms effectively. Solitary mastocytoma may be consisted of mature mast cells and immature mast cells. But the reason why the tumor exhibited epithelial immunophenotype still needs further study.