No abstract available.
Humans ; Mastocytosis

Introduction: Mastocytosis is a disease defined by the proliferation of mast cells in organs, most commonly the skin. It may affect any age group but is usually found in children in the first year of life. We present a case of diffuse cutaneous mastocytosis manifesting in the rare bullous form. Case: A 4-month-old Filipino male presented with multiple bullae on the head, trunk, and extremities after applying chamomile oil. Biopsy of the skin demonstrated numerous mast cells, confirming the diagnosis of bullous mastocytosis. The patient was treated with oral antihistamines and corticosteroids, which was followed by a good response. Conclusion Diagnosis of diffuse cutaneous mastocytosis may be challenging due to its rarity. Proper management requires preventive measures, symptomatic treatment, as well as communication of prognosis with the stakeholders.
Mastocytosis, Cutaneous

No abstract available.
Hypereosinophilic Syndrome* ; Mastocytosis, Systemic*

Humans ; Mastocytosis, Systemic

Telangiectasia macularis eruptiva perstans (TMEP) is a rare disease, previously classified as a variant of cutaneous mastocytosis. While no gold standard of treatment exists, several treatments have been studied. We report a case of a 63-year-old woman who presented with long-standing asymptomatic telangiectatic macules beginning on the upper chest, back, and bilateral arms, with occasional pruritus and no other systemic symptoms. Skin biopsy, along with Giemsa stain, revealed findings consistent with TMEP. The patient underwent testing for serum tryptase level, which was within normal limits. The patient was started on topical steroids for two weeks and antihistamine therapy, with a noted decrease in pruritus but no change in cutaneous lesions. She was then advised to start phototherapy, and subsequently underwent a total of five sessions of narrow-band ultraviolet B phototherapy, after which she noted lightening of the lesions. Due to the COVID pandemic, the patient was shifted to heliotherapy with continued lightening of lesions after two months of thrice weekly sessions. This rare case is supportive of narrow-band ultraviolet B phototherapy and heliotherapy as promising treatment options for cases of TMEP.
mastocytosis ; phototherapy ; heliotherapy

Humans ; Infant, Newborn ; Mastocytosis, Cutaneous

No abstract available.
Flow Cytometry* ; Mastocytosis, Systemic* ; Splenomegaly*

Urticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis in children. It can be diagnosed clinically, based on the appearance of numerous brownish macules and papules that are symmetrically distributed, mostly on the trunk and the extremities. Skin biopsy is helpful in establishing the diagnosis. Treatment options generally include antihistamines and/or topical corticosteroids. In most cases, pediatric UP tends to disappear spontaneously before puberty. We present the case of a 9-month-old male with a history of multiple brownish patches and plaques, which started when he was four months old. He was diagnosed with UP based on clinical and histopathologic findings, and was prescribed oral antihistamines and emollients for symptomatic treatment.
cutaneous mastocytosis ; mast cell degranulation

Telangiectasia macularis eruptiva pestans is one of the mastocytosis. It appears most frequently in adults and only rarely affects young children or infants, unlike other mastocytosis. The clinical diagnosis of telangiectasia macularis eruptive perstans can be difficult because it does not usually present as a typical childhood mast cell disease. We report a case of telagiectasia macularis eruptiva perstans, a rare form of mastocytosis.
Adult ; Child ; Diagnosis ; Humans ; Infant ; Mastocytosis ; Telangiectasis*

BACKGROUND: The presence of mast cells often in increased numbers, has been noted in various cutaneous disorders. Recent studies suggest that mast cells are of primary importance in these conditions and their presence does not merely represent a second event. Previous reports of mast cell enumeration in normal and pathologic tissues have revealed inconsistent results, reflecting technical difficulties in quantitation. OBJECTIVE: The purpose of our study is to develop a new approach for quantifying mast cell in tissue section that is accurate, reproducible and avoids problems caused by the variation in distribution of these cells in the tissue. METHODS: Mast. cells were quantitated in eight cases of mastocytosis and in the flank and back skin of three normal human controls in toluidine blue stain using a computerized image analysis system. RESULTS: Macular and nodular mastocytosis patients had from twenty-fold to nearly a 100-fold greater mast cell content than was observed in normal skin. CONCLUSION: Image analysis in conjunction with toluidine blue stain offers a single and accurate method for mast cell quantification. This technique also provides a practical and important new diagnostic tool for establishing the presence of increased lesional skin mast cells in patients with mastocytosis.
Humans ; Mast Cells* ; Mastocytosis ; Skin ; Tolonium Chloride