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

Humans ; Infant, Newborn ; Mastocytosis, Cutaneous

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

Mastocytosis is an uncommon disease characterized by an abnormal accumulation of mast cells in a variety of tissues, most commonly, in the skin. It may mimic other conditions because of numerous signs and symptoms produced by mast cell mediators. We report a case of an unusual manifestation of cutaneous mastocytosis in a 7-year-old girl who had hypertrophy and periodic swelling of the left labia majora with urticaria pigmentosa.
Child ; Humans ; Hydrazines ; Hypertrophy ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Skin ; Urticaria Pigmentosa

Diffuse cutaneous mastocytosis (DCM) is a rare variant of mast cell disease with widespread erythema and is clinically apparent in early infancy. We report the case of a 1-day-old female neonate who presented with diffuse flush, pruritus, and extensive blistering. DCM was diagnosed by immunohistochemical staining with anti-CD117, which revealed mast cell infiltration. DCM is a severe and heterogeneous cutaneous disease, and is associated with mast cell mediator-related symptoms and risk of anaphylactic shock. We describe this case and provide the first literature review of neonatal onset DCM in Korea.
Anaphylaxis ; Blister ; Erythema ; Female ; Humans ; Infant, Newborn* ; Korea ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous* ; Pruritus

Urticaria pigmentosa (UP) is the most common variant of cutaneous mastocytosis. Primarily a disease of childhood, in over one-half of the cases onset is before 2 years of age, and in 90%, the disease is confined to the skin. UP precedes the diagnosis of systemic mastocytosis and is more common in adults than in children. Therefore, systemic examination should be performed in patients with UP. We report a case of adult-onset UP with systemic involvement.
Adult ; Child ; Humans ; Mastocytosis, Cutaneous ; Mastocytosis, Systemic ; Skin ; Urticaria ; Urticaria Pigmentosa

Mastocytosis is a heterogenous group of disorders characterized by the proliferation and accumulation of mast cells in a variety of tissues, most often skin. Urticaria pigmentosa is the most common type of cutaneous mastocytosis in both adults and children. However, the clinical manifestation of adult-onset urticaria pigmentosa may have some differences such as smaller macules, more pruritic symptoms, and frequent association with systemic symptoms. We report a case of adult-onset urticaria pigmentosa in a 51-year-old woman which we compare to those cases reported in the literatures.
Adult ; Child ; Female ; Humans ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Middle Aged ; Skin ; Urticaria Pigmentosa* ; Urticaria*

Mastocytosis is a heterogenous group of disorders characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Urticaria pigmentosa represents the most common form of cutaneous mastocytosis in children and adults. We report a case of adult-onset urticaria pigmentosa in a 53-year-old man. The patient had pruritic multiple scattered well-demarcated brownish macules and papules on whole body for 4 years. Histopathologic examination showed diffuse and perivascular infiltration of lymphocytes and cuboidal mast cells in the upper dermis. Any sign or symptom of systemic involvement was not detected.
Adult ; Child ; Dermis ; Humans ; Lymphocytes ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Middle Aged ; Urticaria Pigmentosa* ; Urticaria*

Mastocytosis is an uncommon, sporadic, heterogenous illness resulting from hyperplasia of mast cells. Diffuse cutaneous mastocytosis is the rarest subtype of mastocytosis affecting children, with bullous mastocytosis being its least common variety. Systemic manifestations like nausea, vomiting, bone pain, diarrhea, and central nervous system abnormalities are less common in children than adults. We report a four-month old male who presented with a two-month history of generalized yellowish to tan macules, papules and plaques with peau d'orange texture, with some blisters and erosions on the back, abdomen and scalp. Darier's sign was positive. Baseline laboratory workup were negative for systemic involvement. CD117 and Giemsa staining were positive for mast cells. Based on the clinical findings and histopathologic results, a diagnosis of bullous mastocytosis was made. Treatment included ketotifen drops, mupirocin cream and cetirizine drops, which resulted in flattening of most lesions and resolution of blisters and erosions.

Human ; Male ; Infant ; Blister ; Cetirizine ; Diarrhea ; Hyperplasia ; Ketotifen ; Mast Cells ; Mastocytosis ; Mastocytosis, Cutaneous ; Mupirocin ; Nausea ; Vomiting

Seoul, Korea Cutaneous mastocytosis is primarily a disease of children and its onset in adults life is rare. When it develops in adult, lesions appear as urticaria pigmentosa in most cases, and rarely as telangiectasia macularis eruptiva perstans. We report herein a case of cutaneous mastocytosis which appeared for the first time around the age of 40 with mixed or intermediate form of urticaria pigmentosa and telangiectasia macularis eruptiva perstans clinically, and histologically.
Adult ; Child ; Humans ; Korea ; Mastocytosis, Cutaneous* ; Seoul ; Telangiectasis ; Urticaria Pigmentosa