Bullous Mastocytosis in a Filipino infant: A case study

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.

Bubbly orange baby: A rare case of diffuse cutaneous mastocytosis, bullous type

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.

A Case of Diffuse Cutaneous Mastocytosis in a Newborn / 대한주산의학회잡지

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.

Diffuse Cutaneous Mastocytosis with Generalized Bullae

We report on a 9-month-old female infant with multiple tense bullae and erosions covering the entire body, including the face, scalp, and trunk. The histopathological examination revealed sub-epidermal bullae with a dense dermal cellular infiltrate. The infiltrate was identified as a collection of mast cells using toluidine blue and Giemsa stains. The direct immunofluorscence was negative. A diagnosis of cutaneous diffuse mastocytosis with generalized bullae was made based on these clinical and histopathological findings. In cases with diffuse cutaneous mastocytosis with generalized bullae, systemic involvement is more frequent and more severe compared to other types of cutaneous mastocytosis. Some lethal outcomes have been reported. This is the first reported case of diffuse cutaneous mastocytosis in the Korean literature.