Bullous Mastocytosis : A Rare Variant of Diffuse Cutaneous Mastocytosis
Keywords:
Cutaneous mastocytosis, Systemic mastocytosis, Mast cells, Bullous mastocytosisAbstract
Mastocytosis is a rare myeloproliferative disease characterized by excessive proliferation and accumulation of mast cells in various tissues. In cutaneous mastocytosis (CM) mast cells infiltration is limited to the skin, whereas systemic mastocytosis (SM) involves internal organs, particularly bone marrow, lymph nodes, liver and spleen. We reported a case of a four year old girl who presented in dermatology OPD of Mayo hospital Lahore with 1 month history of vesiculobullous eruption that gradually evolve to form multiple erythematous to hyperpigmented papules and plaques with doughy consistency and few papular lesions on forehead as well associated with intense itching with few systemic menifestations. Skin biopsy was performed that showed normal epidermis, sub epidermal blister and papillary dermis contain mixed inflammatory infiltrate composed of mast cells, eosinophils and neutrophils suggestive of "Bullous Mastocytosis" Few such cases have been reported previously. It could be concluded that paediatricians, dermatologists and haematologist should remain aware of varied forms of CM because of its rarity and the distinctive management of each individual case.References
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