Systemic mastocytosis: a (slightly) simplified diagnostic approach

Systemic mastocytosis – a diagnostic challenge

Mastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally. Its molecular pathogenesis is incompletely understood. Th...

Systemic mastocytosis involving bone : a diagnostic challenge for the radiologist

Systemic mastocytosis: a rare entity.

An 18 year old female presented with generalized maculopapular rash and headaches. The skin and bone marrow biopsy revealed numerous mast cells which stained metachromatically with giemsa and toludine blue stains and showed positivity with CD68 and CD117 on immunohistochemistry. Her clinical presentation and laboratory findings were consistent with the diagnosis of indolent systemic mastocytosi...

Systemic mastocytosis--a systematic review.

INTRODUCTION The mast cell lives a hidden life, but it is implicated in several physiological reactions. Its ability to react to different stimuli impacts a variety of conditions such as asthma, atopic dermatitis, urticaria and anaphylaxis. It is not until recent decades that the evolution of the cell has been described and its fascinating biology has only recently been depicted. We here give a...

Indolent systemic mastocytosis.

circular macules, less than 1 cm in diameter, located on his trunk and extremities [Figure A]. Wheals and surrounding erythema developed in the lesions after rubbing (positive Darier sign). Laboratory analyses showed mild eosinophilia with an increased total tryptase level of 40 ng/ml. Densitometry was normal. With the strong clinical suspicion of cutaneous mastocytosis, a skin biopsy was perfo...