Fragility fractures as the initial manifestation of indolent systemic mastocytosis

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...

Delayed Diagnosis of Adult Indolent Systemic Mastocytosis

Systemic mastocytosis (SM) is a rare, heterogeneous disorder characterized by infiltration and accumulation of mast cells within multiple organs, most commonly the skin. Given the rarity of the disease and the fact that many of its symptoms are shared by more common disorders, a diagnosis may be delayed or hindered. These patients have an elevated risk of developing potentially life-threatening...

Indolent systemic mastocytosis in a patient with ileocolitis

Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor cell. It is a rare disorder with unknown incidence in Greece, with an estimate of 2 cases per year in Great Britain. We present a case of an asymptomatic, 72-year-old man who was found to have ileocolitis on endoscopy. Histology revealed mast cells in lamina propria >15 HPF and biochemistry showed high levels of seru...

Indolent systemic mastocytosis associated with light chain deposition disease

Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast cells in one or more organ systems. SM in association with plasma cell dyscrasia is very rare. We report a first case of indolent SM (ISM) associated with light chain deposition disease (LCDD) in a kidney biopsy from a 59-year-old female presenting with skin rash, elevated serum creatinine, hematuria and mild protein...

Oral contraceptive-related transverse sinus thrombosis as an initial manifestation of antiphospholipid syndrome in the absence of systemic lupus erythematosus