A 57-year-old woman had developed pruritic, brownish-red, maculopapular rash on her legs at age 40 (positive Darier's sign) that persisted thereafter, with variable intensity. A physician had suspected urticaria pigmentosa at the time, but no treatment was recommended. At age 51, she had sustained femoral neck fracture and four vertebral body fractures, without any antecedent trauma and without any known risk factors. Osteoporosis was diagnosed and treated with strontium ranelate. At age 57, the patient had fractures of all vertebral bodies from T6 to L5 and threefold elevation of the serum tryptase level. Laboratory findings, skin and bone marrow biopsy fulfilled the criteria for systemic mastocytosis (major: multifocal mast-cell infiltrates, minor: fusiform mast cells, expression of CD25 and CD2, KIT mutation, serum tryptase level). The patient was initially treated with zoledronate 4 mg every six months. At one year, no further compression fractures were seen.
Systemic mastocytosis can cause cytokine-mediated stimulation of osteoclasts, leading to osteoporosis. Osteoporosis of unclear origin (in young patient without any known risk factors) should be thoroughly evaluated, and systemic mastocytosis should be considered in the differential diagnosis.
Dr. med. Martin Gehlen, Dr. Ana Doina Lazarescu, Christian Hinz , Clinic Der Fürstenhof Specialist Clinic for Rheumatology, Orthopedics and Osteology, Clinical Osteological Focus Center and Osteological Research Center DVO, Bad Pyrmont, gehlen@staatsbad-pyrmont.de
Conflict of interest statement: The authors state that they have no conflict of interest.
Translated from the original German by Ethan Taub, MD
Cite this as : Gehlen M, Lazarescu AD, Hinz C: 12 vertebral-body fractures and simultaneous skin changes. Dtsch Arztebl Int 2018; 115: 418 . DOI: 10.3238 / arztebl.2018.0418
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