ABSTRACT
Prolidase deficiency and solitary mastocytoma of the eyelid are both exceedingly rare. Prolidase deficiency is an inherited connective tissue disorder that has systemic sequelae, such as intractable skin ulceration, poor wound healing, recurrent infections, and intellectual impairment. Cutaneous mastocytoma is an isolated, aberrant cutaneous aggregation of mast cells. A case of an adult with severe prolidase deficiency who developed cutaneous mastocytoma of the eyelid was presented. To the authors' knowledge, adult-onset solitary mastocytoma of the eyelid has never been reported previously.
Subject(s)
Eyelid Neoplasms/pathology , Mastocytoma, Skin/pathology , Prolidase Deficiency/complications , Eyelid Neoplasms/etiology , Humans , Male , Mastocytoma, Skin/etiology , Middle AgedABSTRACT
We describe a patient with a solitary mastocytoma arising at a site of trauma. The patient was born with the umbilical cord wrapped around her right thigh and subsequently developed a solitary mastocytoma in the exact site and distribution of this injury. The pathogenesis of mast cell proliferation in solitary mastocytoma is not completely understood. Cytokines released after injury, such as stem cell factor, may stimulate the proliferation of mast cells, as well as fibroblasts and melanocytes to form a mastocytoma. Mast cells in a newborn may be more sensitive to stem cell factor in the presence of cytokines released after injury due to an increased density of c-kit receptors. We present our patient and review the literature to support a hypothesis that this condition represents a reactive, and not neoplastic, process.
