Corneal surface irregularities and episodic pain in a patient with mucolipidosis IV

Corneal surface irregularities and episodic pain in a patient with mucolipidosis IV

Newman NJ; Starck T; Kenyon KR; Lessell S; Fish I; Kolodny EH

Archives Of Ophthalmology

1990

Child; Humans; Male; Preschool; Cataract Extraction; Conjunctiva/pathology; Cornea/pathology; Epithelium/pathology; Mucolipidoses/pathology; Pain/etiology; Q3 Literature Search; Tears/secretion

Mucolipidosis IV is a lysosomal storage disease characterized by prominent involvement of the corneal epithelium. A 5-year-old boy with mucolipidosis IV experienced recurrent episodes of severe ocular pain, tearing, and ipsilateral facial flushing. This was suggestive of reflex sympathetic dystrophy, a syndrome of pain and sympathetic hyperactivity. The examination revealed marked corneal surface irregularities, corresponding to massive accumulations of intracytoplasmic storage material in the epithelium. Episodic pain in patients with mucolipidosis IV is an important symptom, presumably reflecting the distinctive corneal ultrastructural abnormality in this disease.

1990

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