Biomedical subjects
Publications and source records attributed to Alfons L Krol.
An 11-month-old infant girl presented with right-sided features of aplasia cutis congenita of the scalp, unilateral epibulbar dermoids, eccentric pupil, coloboma of the right upper eyelid, and depigmentation of the fundus surrounding the right optic nerve. These findings were similar to the oculoectodermal syndrome reported by other clinicians and researchers.
Incontinentia Pigmenti is an X-linked dominant neurocutaneous disorder with central nervous system manifestations in 30% of cases, including seizures and mental retardation. Ischemic or hemorrhagic cerebrovascular accidents have been reported rarely in incontinentia pigmenti. Chart review and literature search was performed following identification of the index case. We describe a patient with incontinentia pigmenti who developed bilateral cerebrovascular accidents in the neonatal period, with resultant severe neurologic sequelae. This is the second reported case of bilateral cerebrovascular accidents in a patient with incontinentia pigmenti. This finding may be secondary to cerebrovascular anomalies, similar to those observed in the retina. Recognition of cerebrovascular accidents as a complication of incontinentia pigmenti will hopefully lead to earlier recognition and treatment.
BACKGROUND: Granuloma gluteale infantum is a skin disorder of controversial etiology manifested clinically by oval reddish-purple granulomatous nodules on the gluteal surfaces and groin areas of infants. Similar granulomas are noted in adults and the elderly and are referred to as granuloma gluteale adultorum and diaper area granuloma of the aged, respectively. Occlusion from diapers, paper napkins, plastic pants, detergents, starch, powder, halogenated steroids, candidal infection, and urine and feces are postulated as possible etiologies. OBJECTIVE: We report a case of a 40-year-old woman presenting with a 3-year history of multiple, painful, closely set, red-purple, oval, smooth, firm papules and nodules in the genitocrural area. The development of the lesions was associated with prolonged use of topical benzocaine. Histology of the lesions was consistent with granuloma gluteale infantum. Gram stain and culture of representative tissue did not demonstrate bacterial or fungal organisms. The lesions significantly improved with discontinuation of topical benzocaine. Patch testing of skin to determine allergic contact hypersensitivity to benzocaine was negative. CONCLUSION: We propose that topical benzocaine preparations may play a role in the pathogenesis of granuloma gluteale adultorum, independent of contact sensitization to benzocaine.