Abstract
Iris mammillations are rarely described, distinctive villiform protuberances that can cover the iris. In the majority of reported cases they are unilateral and sporadic, and are seen in association with oculodermal melanosis. In past literature and current clinical practice they are frequently confused with the iris nodules seen in neurofibromatosis type 1. Their clinical significance is not established, although it has been suggested that iris mammillations may be an external sign of ocular hypertension or intraocular malignancy. We report a series of 9 patients between the ages of 3 and 28 years with iris mammillations. The mammillations appear as regularly spaced, deep brown, smooth, conical elevations on the iris, of uniform height or increasing in height as the pupil margin is approached. They often overlie a naevus or an exceptionally deeply pigmented iris, such as that seen in melanosis oculi. One case had an associated ciliary body mass. They tend to occur in more highly pigmented ethnic groups and can be dominantly inherited. Iris mammillations may occur in association with systemic conditions including phakomatosis pigmentovascularis type IIb and neurofibromatosis type 1 when they may even coexist with iris hamartomas.
Similar content being viewed by others
Article PDF
References
Coats G . Unilateral diffuse melanosis of the uvea, with small elevations on the surface of the iris. Trans Ophthalmol Soc UK 1912; 32: 164–71.
Fleischer B . Zwei Fälle von einseitiger Melanosis der Skiera, der Iris und des Augenhintergrundes mit warzenförmigen, kleinen Erhebungen an der Iris vorderfläche. Klin Monatsbl Augenheilkd 1913; 51: 170–4.
Waardenburg PJ . Heterochromie en melanosis. Ned T Geneesk 1918; 62: 1453–5.
Friedenwald H, Friedenwald JS . Melanosis of the lids, conjunctiva, and sciera, with wart-like growths on the iris. Arch Ophthalmol 1925; 54: 51–4.
François J . La mélanose congénitale et bénigne de l'oeil Arch d'Ophtalmol 1934; 51: 689–718, 776-92.
François J . Heredity in ophthalmology. St Louis: CV Mosby, 1961: 532–4.
Joondeph BC, Goldberg MF . Familial iris melanosis: a misnomer? Br J Ophthalmol 1989; 73: 289–93.
Traboulsi El, Maumenee IH . Bilateral melanosis of the iris. Am J Ophthalmol 1987; 103: 115–6.
Gilliam AC, Ragge NK, Perez MI, Bolognia JL . Phakomatosis pigmentovascularis type IIb with iris mammillations. Arch Dermatol 1993; 129: 340–2.
Hulke JW . A series of cases of carcinoma of the eyeball. R Lond Ophthal Hosp Rep 1861; 3: 279–86.
Leibreich R . Ophthalmosk. Atlas. In: Pigmentierter Optikus bei sogenannter Cyanosis bulbi. 1863: plate 12, figure 3.
Manz. Melanosis oculi. In: Graefe A, Saemisch T. Handbuch der gesammten Augenheilkunde II. Leipzig, 1876: 117–9.
Collins ET . Case of a patient with congenital excess of pigment in the uveal tract and pigmentation of the sclerotic in one eye, which late in life became the seat of a melanotic sarcoma. Trans Ophthalmol Soc UK 1894; 14: 197–9.
Terson MA . Mélanochromie congénitale de la sclérotique. Ann d'oculist 1904; 131: 127–8.
Hirschberg J . Ueber die angeborene Pigmentirung der Sciera und ihre pathogenetische Bedeutung. Graefes Arch Ophthalmol 1883; 29: 1–12.
Ragge NK, Falk RE, Cohen WE, Murphree AL . Images of Lisch nodules across the spectrum. Eye 1993; 7: 95–101.
Bourquin J . Die angeborene Melanose des Auges. Z Augenheilkd 1917; 37: 129–74, 294-311.
Mann I . Developmental abnormalities of the eye, 2nd ed. London: William Clowes and Sons, 1957: 290–1.
Collins ET . Unilateral melanosis of the uvea and sciera, with numerous small elevations on the surface of the iris. Trans Ophthalmol Soc UK 1912; 32: 171–3.
Lisch K . Ueber Beteiligung der Augen, insbesondere das Vorkommen von Irisknötchen bei der Neurofibromatose (Recklinghausen). Z Augenheilkd 1937; 93: 137–43.
Blodi FC . Ocular melanocytosis and melanoma. Am J Ophthalmol 1975; 80: 389–95.
Jay B . Malignant melanoma of the orbit in a case of oculodermal melanosis. Br J Ophthalmol 1965; 49: 359–63.
Font RL, Reynolds AM, Zimmerman LE . Diffuse malignant melanoma of the iris in the nevus of Ota. Arch Ophthalmol 1967; 77: 513–8.
Kiewe P, Maneff I . Mélanose bilatérale congénitale et familiale de l'oeil et de la face. Contribution au probleme de la consanguinité. Ann Oculistique 1935; 172: 723–8.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ragge, N., Acheson, J. & Murphree, A. Iris mammillations: Significance and associations. Eye 10, 86–91 (1996). https://doi.org/10.1038/eye.1996.13
Issue Date:
DOI: https://doi.org/10.1038/eye.1996.13