Skip to main content

Thank you for visiting You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

Images of Lisch nodules across the spectrum


The presence of melanocytic hamartomas on the iris, known eponymously as Lisch nodules, is highly characteristic of neurofibromatosis type 1 (NF1). Early recognition of NF1 is vital for optimal detection of associated tumours and for genetic counselling of family members. Although the iris nodules are probably not visible at birth, their prevalence in patients with NF1 gradually increases from birth to about 50% of 5-year-olds, 75% of 15-year-olds and 95-100% of adults over the age of 30. Iris hamartomas in NF1 are elevated, pale brown lesions that vary in appearance depending on the underlying colour of the iris. Colour illustrations are shown in this article, enabling the clinician readily to differentiate hamartomas from other iris lesions.


  1. 1

    Lisch K . Ueber Beteiligung der Augen, insbesondere das Vorkommen von Irisknötchen bei der Neurofibromatose (Recklinghausen). Z Augenheilkd 1937;93:137–43.

    Google Scholar 

  2. 2

    Lewis RA, Riccardi VM . von Recklinghausen neurofibromatosis. Incidence of iris hamartomata. Ophthalmology 1981;88:348–54.

    CAS  Article  Google Scholar 

  3. 3

    Treacher Collins E . 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 melan-otic sarcoma. Trans Ophthalmol Soc UK 1894;14:197–9.

    Google Scholar 

  4. 4

    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.

    Google Scholar 

  5. 5

    Fleischer B . Zwei Fälle von einseitiger Melanosis der Skiera, der Iris und des Augenhintergrundes mit warzenför-migen, kleinen Erhebungen an der Iris vorderfläche. Klin Monatsbl Augenheilkd 1913;51:170–4.

    Google Scholar 

  6. 6

    Friedenwald H, Friedenwald JS . Melanosis of the lids, conjunctiva and sciera, with wartlike growths on the iris. Arch Ophthalmol 1925;54:51–4.

    Google Scholar 

  7. 7

    Snell S, Treacher Collins E . Plexiform neuroma (elephantiasis neuromatosis) of temporal region, orbit, eyelid and eyeball. Notes of three cases. Trans Ophthalmol Soc UK 1903;23:157–77.

    Google Scholar 

  8. 8

    Fuchs E . Naevus pigmentosus und Naevus vasculosus der Iris. Graefes Arch Clin Exp Ophthalmol 1913;86:155–169.

    Article  Google Scholar 

  9. 9

    Waardenburg PJ . Heterochromie en melanosis. Ned Tijdschr Geneeskd 1918;62:1453–5.

    Google Scholar 

  10. 10

    Goldstein I, Wexler D . Melanosis uveae and melanoma of the iris in neurofibromatosis (Recklinghausen). Arch Ophthalmol 1930;3:288–96.

    Article  Google Scholar 

  11. 11

    Gabriélidès C . Fibroneurome orbito-temporo-palpébral (Maladie de Recklinghausen). Ann Ocul 1931;168:187–206.

    Google Scholar 

  12. 12

    Kurz J . Tumorbefund in der Iris bei Neurofibromatose. Ofthalm. Sborn 1932;7:294-5. (Abstract in Z Gesamte Ophthalmol 1933;28:566.)

    Google Scholar 

  13. 13

    Van der Hoeve J . The Doyne Memorial Lecture. Eye symptoms in phakomatoses. Trans Ophthalmol Soc UK 1932;52:380–401.

    Google Scholar 

  14. 14

    Sakurai T . Multiple neurofibroma patient showing multiple flecks on the anterior surface of the iris. Acta Soc Ophthalmol Jpn 1935;39:87–93.

    Google Scholar 

  15. 15

    Perry HD, Font RL . Iris nodules in von Recklinghausen's neurofibromatosis. Electron microscopic confirmation of their melanocytic origin. Arch Ophthalmol 1982;100:1635–40.

    CAS  Article  Google Scholar 

  16. 16

    Williamson TH, Garner A, Moore AT . Structure of Lisch nodules in neurofibromatosis type 1. Ophthalmic Paediatr Genet 1991;12:11–17.

    CAS  Article  Google Scholar 

  17. 17

    Boltshauser E, Flüeler U, Kilchhofer A . Iris hamartomas as diagnostic criterion in neurofibromatosis. Ann Neurol 1985;18:415–16.

    Google Scholar 

  18. 18

    Flüeler U, Boltshauser E, Kilchhofer A . Iris hamartomata as diagnostic criterion in neurofibromatosis. Neuropediatrics 1986;17:183–5.

    Article  Google Scholar 

  19. 19

    Huson S, Harper P, Compston D . Von Recklinghausen neurofibromatosis. A clinical and population study in south-east Wales. Brain 1988;111:1355–81.

    Article  Google Scholar 

  20. 20

    Lubs M-LE, Bauer MS, Formas ME, Djokic B . Lisch nodules in neurofibromatosis type 1. N Engl J Med 1991;324:1264–6.

    CAS  Article  Google Scholar 

  21. 21

    Obringer AC, Meadows AT, Zackai EH . The diagnosis of neurofibromatosis-1 in the child under the age of 6 years. Am J Dis Child 1989;143:717–9.

    CAS  PubMed  Google Scholar 

  22. 22

    Zehavi C, Romano A, Goodman RM . Iris (Lisch) nodules in neurofibromatosis. Clin Genet 1986;29:51–5.

    CAS  Article  Google Scholar 

  23. 23

    Markowitz JA, Hawkins BS, Diener-West M, Schachat AP . A review of mortality from choroidal melanoma. I. Quality of published reports, 1966-88. Arch Ophthalmol 1992;110:239–44.

    CAS  Article  Google Scholar 

  24. 24

    Diener-West M, Hawkins BS, Markowitz JA, Schachat AP . A review of mortality from choroidal melanoma. II A meta-analysis of 5-year mortality rates following enucleation, 1966 through 1988. Arch Ophthalmol 1992;110:245–50.

    CAS  Article  Google Scholar 

  25. 25

    Toonstra J, Dandrieu MR, Ippel PF, Delleman JW, Rupert Jr PHJM, Huitema HB . Are Lisch nodules an ocular marker of the neurofibromatosis gene in otherwise unaffected family members? Dermatologica 1987;174:232–5.

    CAS  Article  Google Scholar 

  26. 26

    Ponder B . Neurofibromatosis gene cloned. Nature 1990;346:703–4.

    CAS  Article  Google Scholar 

  27. 27

    Riccardi VM, Lewis RA . Penetrance of von Recklinghausen neurofibromatosis: a distinction between predecessors and descendants. Am J Hum Genet 1988;42:284–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  28. 28

    Allanson J, Upadhyaya M, Watson G, Partington M, Mackenzie A, Lahey D, et al. Watson syndrome—is it a subtype of type-1 neurofibromatosis? J Med Genet 1991;28:752–6.

    CAS  Article  Google Scholar 

  29. 29

    Weleber RG, Zonana J . Iris hamartomas (Lisch nodules) in a case of segmental neurofibromatosis. Am J Ophthalmol 1983;96:740–3.

    CAS  Article  Google Scholar 

  30. 30

    Charles SJ, Moore AT, Yates JRW, Ferguson-Smith MA . Lisch nodules in neurofibromatosis type 2. Arch Ophthalmol 1989;107:1571–2.

    CAS  Article  Google Scholar 

  31. 31

    Garretto NS, Ameriso S, Molina HA, Arberas C, Salvat J, Monteverde D, Sica REP . Type 2 neurofibromatosis with Lisch nodules. Neurofibromatosis 1989;2:315–21.

    CAS  PubMed  Google Scholar 

  32. 32

    Parry DM, Sherman JL, Pilus A, Kaiser-Kupfer MI, Eldridge R . In: Mulvihill JJ (moderator). Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis): an update. Ann Intern Med 1990;113:39–52.

    Article  Google Scholar 

Download references

Author information



Corresponding author

Correspondence to Nicola K Ragge.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Ragge, N., Falk, R., Cohen, W. et al. Images of Lisch nodules across the spectrum. Eye 7, 95–101 (1993).

Download citation


  • Iris hamartoma
  • Lisch nodules
  • Neurofibromatosis type 1
  • NF1
  • Ophthalmic manifestations
  • von Recklinghausen neurofibromatosis

Further reading


Quick links