Ann Dermatol.  2009 Feb;21(1):53-55. 10.5021/ad.2009.21.1.53.

A Case of Isolated Plexiform Neurofibroma in a Patient with Myasthenia Gravis

• Back SJ
• Kim DH
• Kim N
• Lee Y
• Seo YJ
• Park JK
• Lee JH

• KMID: 2172067
• DOI: http://doi.org/10.5021/ad.2009.21.1.53

Abstract

We report a case of an isolated plexiform neurofibroma occurring in a patient with myasthenia gravis. A 48-year-old man presented with asymptomatic skin-colored nodules on the tip of his 4th finger. Microscopically, a plexiform neurofibroma was identified located in the dermis that appeared to originate from small superficial nerves. He had a 20-year history of treated myasthenia gravis; otherwise, his personal and family histories were unremarkable. Given that myasthenia gravis is a disorder of the peripheral nerves, plexiform neurofibromas could be associated with myasthenia gravis. However, the development of an isolated plexiform neurofibroma in a case of myasthenia gravis has not yet been reported. The occurrence of a neurofibromas in a patient with myasthenia gravis suggests a link in the pathogenesis of these two diseases.

Keyword

Isolated; Myasthenia gravis; Plexiform neurofibroma

MeSH Terms

Aluminum Hydroxide
Carbonates
Dermis
Fingers
Humans
Middle Aged
Myasthenia Gravis
Neurofibroma
Neurofibroma, Plexiform
Peripheral Nerves
Aluminum Hydroxide
Carbonates

Figure

• 

  Fig. 1 0.7×0.5 cm sized rubbery nodule on right fourth finger tip.

• 

  Fig. 2 (A) Well circumscribed numerous nerve fascicles with irregular configurations embedded in cellular matrices (H&E stain, ×40). (B) Spindle shaped cells with wavy collagen fibrils in myxoid stroma (H&E stain, ×100). (C) Numerous cells of the fascicles present intensive positivity for S-100 protein (S100 stain, ×200).

Reference

1. Robert L, Joel C. Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. The neurofibromatoses. Fitzpatrick's dermatology in general medicine. 2008. 7th ed. New York: McGraw-Hill;1332–1334.

2. Legius E, Descheemaeker MJ, Fryns JP, Van den Berghe H. Neurofibromatosis type 1. Genet Couns. 1994. 5:225–241.

3. Kubota A, Komiyama A, Hasegawa O. Myasthenia gravis and alopecia areata. Neurology. 1997. 48:774–775.

4. Younus J, Ahmed AR. The relationship of pemphigus to neoplasia. J Am Acad Dermatol. 1990. 23:498–502.

5. Kwan SY, Lin JH, Su MS. Coexistence of epilepsy, myasthenia gravis and psoriasis vulgaris. Zhonghua Yi Xue Za Zhi (Taipei). 2000. 63:153–157.

6. Korf BR. Plexiform neurofibromas. Am J Med Genet. 1999. 89:31–37.

7. Crawford AH, Schorry EK. Neurofibromatosis update. J Pediatr Orthop. 2006. 26:413–423.

8. Enzinger FM, Weiss SW. Soft tissue tumors. 1988. 2nd ed. St Louis: Mosby;748–749.

9. Aloi FG, Massobrio R. Solitary plexiform neurofibroma. Dermatologica. 1989. 179:84–86.

10. Fisher DA, Chu P, McCalmont T. Solitary plexiform neurofibroma is not pathognomonic of von Recklinghausen's neurofibromatosis: a report of a case. Int J Dermatol. 1997. 36:439–442.

11. Lee HJ, Koh BK, Ha SJ, Kim JW. A case of isolated plexiform neurofibroma. Ann Dermatol. 2000. 12:271–274.

12. Younger DS, Worrall BB, Penn AS. Myasthenia gravis: historical perspective and overview. Neurology. 1997. 48:Supp 5. S1–S7.

13. Lovelace RE, Younger DS. Myasthenia gravis with thymoma. Neurology. 1997. 48:Supp 5. S76–S81.

14. Tan RS. Ulcerative colitis, myasthenia gravis, atypical lichen planus, alopecia areata, vitiligo. Proc R Soc Med. 1974. 67:195–196.

15. Waugh WH. The development of myasthenia gravis in a case of multiple neurofibromatosis. South Med J. 1953. 46:1132–1134.