Journal of the Chinese Medical Association
Volume 73, Issue 5 , Pages 275-278, May 2010

Concurrence of Incontinentia Pigmenti and Behçet's Disease

  • Heng-Kuei Lin
    • ,
  • Lin-Shien Fu

      Affiliations

    • Corresponding Author InformationCorrespondence to: Dr Lin-Shien Fu, Department of Pediatrics, Taichung Veterans General Hospital, 160, Section 3, Chung-Kang Road, Taichung 407, Taiwan, R.O.C.

Division of Immunology and Nephrology, Department of Pediatrics, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C.

Received 14 September 2009; accepted 19 March 2010.

Article Outline

We report here a rare case of incontinentia pigmenti (IP) in a 10-year-old girl who developed Behçet's disease. IP was diagnosed in infancy and Behçet's disease was diagnosed at 10 years of age. The initial presentations of Behçet's disease were spiking fever and recurrent painful oral and genital ulcers that were refractory to antibiotics. After corticosteroid treatment, her fever subsided and ulcers subsequently healed. The patient's mother and sister were also diagnosed with IP. Her mother had suffered from Behçet's disease since her teenage years and it was complicated with colon perforation. Although there are several reports on the combination of IP and Behçet's disease, this is the first reported case of a family with such concurrence.

Key Words:  Behçet's disease , family , incontinentia pigmenti

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References 

  1. Bell S , Degitz K , Quirling M , Jilg N , Page S , Brand K . Involvement of NF-kappaB signalling in skin physiology and disease . Cell Signal . 2003;15:1–7
  2. Berlin AL , Paller AS , Chan LS . Incontinentia pigmenti: a review and update on the molecular basis of pathophysiology . J Am Acad Dermatol . 2002;47:169–187 quiz 188-90
  3. Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, et al. Behcet's disease: a contemporary review . J Autoimmun . 2009;32:178–188
  4. Endoh M , Yokozeki H , Maruyama R , Matsunaga T , Katayama I , Nishioka K . Incontinentia pigmenti and Behcet's disease: a case of impaired neutrophil chemotaxis . Dermatology . 1996;192:285–287
  5. Menni S , Piccinno R , Biolchini A , Delle Piane RM , Bardare M . Incontinentia pigmenti and Behcet's syndrome: an unusual combination . Acta Derm Venereol . 1986;66:351–354
  6. Landy SJ , Donnai D . Incontinentia pigmenti (Bloch-Sulzberger syndrome) . J Med Genet . 1993;30:53–59
  7. Sarica R , Azizlerli G , Köse A , Dişçi R , Ovül C , Kural Z . Juvenile Behçet's disease among 1784 Turkish Behçet's patients . Int J Dermatol . 1996;35:109–111
  8. Kenwrick S, Woffendin H, Jakins T, Shuttleworth SG, Mayer E, Greenhalgh L, et al  Survival of male patients with incontinentia pigmenti carrying a lethal mutation can be explained by somatic mosaicism or Klinefelter syndrome . Am J Hum Genet . 2001;69:1210–1217
  9. Senturk N, Aydin F, Haciomeroglu P, Yildiz L, Totan M, Canturk T, et al. Pulmonary tuberculosis and cutaneous mycobacterial infection in a patient with incontinentia pigmenti . Pediatr Dermatol . 2004;21:660–663
  10. Eksioglu-Demiralp E , Direskeneli H , Ergun T , Fresko I , Akoglu T . Increased CD4+ CD16+ and CD4+ CD56+ T cell subsets in Behçet's disease . Rheumatol Int . 1999;19:23–26
  11. Yato H , Matsumoto Y . CD56+ T cells in the peripheral blood of uveitis patients . Br J Ophthalmol . 1999;83:1386–1388
  12. Suzuki Kurokawa M , Suzuki N . Behçet's disease . Clin Exp Med . 2004;4:10–20
  13. Akpolat T, Koc Y, Yeniay I, Akpek G, Güllü I, Kansu E, et al  Familial Behçet's disease . Eur J Med . 1992;1:391–395
  14. Ferraz MB , Walter SD , Heymann R , Atra E . Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach . Br J Rheumatol . 1995;34:932–935
  15. Criteria for diagnosis of Behçet's disease  . International Study Group for Behçet's Disease . Lancet . 1990;335:1078–1080
  16. Kim DK , Chang SN , Bang D , Lee ES , Lee S . Clinical analysis of 40 cases of childhood-onset Behçet's disease . Pediatr Dermatol . 1994;11:95–101
  17. Chung YM , Lin YC , Tsai CC , Huang DF . Behçet's disease with uveitis in Taiwan . J Chin Med Assoc . 2008;71:509–516
  18. Chou LC, Sheu SJ, Hong MC, Hsiao YC, Wu TT, Chuang CT, et al. Endogenous uveitis: experiences in Kaohsiung Veterans General Hospital . J Chin Med Assoc . 2003;66:46–50
  19. Ammann AJ , Johnson A , Fyfe GA , Leonards R , Wara DW , Cowan MJ . Behçet syndrome . J Pediatr . 1985;107:41–43
  20. Kone-Paut I . Behçet's disease: pediatric features . Ann Med Interne (Paris) . 1999;150:571–575 [In French]

PII: S1726-4901(10)70060-5

doi:10.1016/S1726-4901(10)70060-5

Journal of the Chinese Medical Association
Volume 73, Issue 5 , Pages 275-278, May 2010

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