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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2006  |  Volume : 24  |  Issue : 5  |  Page : 24-26

Incontinentia pigmenti

Department of Pedodontics and Preventive Dentistry, Yenepoya Dental College; Mangalore, Karnataka, India

Correspondence Address:
Sundeep K Hegde
Department of Pedodontics and Preventive Dentistry, Yenepoya Dental College, Nithyananda Nagar Post, Derlakatte, Mangalore - 575 018, Karnataka
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Source of Support: None, Conflict of Interest: None

PMID: 16891746

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Incontinentia pigmenti or Bloch-Sulzberger syndrome, is a rare X linked dominant disorder with characteristic skin, hair, eye, dental and neurological abnormalities commonly affecting females. This article reports the clinical features and management of a 11 year old girl diagnosed with Incontinentia pigmenti.

Keywords: Incontinentia pigmenti, hypodontia,multidisciplinary approach

How to cite this article:
Hegde SK, Bhat S S, Soumya S, Pai D. Incontinentia pigmenti. J Indian Soc Pedod Prev Dent 2006;24, Suppl S1:24-6

How to cite this URL:
Hegde SK, Bhat S S, Soumya S, Pai D. Incontinentia pigmenti. J Indian Soc Pedod Prev Dent [serial online] 2006 [cited 2022 Dec 9];24, Suppl S1:24-6. Available from: http://www.jisppd.com/text.asp?2006/24/5/24/26033

  Introduction Top

Incontinentia pigmenti (IP) or Block-Sulzberger syndrome is a rare X linked dominant disorder usually fatal prenatally in males and in affected females it causes highly variable abnormalities of the skin, eyes, hair, nails, teeth and the central nervous system.[1] The disease is characterised by appearance of erythematous eruptions with linear vesiculations at birth or shortly thereafter. Weeks to months after the onset it progresses as veruccous growths or the appearance of irregular macules, streaks and splashes of brown to slate grey pigmentations, usually on the torso and less frequently on the extremities. These are known to gradually fade or disappear by adulthood.[2],[3]

Dental manifestations like hypodontia, peg shaped or malformed teeth occur in 30-43% of patients, in 18% of patients delayed eruption is observed and dysplastic teeth in about 30-80% of the cases.[2],[4],[5] Neurological manifestations include mental deficiency, microcephaly, spasticity and /or seizures in about 30% of the cases.[2] Hair, nails and eyes are affected in most cases and musculoskeletal disorders may be seen.[2],[5],[6]

  Case Report Top

An eleven year old girl reported to pediatric dental clinic with a complaint of mobile upper left front teeth since three months. The child showed hyperpigmented skin lesions, wooly hair and abnormal gait.

History revealed that patient is a known case of Incontinentia pigmenti. The mother reported that this child was delieverd by a ceasarian section post term and had a history of previous abortion with a still born male child. The mother reported that she had similar skin lesions as her daughter, few missing teeth and delayed eruption of teeth. The child was diagnosed of Incontinentia pigmenti at five months of age as she developed skin lesions of the vesicular type. The mother revealed that the child had developed high-grade fever and convulsions at the age of five months and was in coma for three days. She later developed cerebral palsy with left hemiparesis and is presently under medication for epilepsy. The child showed delayed developmental milestones, delayed eruption of the deciduous and permanent teeth. The permanent upper central incisors had erupted at the age of nine years with the delayed exfoliation of deciduous central incisors.

The child showed hyperpigmented skin lesions which were brown to slate blue grey in colour on the hands [Figure - 1] and the medial aspect of the thigh [Figure - 2] and also on the inner aspect of the pinna. Wooly hair and strabismus were also noticed. The abnormal gait was due to the left sided hemiparesis. Child also showed neurological manifestations like mental deficiency and seizures.

The intra oral examination revealed hypodontia, teeth present were: Permanent central incisor, deciduous canine, deciduous second molar in the upper right quadrent. Permanent central incisor, over-retained deciduous lateral incisor, buccally erupting permanent lateral incisor, deciduous canine, deciduous second molar, permanent first molar in the upper left quadrant. Permanent lateral incisor, deciduous canine, deciduous first molar and permanent first molar in the lower left quadrant. The permanent central and lateral incisor, deciduous canine, deciduous second molar, permanent first molar in the lower right quadrant.

Notching was evident in both the upper permanent central incisors. Left permanent lateral incisor was erupting buccal to the overretained mobile deciduous upper left lateral incisor as seen in [Figure - 3]. The lower incisors were cone shaped and spiky in appearance [Figure - 4]. Carious lesions were found on upper right and left deciduous second molar.

An orthopantomogram was taken, it revealed that the patient was in an early mixed dentition stage with congenitally missing teeth. Occlusal radiograph of the upper arch revealed root resorption in deciduous upper left lateral incisor and the erupting permanent left lateral incisor [Figure - 5].

Carious lesions were restored and pit and fissure sealants were applied to the erupted permanent molars. The over retained deciduous lateral incisor was extracted. Systemic abnormalities of the patient were attended by the medical fraternity simultaneously.

  Discussion Top

Incontinentia pigmenti is a rare X linked inherited disorder where the male feotus usually dies in utero and the female survives due to moderating effects of lyonisation but however manifest with multiple organ defects.[8] It is called as Incontientia pigmenti because of the incontinence of the melanin pigment from the epidermis into the dermis.[9]

In literature suggests that over 80% of the cases are known to have alterations of the NEMO protien which is essentially a regulatory component of the 1KappaBkinase (KK) complex. NF-Kappa B responses are required to regulate apoptosis in certain cells.[1] Many of the clinical signs of Incontinentia pigmenti such as delayed tooth eruption are related to the functions of NF-kappa B pathway.[8]

Dental findings in this case include hypodontia, peg shaped teeth,malformed teeth, dysplastic teeth and delayed eruption like in most reported cases.[2],[4],[5] The skin lesions were also typically slate grey to brown in colour and the appearance of the skin lesions were also classical to the description in the literature.[2],[3]

The clinical manifestations including the dental and dermatological findings closely resemble those of ectodermal dysplasia but the skin lesions in IP are preceeded by inflammatory vesiculations.[1]

Since patients with Incontinentia pigmenti manifest with varying degree of hypodontia they require the combined care of a pedodontist, orthodontist and prosthodontist for total rehabilitation.[2],[3],[10] Prosthetic rehabilitation is usually planned at the appropriate age as specific problems arise with the developing dentition and occlusion as in this case.[1]

Since Incontinentia pigmenti shows varying degree of involvement of different systems as presented in this case, a multidisciplinary approach is therefore warranted for the rehabilitation of such patients. The patients suffering from IP should be continuously reviewed by the ophthalmologist, dental surgeon and neurologist. The periodic dental follow up should emphasize the assesment of caries risk and implementation of preventive protocols.

  References Top

1.Cho SY, Lee CK, Drummond BK. Surviving male with incontinentia pigmenti: A case report. Int J Pediat Dentist 2004;14:69-72.  Back to cited text no. 1    
2.Welbury TA, Welbury RR. Incontinentia pigmenti (Bloch-Sulzberger syndrome): Report of case. J Dentist Child 1999;213-5.  Back to cited text no. 2    
3.Yamashiro T, Nakagawa K, Takada K. Case report: Orthodontic treatmant of dental problems in Incontinentia pigmenti. Angle Orthodont 1998;68:281-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Baddour HM, Steed DL, Tilson HB. Incontinentia pigmenti: report of case. J Oral Surg 1981;39:57-9.  Back to cited text no. 4  [PUBMED]  
5.Garney RG. Incontinentia pigmenti: A report of five cases and review of literature. Arch Dermatol 1976;112:535-42.  Back to cited text no. 5    
6.Berlin AL, Palier AS, Chan LS. A review and update on the molecular basis of pathophysiology. J Am Acad Dermatol 2002;47:169-87.  Back to cited text no. 6    
7.Tanboga I, Kargul B, Semra EM, Aydin Y. Clinical features of incontinentia pigmenti with emphasis on dermatoglyphic findings. J Clin Pediat Dentist 2000;25:161-5.  Back to cited text no. 7    
8.Mansour S, Woffendin H, Mitton S, Jeffery I, Jakins T, Kenwrick S, et al . Incontinentia pigmenti in a surviving male is accompanied by hypohydrotic ectodermal dysplasiaand recurrent infection. Am J Med Genet 2001;99:172-7.  Back to cited text no. 8    
9.Aradhya S, Courtois G, Rajkovic A, Lewis RA, Levy M, Israel A, et al . Atypical forms of incontinentia pigmenti in male individuals result from mutations of a cytosine tract in exon 10 of NEMO. Am J Hum Genet 2001;68:765-71.  Back to cited text no. 9    
10.Rouse JS. Incontinentia pigmenti: Overcoming cosmetic challenges. Compend Contin Educ Dent 1999;20:1034-41.  Back to cited text no. 10  [PUBMED]  


[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

This article has been cited by
1 Incontinentia pigmenti with macrocephaly
Subramoniam, C., Marthandan, P.T.
Indian Journal of Practical Pediatrics. 2010; 12(2): 210-213


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