Cessation in root development: Ramifications of ...

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Case Report

Cessation in root development: Ramifications of ‘Stevens–Johnson’ syndrome Abstract The case presented here is of a 15‑year‑old girl in whom nearly all the teeth except for lower first molars and lower central incisors showed short roots as observed through panoramic radiograph. At the same time there was difference in the length of roots of various teeth. The patient suffered an acute attack of Stevens–Johnson syndrome (SJS) at the age of 8 years and since no other possible explanation for the short roots anomaly could be found, it could be concluded that the cessation in root development may have been caused by the destruction or damage of the epithelial root sheath during the SJS disease.

Bajaj N, Madan N1, Rathnam A2

Department of Pedodontics and Preventive Dentistry, PDM Dental College and Research Institute, Bahadurgarh, Haryana, 1 SBB Dental College, Masuri, Ghaziabad, 2Department of Pedodontics and Preventive Dentistry, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India Correspondence: Dr. Neeti Bajaj, Department of Pedodontics and Preventive Dentistry, PDM Dental College and Research Institute, Bahadurgarh, Haryana, India. E‑mail: [email protected] Access this article online Quick Response Code:

Key words

Website: www.jisppd.com DOI: 10.4103/0970-4388.105023

Short root anomaly, Stevens–Johnson syndrome, Epithelial root sheath

PMID: ***

Introduction Von Hebra in 1860,[1] for the first time, described a mild skin disease without abnormalities of the mucous membranes and without systemic manifestations, which he called erythema exudativum multiforme. In 1922, Stevens and Johnson[2] described this syndrome again with reference to two cases without realizing that the disease was previously reported by Von Hebra. Their observation of the disease highlighted a combination of exudative multiform erythema, stomatitis, conjunctivitis (sometimes leading to keratitis and panophthalmitis), and severe constitutional symptoms. The disorder subsequently was referred to as Stevens–Johnson syndrome (SJS) that represents a more severe form of erythema multiforme with or without systemic complications. In later years, several cases involving systemic complications such as myocardial injury, degenerative changes of the liver cells, transverse myelitis, nephritis, acute tubular necrosis, and permanent anonychia were observed that

implied internal organs getting impacted and indicated that SJS is not merely confined to skin disorders.[3] The case report presented here suggests that the SJS may also interfere with the development of the teeth.

Case Report A 15‑year‑old female patient accompanied by her father, reported to the department of Pedodontics and Preventive dentistry with the chief complaint of pain in the lower left back tooth since few days. Examination of the soft tissue presented absence of any lesion on the oral mucosa, however, mild marginal gingivitis was seen in relation to the lower anteriors. Hard tissue examination showed the presence of complete permanent dentition with unerupted upper third molars. The carious teeth noted were: 36, 37, 38, 46, 47, 48. Teeth 38 and 48 were grossly decayed and

JOURNAL OF INDIAN SOCIETY OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jul - Sep 2012 | Issue 3 | Vol 30 |

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Bajaj, et al.: Cessation in root development - ramifications of ‘Stevens – Johnson’ syndrome

indicated for extraction [Figure 1]. The enamel of the healthy teeth was of normal structure and normal color. Tongue, lips, and buccal mucosa showed no abnormal findings. The past medical history of the patient from the clinical records maintained by the parents highlighted development of painful lesions in the mouth as well as on the arms along with high fever at the age of 8 years. The patient at that time was prescribed Acyclovir, Roxid Kid, Nimusulide‑P by the family physician. During the course of medicines, the patient developed generalized erythema with several vesicles and bullae on both the cheeks filled with a clear fluid. Eyelids glued together due to purulent conjunctivitis along with the involvement of cornea. Red macupapular skin lesions developed whole over the body. The disease was diagnosed to be SJS and was thought to be due to the suppositories, which the patient had received from the family doctor. The patient was hospitalized and given the course of steroids, analgesics, and antipyretics. The patient was transferred to the eye hospital for further treatment. There was no relevant past dental history. Patient did not reveal tobacco consumption and she brushes her teeth once daily with a toothbrush and toothpaste. Patient’s family history was noncontributory. When the patient reported to the department of Pedodontics and Preventive dentistry, she was 15 years of age, had normal weight, height, and of normal mental status. The IQ testing revealed average level. The vital signs were within normal limits. She was partially blind and had great difficulty in opening the eyes [Figure 2]. Partial Anonychia was seen in both the hands and feet [Figures 3 and 4]. These changes had been caused by the SJS.

Investigations Routine blood and urine examination was carried out, which did not reveal any abnormalities. The most noticeable feature of the dentition was revealed by the panoramic radiograph. Lower first molars as well as the lower central incisors showed complete root formation while the upper molars, canines, premolars, and second molars (both upper and lower) had short roots and varied lengths [Figure 5]. The roots of the maxillary and mandibular lateral incisors were the longest while those of premolars were the shortest [Figure 6]. The pulp chamber and root canal of all teeth were of normal width. A normal periodontal membrane space was present around the flattened roots of all the teeth. 268

Final diagnosis SJS and chronic irreversible pulpitis with 38 and 48 and chronic dentinal caries with 36, 37, 46, and 47.

Treatment Tablet Mox 250 mg thrice daily was prescribed for 5 days and then extraction was done with 38 and 48 under inferior alveolar nerve block, lingual nerve block, and long buccal nerve block. Patient was followed for prescription of steroids, if needed. Silver amalgam restoration was done with 36, 37, 46, and 47. After 5 days, uneventful healing of the extraction site was observed. Patient was motivated for regular oral hygiene practices and dental follow up every 3 months. She was referred to ophthalmologist, dermatologist, and other concerned departments in the medical hospital.

Discussion Generalized root shortening is quite uncommon; it represents a phenomenon in which nearly all the permanent teeth are markedly shorter than normal. Several studies have been performed indicating short root anomalies attributed to factors such as Thalassemia, Endocrine derangements (such as hypoparathyroidism and hypocalcemia[4]), and hereditary dental disorders (such as Dental dysplasia and Dentinogenesis Imperfecta). Hazza and Al‑jamal[5] examined the panoramic radiographs of 50 Thalassemic patients and observed that spiky‑shaped and short roots were found to be reliable diagnostic criteria for Thalassaemia major. Lind[6] in his study on 112 children, with abnormally short roots of the maxillary central incisors concluded that the shortness of roots was not due to resorption or to any developmental disturbance of exogenous origin. He called this condition as short root anomaly and stated that the condition is a constitutional anomaly and its prevalence varies with sex, race, and hereditary predisposition. Radiographically short blunted roots, complete pulpal obliteration, periapical abscesses or cysts without an obvious causative factor, and spontaneous exfoliation are also the features of Dentin dysplasia.[7] Roots smaller than nor mal are also seen in Dentinogenesis Imperfecta. However, there are noticeable differences between the various types of short roots and the case that we present here. As in this case, the teeth not only showed short roots but there also were marked differences




Figure 1: Intraoral (mandibular arch) view showing carious teeth

Figure 2: Partial blindness in the patient due to Stevens‑Johnsons syndrome

Figure 4: Foot of the patient showing partial Anonychia Figure 3: Hands of the patient showing partial Anonychia

Figure 6: OPG (Orthopantomogram) of the patient showing premolars with shortest roots Figure 5: OPG (Orthopantomogram) of the patient showing shortened roots

between the lengths of the roots observed through panoramic examination. It was particularly these findings that excluded this case from the developmental disturbances affecting the teeth. It was also seen that compared with the roots of upper molars, premolars and second molars (maxillary and mandibular) lower central incisors and lower molars showed complete root formation suggesting that the cessation of growth of the teeth occurred at the age of 7 or 8 years, which

coincided with the time the patient suffered the acute attack of SJS. Similar findings were reported by De Man,[8] wherein nearly all the teeth of the permanent dentition had short roots. It was concluded in his findings that the cessation of root development may have been precipitated by a fulminant attack of SJS, which occurred at the age of 7 or 8 years. So far, only irradiation of permanent teeth at various stages of development is known to cause such developmental disturbances and the final shape and size of these teeth are also determined by the stage of development


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reached at the moment of irradiation.[9] In SJS, there is a necrosis of the epidermal cells and in more severe cases of the whole epidermis, it is quite possible that ‘Hertwigs’ epithelial root sheath cells also get destroyed, as a result of which further growth of the root could be arrested. This explains the short roots of premolars and second molars observed through the panoramic radiograph in the present case. Ranalli et al. reported a case of abnormal root development due to SJS. They noted that the patient showed varying degrees of abnormal root development wherein the premolars and the permanent second molars were most severely impacted, similar to the radiographic findings in the present case. [10]

Dental abnormalities involving shorter roots were also noted by Gaultier et al.[11] in three young patients who suffered an attack of SJS during childhood (at ages 5-11 years). The likely reasons for such dental abnormalities were attributed to the ‘Hertwig’ epithelial root sheath getting affected by the acute process of apoptosis that lead to the root‑building abortion. Similar findings were made by Brook[12] that involved varying degrees of arrested root development in permanent dentition in a 14‑year‑old boy who suffered an acute attack of SJS at the age of 9 years. Thornton and Worley[13] also reported a similar case involving short root anomaly in a patient with a history of SJS.

Conclusion Lately, some cases showing the correlation of dental findings and the SJS have been sighted with observations similar to this case report that establishes a high possibility of the developing dentition getting affected during the acute phase of SJS. We can conclude that the typical differences in length of the roots of various teeth in this case must have been due to the complete cessation of the growth of the teeth at the age of 7 or 8 years when patient suffered a fulminant

attack of SJS. A proactive approach should be taken involving educating the patients suffering from SJS over the probable ramifications in later stages of life and subsequent preventive measure that can be taken. Regular dental follow‑up examinations should be recommended to patients with the history of SJS.

References 1. Von Hebra F. Diseases of the skin. 1 st ed. London: New Sydenham Society; 1866. p. 285. 2. Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia. Am J Dis Child 1922;24:526‑33. 3. Claxton RC. A review of 31 cases of Stevens‑Johnson syndrome. Med J Aust 1963;50:963‑7. 4. Sunde OE, Hals E. Dental changes in a patient with hypoparathyroidism. Br Dent J 1972;111:112‑7. 5. Hazza’a AM, Al‑Jamal G. Radiographic features of the jaws and teeth in thalassaemia major. J Dentomaxillofac Radiol 2006;35:283‑8. 6. Lind V. Short root anomaly. Scand J Dent Res 1972;80:85‑93. 7. Wesley RK, Wysoki GP, Mintz SM, Jackson J. Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case. Oral Surg Oral Med Oral Pathol 1976;41:516‑24. 8. De Man K. Abnormal root development, probably due to erythema multiforme (Stevens‑Johnson syndrome). Int J Oral Surg 1979;8:381‑5. 9. Bruce KW, Stafne EC. The effect of irradiation on the dental system as demonstrated by the roentgenogram. J Am Dent Assoc 1950;41:684‑9. 10. Ranalli DN, Elliot MA, Whalez T, Campagna ED Jr. et al. Stevens‑Johnson Syndrome; report of case with abnormal root development. J Dent Child 1984;51:298‑301. 11. Gaultier F, Rochefort J, Landru MM, Allanore L, Naveau A, Roujeau JC, et al. Severe and Unrecognized Dental Abnormalities after Drug‑Induced Epidermal Necrolysis. Arch Dermatol 2009;145:1332‑3. 12. Brook U. Stevens–Johnson syndrome and abnormal root development: A case report. Int J Paediatr Dent 1994;4:101‑3. 13. Thornton JB, Worley SL. Short root anomaly in a patient with a history of Stevens‑Johnson syndrome: Report of case. ASDC J Dent Child 1991;58:256‑9. How to cite this article: Bajaj N, Madan N, Rathnam A. Cessation in root development: Ramifications of 'StevensJohnson' syndrome. J Indian Soc Pedod Prev Dent 2012;30: 267-70. Source of Support: Nil, Conflict of Interest: None declared.

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