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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 2  |  Page : 219-222

Werewolf syndrome associated with gingival fibromatosis: A rare case report


Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College and Research Institute, Sunam, Punjab, India

Date of Submission21-Aug-2016
Date of Acceptance21-Nov-2016
Date of Web Publication02-Dec-2016

Correspondence Address:
Dr. Mysore K Sunil
Department of Oral Medicine and Radiology, Guru Nanak Dev Dental College and Research Institute, Sunam - 148 028, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.195144

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   Abstract 

Hypertrichosis is a rare disorder that causes abnormal excessive body hair growth. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to that of a werewolf. It is often associated with additional anomalies including gingival hyperplasia, deafness, cardiomegaly and bone abnormalities. The association of gingival fibromatosis and a coarse facies could further worsen the esthetics. Thus, a multidisciplinary approach involving a psychologist, a dentist as well as a dermatologist would be mandatory. We present this rare syndrome describing a case of a 9-year-old boy with congenital hypertrichosis and gingival fibromatosis. The growth being normal, the features of this boy were similar to hypertrichosis. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to the werewolf.

Keywords: Fibromatosis, hypertrichosis, werewolf syndrome


How to cite this article:
Sunil MK, Trivedi A, Arora S, Gupta S. Werewolf syndrome associated with gingival fibromatosis: A rare case report. J Indian Acad Oral Med Radiol 2016;28:219-22

How to cite this URL:
Sunil MK, Trivedi A, Arora S, Gupta S. Werewolf syndrome associated with gingival fibromatosis: A rare case report. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2020 Oct 23];28:219-22. Available from: https://www.jiaomr.in/text.asp?2016/28/2/219/195144


   Introduction Top


Hypertrichosis is the term used for hair growth on any part of the body in excess of the amount usually present in persons of the same age, race and sex, excluding androgen-induced hair growth. In its generalized and circumscribed forms, hypertrichosis may either be an isolated finding, or may be associated with other abnormalities. Hypertrichosis, informally called "werewolf syndrome" is a very rare condition, with fewer than 100 cases documented worldwide. [1],[2]

Hypertrichosis syndromes fall under the larger umbrella of ectodermal dysplasias or abnormal development of the hair, skin, nails, teeth, and/or eccrine glands, and are often associated with additional anomalies including gingival hyperplasia, deafness, cardiomegaly and bone abnormalities. [2] Hypertrichosis may be congenital or acquired. People with congenital hypertrichosis were often referred to as "wolf men," "werewolves," and "ape-men" in the 19 th century, and perhaps even today, they are crowd-drawers at sideshow acts. It can be caused by a number of factors including malnutrition and untreated infection. [3]


   Case Report Top


A 9-year-old boy reported to the department of oral medicine and maxillofacial radiology with a chief complaint of gums enlargement in the upper and lower teeth region. The boy was born out of non-consanguineous marriage, and presented with excessive hair on the whole body. The baby was normally delivered, and the mother stated that he had pigmented hair all over the body, which increased with age. The boy had average growth and achieved normal developmental milestones. There was no history of convulsion in the neonatal period or early childhood and no long-term drugs were given. Central nervous system examination and other systems were normal. Clinical examination showed a mentally alert, well-built child with hypertrichosis, a prominent feature of which was extremely bushy eyebrows meeting in the midline over a wide and retruded nasal bridge. A downy growth of hair was present on the cheeks, chin, upper lip, and chin [Figure 1], and a profuse growth of dark brown hair was present on both the upper and lower limbs the mid back [Figure 2]a and b. The hair on the forearms were 1-2 cm in length [Figure 3]a,b and [Figure 4]a,b.
Figure 1: Downy growth of hair was present on the cheeks and bushy eyebrows

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Figure 2: (a) Scanty hair growth was seen on the dorsal aspect and (b) profuse hair growth on the ventral part of the body

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Figures 3: (a and b) Profuse hair growth seen on legs

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Figure 4: (a) Hair growth also seen on arms with (b) scanty hair growth on dorsal surface of the arms

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On intraoral examination, generalized gingival hyperplasia was present, the tissue being pale pink in color, exceedingly thick, conspicuously stippled and covered at least a half of the anatomical crowns of the deciduous teeth [Figure 5]a and b. Lips were incompetetent due to gingival hyperplasia. The gingival tissue was exceedingly thick, conspicuously stippled, and devoid of signs of inflammation. The only permanent teeth which had erupted into the mouth were 11, 12, 16, 21, 22, 26, 31, 32, 36, 41, 42, 46, and deciduous teeth were present. In the upper jaw, the palatal anatomy was distorted by gross thickening of the palatal mucosa covering the hard palate, especially close to the gingival margins in the molar regions. The dentition in all other respects appeared normal. No significant abnormality could be detected elsewhere on X-ray examination of the chest, cervical spine, hands, and skull, and bone age was within the normal range. His height and weight were normal.
Figure 5: Gingival enlargement in relation to (a) maxillary teeth and (b) mandibular teeth region

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   Discussion Top


Hypertrichosis is a rare disorder that causes abnormal excessive growth of body hair. Hypertrichosis has informally been called werewolf syndrome because the appearance is similar to that of a werewolf. The two distinct types of hypertrichosis are generalized hypertrichosis, which occurs over the entire body, and localized hypertrichosis, which is restricted to a certain area. [3] Hypertrichosis can be either congenital (present at birth) or acquired later in life. The excess growth of hair occurs in areas of the skin with the exception of androgen-dependent hair of the pubic area, face, and axillary regions. In contrast, hirsutism is characterized by excessive growth of androgen-dependent hair.

Hypertrichosis may involve: [3],[4]

  • Lanugo hair,
  • vellus hair, and
  • terminal hair.
Lanugo hair are long, thin and unpigmented such as wool. Vellus or intermediate hair are short, unpigmented hair, and are variably medullated. Terminal hair contain a medulla, and are long, thick, and pigmented.

Hypertrichosis is classified based on:

  • The age of onset (congenital or acquired),
  • the extent of distribution (generalized or circumscribed), and
  • the site involved (pinnae auris, elbows, anterior or posterior neck, lumobosacral region).
In both its generalized and circumscribed forms, hypertrichosis may be an isolated finding or may be associated with other abnormalities. The first recorded case of hypertrichosis was that of Petrus Gonsalvus of the Canary Islands. This was documented by Altrovandus in the mid-17 th century and published in his text Monstrorum Historia cum Paralipomeni historiae omnium animalium published in 1642. [1] He noted two daughters, a son, and a grandchild in the Gonsalvus family who had hypertrichosis. Altrovandus dubbed them the Ambras family, after the Ambras Castle near Innsbruck, where portraits of the family were found. During the next 300 years, approximately 50 cases were observed. The scientist Rudolf Virchow described a form of hypertrichosis accompanied with gingival hyperplasia in 1873. [3],[5]

Bondeson and Miles described (1993) the case of Julia Pastrana (1834-1860) who had gained immortality as one of the most extreme cases of generalized hypertrichosis upon record. After Julia Pastrana's death in childbirth, her corpse was embalmed in a very life-like manner and exhibited all over Europe for several decades. Later, the mummy was believed to be lost, but in 1990, it was discovered at the Oslo Forensic Institute. Some writers have included Julia Pastrana among the cases of congenital hypertrichosis languinosa. [6] However, a microscopic examination of hair samples from the mummy shows that her hairy growth is unmistakably terminal in character. She was an example of congenital, generalized hypertrichosis terminalis with associated gingival hyperplasia. Weski (1920) and Garn and Hatch (1950) suggested extreme hirsutism with gingival fibromatosis follows a dominant pattern of inheritance. There is no necessary relationship between the age of development of gingival changes and hypertrichosis.

Other conditions reported presented with additional systemic disorders. [6] Mucopolysaccharidosis (MPS) are a family of lysosomal storage disorders resulting from the partial catabolism of several glycosaminoglycans (GAGs). Depending on which particular enzyme is deficient, the MPS syndromes are defined into groups MPS I through VII, with several subgroups for a total of 10 disorders. In humans, clinical features include dysmorphic features, hepatosplenomegaly, hypertelorism, macroglossia, hypoplastic, and irregularly shaped teeth, hyperplastic lips and gingiva, facial dysmorphia, corneal clouding, and mental retardation. Gingival enlargement is considered to be one of the main oral manifestations of Maroteaux-Lamy syndrome, and a common feature in Hurler syndrome, where hypertrichosis is associated with severe mental retardation, clouding of cornea, enlargement of liver and spleen, dwarfism with hunched back, short limbs and clawed hands, a large head with wide-set eyes, heavy brow ridges and deep bridge of nose, and poorly formed teeth. In this case, the patient does not present with mental retardation or any systemic abnormality. [6]

There are a number of more syndromes encountered with hypertrichosis, however, discussing all would be beyond the scope of this article. Because these disorders are associated with a multitude of systemic involvement, a multidisciplinary approach would be required for managing these patients, involving the concerned specialty in the treatment. As far as the management of hypertrichosis is concerned, long-term removal of hair in these patients poses to be a challenging concern. This in turn is dependent upon the degree of hair growth, the patient's psychologic profile, and the issue of social acceptance. Epilatory methods of hair removal, whereby the entire hair shaft is uprooted, would be a better method here than the depilatory methods because of the longer duration of hair free skin. [5],[6] Epilatory methods include mechanical or electronic tweezers, wax epilation, or the use of a twisted string running rapidly over a hair bearing area, thus epilating the hair. These methods maybe of use in small areas of the body, for example, the face, as they are painful and may not receive the full cooperation of the child. Other newer therapies using lasers and lights may be of help for removing extensive areas of excessive hair growth, where the principle of thermolysis is applied to selectively target the melanin-rich hair follicles, which are destroyed causing minimal surrounding tissue affection. [6],[7]

However, their use in prepubertal children has not been extensively studied. Morley et al. assessed the use of the long-pulsed ruby laser in prepubertal children and have concluded that it is a safe and effective technique for epilation with pertinent levels of patient and parent satisfaction. According to De Raeve, the Q-switched Nd:Yag laser at low fluences was effective in markedly reducing the hair density in a patient of hypertrichosis lanuginose congenita. Because these disorders are so rare, large studies in this aspect is lacking. A novel therapy, using eflornithine hydrochloride, an ornithine decarboxylase inhibitor, available as a 13.9% cream has shown to hold promise in the issue of hair growth retardation in women. However, in the pediatric population, the efficacy and safety for the same has not been established. [8]


   Conclusion Top


To conclude, hypertrichosis on its own is still an enigma, which needs further research in order to get a vivid understanding in this context. Second, as far as therapy is concerned, a better knowledge of the clinicopathogenesis could tremendously prove useful in this setting for the exploration of newer modalities, which could definitely help to outline better quality of life in these patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Horning GM, Fisher JG, Barker BF, Killoy WJ, Lowe JW. Gingival fibromatosis with hypertrichosis: A case report. J. Periodont 1985;56:344-7.  Back to cited text no. 1
    
2.
Angik R, Nanda S, Bhola N, Borle R, Sinha AK. Hypertrichosis Terminalis (Mangino Variant)-A Case report. J Res Adv Dent 2013;2:3s: 54-7.  Back to cited text no. 2
    
3.
Winter GB, Simpkiss MJ. Hypertrichosis with hereditary gingival hyperplasia. Arch Dis Child 1974;49:394.  Back to cited text no. 3
    
4.
Baumeister FA, Schwarz HP, Stengel-Rutkowski S. Childhood hypertrichosis: Diagnosis and management. Arch Dis Child 1995;72:457-9.  Back to cited text no. 4
    
5.
Trüeb RM. Causes and Management of Hypertrichosis. Am J Clin Dermatol 2002;3:617-27.  Back to cited text no. 5
    
6.
Bubna AK, Veeraraghavan M, Anandan SS, Rangarajan S. Congenital Generalized Hypertrichosis, Gingival Hyperplasia, a Coarse Facies with Constriction Bands: A Rare Association. Int J Trichol 2015;7:67-71.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Vashi RA, Mancini AJ, Paller AS. Primary generalized and localized hypertrichosis in children. Arch Dermatol 2001;137:877-84.  Back to cited text no. 7
    
8.
De Raeve L, Keymolen K. Congenital hypertrichosis lanuginosa in a father and son. Arch Dermatol 2011;147:746-7.  Back to cited text no. 8
    


    Figures

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



 

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