Congenital Generalized Hypertrichosis, Gingival Hyperplasia, a Coarse Facies with Constriction Bands: A Rare Association

Abstract

Congenital generalized hypertrichosis terminalis is a rare primary hypertrichotic condition, of unknown etiology presenting in the pediatric population. Though benign in nature, there is considerable psychosocial trauma attached to this, owing to the cosmetic disfigurement it produces. The association of gingival fibromatosis and a coarse facies could further worsen the cosmesis. Thus, a multidisciplinary approach involving a psychologist, a dentist apart from the dermatologist would be mandatory. We present this rare syndrome with the purpose of getting a better insight regarding the inheritance, the clinical features and the best available treatment modalities, especially the modern and novel techniques of hair removal that could be utilized to manage such individuals.

Keywords: Coarse facies; constriction bands; gingival hyperplasia; hypertrichosis.

Figure 1

Terminal hair, seen over the face, chest, abdomen and the upper extremities. Also to note here is the coarse facies with low set slightly enlarged ears and constriction bands over the arms

Figure 2

Terminal hair over the back

Figure 3

Bushy eye-brows as seen in our patient

Figure 4

Prominent palmar dermatoglyphics

Figure 5

Terminal hair seen over bilateral lower limbs with incomplete constriction bands over the medial aspect of both the thighs

Figure 6

Hyperplastic stippled gingival architecture with dental disarray