Generalized hypertrichosis.

Sir,

A 4-year-old girl born out of non-consanguinous marriage presented with generalized increase in body hair noticed since birth. None of the other family members were affected. Hair was pigmented and soft suggesting vellus hair. There was generalized increase in body hair predominantly affecting the back of trunk arms and legs [Figures 1 and 2]. Face was relatively spared except for fore head. Palms and soles were spared. Scalp hair was normal. Teeth and nail were normal. There was no gingival hypertrophy. No other skeletal or systemic abnormalities were detected clinically. Routine blood investigations were normal. Hormonal study was within normal limit for her age. With this clinical picture of generalized hypertrichosis with no other associated anomalies a diagnosis of universal hypertrichosis was made.

Figure 1

Hypertrichosis of arms

Figure 2

Back showing inverted fir-tree pattern

Hypertrichosis is the growth of hair of an excessive amount and thickness on any part of the body.[1] Generalized hypertrichosis may be congenital or acquired. Congenital generalized hyperkeratosis affecting the lanugo hair is a rare autosomal dominant disease, where soft nonpigmented nonmedullated lanugo hair covers the whole body except palms and soles and is not replaced by terminal hair after birth.[2] Lanugo hair can grow up to 10 cm in length. In some cases, it may acquire terminal characteristics.[2] Associated dental anomalies are reported. Generalized hypertrichosis of vellus or terminal hair can occur as sole feature in universal hypertrichosis.[1] in which normally hairy areas like thorax, back, and limbs show increase in thickness and density of hair. Some people do not consider it as pathological. But it is psychogically disturbing in case of female. Ambras syndrome is a type of universal hypertrichosis affecting the vellus hair, where there is uniform overgrowth of hair over the face and external ear with or without dysmorphic facies.[3] Patients with Gingival fibromaatosis also have generalized hypertrichosis especially on the face.[4] Congenital hypertrichosis can occur due to fetal alcohol syndrome and fetal hydentoin syndrome.[5] Prepubertal hypertrichosis is seen in otherwise healthy infants and children. There is involvement of face back and extremities Distribution of hair shows an inverted fir-tree pattern on the back. More commonly seen in Mediterranean and South Asian descendants.[6] There is usually no hormonal alterations. Various genodermatosis associated with hypertrichosis as the main or secondary diagnostic symptom are:

Lipoatrophy (Lawrernce Seip syndrome)

Cornelia de Lange syndrome

Craniofacial dysostosis

Winchester syndrome

Rubinstein–Taybi syndrome

Mucopolysaccharidoses

Dystrophic epidermolysis bullosa

Porphyrias

Ostheochondrodysplasia

Gingival fibromatosis

Globoid leukodystrophy(Krabbe disease)

Piebaldism

Waardenburg syndrome

Hammerschlag-Telfer syndrome

Dystrichiasis-lymphedema syndrome

Oliver-MacFarlane syndrome

Incontinentia pigmenti achromians of Ito