Congenital hypertrichosis lanuginosa

Lalit K Gupta, Ashok Kumar Khare, Asit Mittal, Anubhav Garg

 Department of Dermatology, Venereology and Leprosy, RNT Medical College, Udaipur, Rajasthan, India

Correspondence Address:
Lalit K Gupta
A- 3, Sai Villa, Opposite Head Post Office, Madhuvan, Udaipur- 313001, Rajasthan
India

A 3-month-old male infant of non-consanguineous parents, born full term by normal vaginal delivery, was seen with excessive hair growth over entire body since birth. None of the family members had abnormal hair growth. Mother denied intake of alcohol or any other medication during pregnancy. There was no history of seizures in infant. The developmental milestones were normal for his age. He weighed 5 kg. The head circumference and crown-heel length were 41 and 57 cm, respectively. Dermatological examination revealed dense growth of gray to light brown, silky hair over most of the body [Figure - 1]. Hair was relatively profuse and long over face [Figure - 2], axillae, extremities and genitalia [Figure - 3]. The hair over scalp was dark and coarse [Figure - 2]. The palms, soles [Figure - 3] and lips were spared. Nails and mucosae were normal. Systemic examination did not reveal any abnormality. The diagnosis of congenital hypertrichosis lanuginosa (CHL) was made on the basis of above findings.

Figure 1 :Generalized hypertrichosis lanuginosa affecting most of the body

Figure 2 :Profuse growth of long, gray to light brown hair on face with dark and coarse hair on the scalp

Figure 3 :Marked hypertrichosis lanuginosa affecting the genitalia and lower limbs with sparing of soles

The first description of CHL appeared in the German literature in the 1870s, and by 1969, 32 cases had been reported. Another report published in 2008 mentions that fewer than 50 patients have been reported in the literature. The condition is characterized by excessive hair growth, usually since birth. The hair gradually lengthens until early childhood. The entire skin, apart from palms and soles, is covered by silky hair, which may be 10 cm or long. Long eyelashes and thick eyebrows are conspicuous features. The overall effect gives the face a "dog" or "monkey" appearance. The fetal pelage in CHL is not replaced by vellus and terminal hair, but persists, grows excessively and is constantly renewed throughout life in contrast to the acquired form, where previously normal follicles of all types revert at any age to the production of hair with lanugo characteristic. In the majority of the patients, this condition only causes an esthetic problem; however, there are cases in which there has been an association with hypo- or anodontia, and even early dentition, defects of the ear, glaucoma, pyloric stenosis, skeletal abnormalities, and exceptionally, physical and mental retardation and photophobia. Some affected individuals are normal at birth and sometimes for the first few years of life, before the universal replacement of other types of hair by lanugo. Once established, the hypertrichosis is permanent, but some diminution of hairiness of trunk and limbs may be noted in later childhood. The treatment of this condition is frustrating. There have been reports on cosmetic benefits from shaving, Nd:YAG laser and long pulsed ruby laser for hair removal.