From Dupre's page:
Both CHL and Ambras syndrome share the characteristics of excessive and abnormal hair growth with a consistent pattern of areas that are spared. In both disorders, hair is found only in areas where it is usually present. The palms, soles, mucous membranes, dorsal terminal phalanges, labia minora, prepuce, and glans penis are spared (Suskind, 1971).
* Congenital hypertrichosis lanuginosa
* In CHL, most of the body is covered with fine, blond or unpigmented hair at birth.
* The length of the hair and its distribution may continue to increase until the individual is aged approximately 2 years.
* Classically, by adulthood, patients have begun to lose some or all of their excessive lanugo hair.
* No abnormalities of other organ systems are consistently associated with CHL, although single case reports note abnormalities such as supernumerary teeth, glaucoma, aortic and cardiac valve abnormalities, and macromastia (Partridge, 1987; Judge, 1991; Li, 1986).
* Ambras syndrome
* As Baumeister described, the entire body is covered with fine long hair, which spares only the palms, soles, and genitalia.
* Hypertrichosis characteristically occurs on the shoulders, face, nose, and ears.
* The forehead, eyelids, nose, cheeks, and preauricular regions are uniformly covered with hair, which can reach a length of several decimeters. The hair is longest over the spine. Most patients have hair in a characteristic shawl distribution on their back.
* The hair of the external auditory canal is typically long and thick, and it may hinder inspection of the auditory meatus.
* Abnormalities of the teeth may be present. Adontia may occur, with an absence of the upper molars and the premolar teeth and a lag in the development of the first and second dentition.
* Features associated with facial dysmorphism include the following:
* Triangular, coarse face
* Large intercanthal distance
* Broad palpebral fissures
* Long, prominent back of the nose and a round nose tip
* Large interalar distance
* Anteverted nares
* Short integumental lower lip
* Flat sulcus mentolabialis
* Other findings noted in solitary case reports include the following:
* Bushy eyebrows with hair darker and coarser than that of the rest of the face, shoulders, body, and extremities
* Hair on the forearms and legs, in excess of and darker than the rest of the hair on the extremity
* Six accessory nipples
It seems like this kid has Ambras syndrome, not yer standard congenital hirsutism.
The teeth thing seems to make some kind of sense, but the extra nipples is really... throwbackish. (there's a better word, but it's escaping me). |
