Genetically transmitted syndrome characterized by
disorders of hair (short anagen growth phase of hair follicles) and teeth
Short (or Loose) Anagen Syndrome; Trichodental Syndrome;
Trichodental Dysplasia-Microcephaly-Mental Retardation Syndrome.
Unknown but probably not
uncommon (often misdiagnosed as alopecia areata or trichotillomania). Only
reported in whites; more common in females than in males. Autosomal
Suspected by “gentle hair pull” that yields much more
hairs than the zero to two hairs per pull in normal patients; confirmed
microscopic examination. Affected patients do not feel discomfort as hairs
Onset between 2 and 5 years of age. The most
common dental abnormalities are missing teeth, peg-shaped incisors, and
shell teeth. The hair is sparse, brittle, and slow-growing because of a
short anagen phase of the hair cycle (hair cannot grow long and affected
patients almost never need to have their hair cut). Eyebrows and eyelashes
are typically curly. Microcephaly with biparietal narrowing and a thin nose
are usually seen. Mild mental retardation is common.
Careful manipulation of the airway must
be provided to avoid damaging potentially brittle teeth. Craniofacial
abnormalities must be evaluated for abnormal airway anatomy that could
render face-mask ventilation and/or laryngoscopy difficult. Patients
with mental retardation may be uncooperative and sedative premedication as well as
the presence of the primary caregiver for induction of anesthesia may be helpful.
Alopecia Areata: Autoimmune disease resulting in varying amounts
of patchy hair loss (can be a variant of vitiligo).
Pseudopelade of Brocq: Scarring alopecia resembling alopecia
areata resulting in dermal atrophy (“footprints in the snow”), mostly
because of lichen planopilaris and chronic cutaneous lupus erythematosus.
Telogen Effluvium: Nonscarring alopecia with diffuse hair shedding
caused by metabolic or hormonal stress or by drug absorption.
Tinea Capitis: Superficial fungal infection of the skin of the
scalp, eyebrows, and eyelashes.
Barraud-Klenovsek MM, Trueb RM: Congenital hypotrichosis due to short
anagen. Br J Dermatol
Giannotti A, Digilio MC, Albertini G, et al: Sporadic trichodental dysplasia
with microcephaly and mental retardation. Clin Dysmorphol