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Genetically transmitted syndrome characterized by disorders of hair (short anagen growth phase of hair follicles) and teeth growth.

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 dominant transmission.

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 are extracted.

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 143:612, 2000.  [PubMed: 10971339]
Giannotti A, Digilio MC, Albertini G, et al: Sporadic trichodental dysplasia with microcephaly and mental retardation. Clin Dysmorphol 4:334, 1995.  [PubMed: 8574424]

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