Ackerman syndrome or interstitial granulomatous

dermatitis Dermatitis is inflammation of the skin, typically characterized by itchiness, redness and a rash. In cases of short duration, there may be small blisters, while in long-term cases the skin may become thickened. The area of skin involved can ...

is a familial

syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek σύνδρομον, meaning "concurrence". When a syndrome is paired ...

of fused molar roots with a single canal (

taurodontism Taurodontism is defined as the enlargement of pulp chambers with the furcation area being displaced toward the apex of the root. It cannot be diagnosed clinically and requires radiographic visualization since the crown of a taurodontic tooth a ...

hypotrichosis Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarr ...

, full upper lip without a cupid's bow, thickened and wide

philtrum The philtrum ( la, philtrum from Ancient Greek ''phíltron,'' lit. "love charm"), or medial cleft, is a vertical indentation in the middle area of the upper lip, common to therian mammals, extending in humans from the nasal septum to the tuber ...

, and occasional juvenile

glaucoma Glaucoma is a group of eye diseases that result in damage to the optic nerve (or retina) and cause vision loss. The most common type is open-angle (wide angle, chronic simple) glaucoma, in which the drainage angle for fluid within the eye re ...

. It was described by James L. Ackerman, A. Leon Ackerman, and A. Bernard Ackerman.

Signs and symptoms

* Fused molar roots * Single root canal * Juvenile glaucoma * Sparse body hair * Distinct facial features: full upper lip, absence of

cupid's bow The Cupid's bow is a facial feature where the double curve of a human upper lip is said to resemble the bow of Cupid, the Roman god of erotic love. The peaks of the bow coincide with the philtral columns giving a prominent bow appearance to the ...

, thick

Syndactyly Syndactyly is a condition wherein two or more digits are fused together. It occurs normally in some mammals, such as the siamang and diprotodontia, but is an unusual condition in humans. The term is from Greek σύν, ''syn'' 'together' and δά ...

* Increased pigmentation of finger joints *

Clinodactyly Clinodactyly is a medical term describing the curvature of a digit (a finger or toe) in the plane of the palm, most commonly the fifth finger (the " little finger") towards the adjacent fourth finger (the " ring finger"). It is a fairly common is ...

of fifth finger.

Diagnosis

Treatment

References

External links

* Rare genetic syndromes Syndromes affecting teeth Syndromes with craniofacial abnormalities Syndromes with dysmelia {{genetic-disorder-stub