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Oral manifestations of Ehlers-Danlos syndrome
________ JADA__________ L E T T E R S
V irg in Isla n d s a n n u a l m e e tin g □ I am w riting to inform your readers that despite any rumors or inform ation to the contrary, organized dentistry is continuing here in the Virgin Islands. A lthough we can in no way downplay the terrible traum a and destruction that Hurricane Hugo caused on the islands, the members of the Virgin Islands Dental Association wish to assure our colleagues that the features that make us such a large tourist attraction remain. We are most concerned that the sensationalized reports of widespread looting and civil unrest will leave the impression that it is unsafe to travel here. T his is completely untrue. O ur hotels, restaurants, shops, and other attractions are open and happy to greet our visitors. We have continued with plans for our annual meeting to be held in February 1990. We look forward to reacquainting ourselves w ith our many friends and colleagues.
TO
T H E
E D I T O R
She was born to a gravida 3, para 3, 28year-old mother at 38 weeks’ gestation with a birth weight of 2.7 kg. cesarean section was performed because of breech presen tation and mild hydramnios. At birth, the cranial shape was dolichocephalic with a prom inent occiput. An unusual pattern with lateral notches was noted on the gingiva. There was no family history of EhlersDanlos syndrome. W hen exam ined a t 4 m o n th s, the patient’s weight was 5.5 kg (10th percen tile), and length 62 cm (25th percentile). T he dolichocephaly present at birth was no longer evident. T he m andibular gin giva was unusual with notches at the area corresponding to the future canines with fleshy gingival proliferation posterior to the notches (Fig 1). There was decided articular hypermobility especially evident in the metacarpophalangeal joints, wrists, and ankles. T he skin had an unusual velvety texture but was of norm al elastic ity. T he rest of the exam ination was normal.
HENRY E. KARLIN, DDS PRESIDENT, VIRGIN ISLANDS DENTAL ASSOCIATION
O ra l m a n ife sta tio n s of E hlers-D an lo s sy n d ro m e □ We read with great interest the article “Oral m anifestations of Ehlers-Danlos syndrome” by Drs. E. M. Sadeghi, P. R. Ostertag and A. Eslami (February). We would like to report an unusual oral finding in an Ehlers-Danlos syndrome patient that has not been reported before. A 4-month-old girl with floppy joints and abnormal gingiva came for treatment. 696 ■ JADA, Vol. 119, December 1989
An occlusal radiograph indicated nor mal tooth development. T he gingival abnormalities in this girl with Ehlers-Danlos syndrome were not associated with other dysmorphic features. We suggest that notches in the gingiva and gingival hypertrophy may be a feature of an Ehlers-Danlos syndrome in infancy, and should be added to the list of oral co m p licatio n s such as p erio d o n titis,
fragility of the oral mucosa, bleeding after toothbrushing, excessive bleeding after tooth extraction, presence of pulp stones in the pulp chambers and root canals, malformation of the radicular portion of the teeth, fragility of teeth, and frequent subluxation of the temporom andibular joint.14 ALEXANDER K.D. LEUNG, MBBS, FRCPC ROBERT L. BARKSY, DMD, MRCD(C) RAYMOND M. LEWKONIA, MB ChB, FRCPC DEPT OF PEDIATRICS AND ORAL MEDICINE, UNIVERSITY OF CALGARY, ALBERTA C H ILD REN ’S HOSPITAL, CALGARY, ALBERTA, CANADA
L -try p to p h a n □ Since publication of our article (“The effect of L-tryptophan supplem entation and dietary instruction on chronic myofas cial pain,” April), we have received several comments and letters. We would like to clarify our concerns in response to these letters and in response to a letter published in the June issue of The Journal. The letter from Dr. Ira L. Shapira illustrates a crucial difference between a well-intended but anecdotal approach and a scientific approach to the treatment of temporom andibular (TM) disorders. T he main point of our article was that all groups of patients improved, but the groups receiving L-tryptophan failed to show more improvement than the groups receiving the placebo. Little attention was given by Dr. Shapira to our conclusions as he gave his own method for using Ltryptophan. O ur objection to the letter is not his observation that patients get better with L-tryptophan. We agree with that obser v atio n as we rep o rted th a t p a tie n ts improve w ith L -tryptophan and with placebo. Our objection is the implied causal link between the L-tryptophan and the reduced pain. W ithout control groups, we cannot know whether the improvement arose from the drug, from a placebo, from an unreported but concom itant treatment such as counseling or attention, from natural remission of symptoms, or from some other unidentified cause. Thus, we
V irg in Isla n d s a n n u a l m e e tin g □ I am w riting to inform your readers that despite any rumors or inform ation to the contrary, organized dentistry is continuing here in the Virgin Islands. A lthough we can in no way downplay the terrible traum a and destruction that Hurricane Hugo caused on the islands, the members of the Virgin Islands Dental Association wish to assure our colleagues that the features that make us such a large tourist attraction remain. We are most concerned that the sensationalized reports of widespread looting and civil unrest will leave the impression that it is unsafe to travel here. T his is completely untrue. O ur hotels, restaurants, shops, and other attractions are open and happy to greet our visitors. We have continued with plans for our annual meeting to be held in February 1990. We look forward to reacquainting ourselves w ith our many friends and colleagues.
TO
T H E
E D I T O R
She was born to a gravida 3, para 3, 28year-old mother at 38 weeks’ gestation with a birth weight of 2.7 kg. cesarean section was performed because of breech presen tation and mild hydramnios. At birth, the cranial shape was dolichocephalic with a prom inent occiput. An unusual pattern with lateral notches was noted on the gingiva. There was no family history of EhlersDanlos syndrome. W hen exam ined a t 4 m o n th s, the patient’s weight was 5.5 kg (10th percen tile), and length 62 cm (25th percentile). T he dolichocephaly present at birth was no longer evident. T he m andibular gin giva was unusual with notches at the area corresponding to the future canines with fleshy gingival proliferation posterior to the notches (Fig 1). There was decided articular hypermobility especially evident in the metacarpophalangeal joints, wrists, and ankles. T he skin had an unusual velvety texture but was of norm al elastic ity. T he rest of the exam ination was normal.
HENRY E. KARLIN, DDS PRESIDENT, VIRGIN ISLANDS DENTAL ASSOCIATION
O ra l m a n ife sta tio n s of E hlers-D an lo s sy n d ro m e □ We read with great interest the article “Oral m anifestations of Ehlers-Danlos syndrome” by Drs. E. M. Sadeghi, P. R. Ostertag and A. Eslami (February). We would like to report an unusual oral finding in an Ehlers-Danlos syndrome patient that has not been reported before. A 4-month-old girl with floppy joints and abnormal gingiva came for treatment. 696 ■ JADA, Vol. 119, December 1989
An occlusal radiograph indicated nor mal tooth development. T he gingival abnormalities in this girl with Ehlers-Danlos syndrome were not associated with other dysmorphic features. We suggest that notches in the gingiva and gingival hypertrophy may be a feature of an Ehlers-Danlos syndrome in infancy, and should be added to the list of oral co m p licatio n s such as p erio d o n titis,
fragility of the oral mucosa, bleeding after toothbrushing, excessive bleeding after tooth extraction, presence of pulp stones in the pulp chambers and root canals, malformation of the radicular portion of the teeth, fragility of teeth, and frequent subluxation of the temporom andibular joint.14 ALEXANDER K.D. LEUNG, MBBS, FRCPC ROBERT L. BARKSY, DMD, MRCD(C) RAYMOND M. LEWKONIA, MB ChB, FRCPC DEPT OF PEDIATRICS AND ORAL MEDICINE, UNIVERSITY OF CALGARY, ALBERTA C H ILD REN ’S HOSPITAL, CALGARY, ALBERTA, CANADA
L -try p to p h a n □ Since publication of our article (“The effect of L-tryptophan supplem entation and dietary instruction on chronic myofas cial pain,” April), we have received several comments and letters. We would like to clarify our concerns in response to these letters and in response to a letter published in the June issue of The Journal. The letter from Dr. Ira L. Shapira illustrates a crucial difference between a well-intended but anecdotal approach and a scientific approach to the treatment of temporom andibular (TM) disorders. T he main point of our article was that all groups of patients improved, but the groups receiving L-tryptophan failed to show more improvement than the groups receiving the placebo. Little attention was given by Dr. Shapira to our conclusions as he gave his own method for using Ltryptophan. O ur objection to the letter is not his observation that patients get better with L-tryptophan. We agree with that obser v atio n as we rep o rted th a t p a tie n ts improve w ith L -tryptophan and with placebo. Our objection is the implied causal link between the L-tryptophan and the reduced pain. W ithout control groups, we cannot know whether the improvement arose from the drug, from a placebo, from an unreported but concom itant treatment such as counseling or attention, from natural remission of symptoms, or from some other unidentified cause. Thus, we