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Palmar dermatoglyphics as diagnostic tool: Mayer-rokintansky-kuster-hauser syndrome
2 Dept. of Anatomy, KS Hegde Medical Academy, Mangalore - 575 018, India
Dept. of Anatomy, Centre for Basic Sciences, Kasturba Medical College, Bejai, Mangalore - 575 004
|How to cite this article:
Kumar A, Manou S J. Palmar dermatoglyphics as diagnostic tool: Mayer-rokintansky-kuster-hauser syndrome. Indian J Dermatol Venereol Leprol 2003;69:95-96
AbstractThe congential anomalies involving Mullerian duct dysgenesis, the MRKH (Mayer-Rokitansky-Kuster-Hauser) syndrome overlaps a 'community of syndromes' which share, apart from a complete absence of uterus and vagina, associated presentations that include developmental abnormalities of skeletal, renal, dental and other dysmorphias.
First described more than 400 years ago, this complement of anomalies now rechristened, the MRKH syndrome has been extensively studied. Found to occur once in every 4000 females; estimated on its incidence vary from 1/5000 to 1/20,000.
Despite its fairly wide prevalence, this syndrome has remained largely underdiagnosed or cryptic. Social and cultural perceptions have perforce driven, the wide existence of the very personal handicap underground and unacceptable.
Accidentally discovered during routine examination, during secondarily associated medical problems, the mullerian agenecies syndrome has been subject of a wide range of clinical and laboratory investigations.
The oddity in the syndrome is that the patient appears absolutely normal and that it presents with no genetic marker.
We herein report the palmar dermatoglyphic and doctylographic features of a patient with MRKH syndrome. However interpretation, show conclusively, at least in this single case that the MRKH syndrome can be diagnosed through its characteristic dermatography patterns.
In our view, routine dermatographic study of all live females births may lead to not only an early but also a cost effective diagnostic method.
The history of dactylography as a fool-proof tool for identity establishment has special significance to India-the first ever fingerprint bureau, established for medico legal purpose in the world was at Culcutta. Today, digital dermatoglyphics form an indispeseble and reliable tool for criminal investigation at international level. The patterns and characteristics of whorls, loops, ridges and creases of the palm and some are exclusively individualistic in their presentation. While exhaustive data banks containing millions of individual prints act as a ready sources for criminal investigations, in our view, the value of dermatoglyphics as an additionally reliable medical diagnostic tool has been largely overlooked.
In this brief case report, we present our findings on a rare congenital affliction, the (Mayer-Rokitansky-Kuster-Hauser) MRKH syndrome, with hope that forensic scientists could extend their expertise and experience in dermatoglyphics to aiding clinicians in medical diagnostic investigation.
Materials and Methods
Bilateral palm prints and fingerprints of a 34 years old female, a known case of MRKH syndrome, were recorded and analysed.
The finger prints showed, on the right side loop, arch, arch, loop, loop and, arch, arch, arch, loop, whorl for the left. The A-B ridge count on the right was 25 and 32 on the left. The ATD angle was 410 for the right and 390 for the left [Table - 1].
Females generally have lower frequencies of whorls and radial loops and a higher number of arches. Recorded data reveals 42.6% whorls, ulnar loop, 2.2% radial loop, and 3.1 % arches in Indian females.
The thenar area on both sides were uncharacteristic. The hypothenar areas however showed the very rare ′open fields′ straight ridge pattern on both sides.
The area V (H) shows open fields (no. 42), open fields (no. 43), consisting of series of almost straight ridges, said to exceedingly rare. This variety is classified as ′O′ [Figure - 1]
The fact that palm print of both right and left hands in this case displayed the rare ′0′ type makes this record of extreme dermatoglyphic significance. That palm print study alone could possibly exclusively diagnose this rare, MRKH syndrome is an exciting prospect.
The congenital anomalies involving Mullerian duct dysgenesis, the MRKH syndrome overlaps a "community of syndromes" which share, apart from complete absence of uterus and vagina, associated presentations that include developmental abnormalities of skeletal, renal, dental and other dismorphias.
First described more than 400 years ago, this complement of anomalies, now rechristened the MRKH syndrome has been extensively studied, and found to occur once in every 4000 female births. Estimates on its incidence vary from 1/5000 to 1/20,000. The oddity in the syndrome is that the patient appears absolutely normal and that it presents with no generic markers.
Usually diagnosed incidentally or accidentally the patient with MRKH syndrome presents no externally identifiable markers, Permitting the afflicted to lead apparently normal lives. The cryptic and latent prevalence of the syndrome in substantial sections of female population has been reported to be one of the major causes of a wide range of psychosomatic or psychiatric problems. Early diagnosis, at birth itself, using dermatoglyphics as a tool, may not only help integrate these isolated sections of the population, but also allow early corrective surgical interventions.
To our knowledge this presentation is the first ever report on the peculiarities of palmar dermatoglyphics of this syndrome in the world. It would be interesting to compare these prints with other patients with MRKH.