Physicians and dentists sharing information about rarely seen diseases can help future health-care specialists when patients seek help with unusual symptoms.
Dental professionals recently received information from periodontists from Case Western Reserve University School of Dental Medicine and the King Hussein Medical Center at the Royal Medical Services in Jordan.
They describe the first dental case study of two sisters with a rare genetic disorder, called Haim-Munk Syndrome (HMS), in the August issue of Clinical Advances in Periodontics in an article titled, “Papillon-Lefèvre or Haim-Monk Syndrome? Report on Two Sisters in a Consanguineous Family.”
HMS is an aggressive gum and skin disease that causes infants to lose their primary teeth as early as age 1, and their permanent teeth by age 17. Eventually, the girls in the case study will require dentures or implants.
The sisters, now 10 and 13, began showing symptoms as infants: thick, white skin coatings on the palms of their hands, soles of their feet, elbows, knees and around their mouths.
In the 35 years of his periodontal practice, Nabil Bissada, chair and professor in the Department of Periodontics at the Case Western Reserve School of Dental Medicine, has seen a couple of cases of a similar syndrome, called Papillon-Lefèvre Syndrome (PLS). PLS also results in early tooth loss and skin problems.
Reem H. Dababneh, his former postdoctoral fellow from King Hussein Medical Center, contacted Bissada about the sisters’ rare condition in 2012.
Bissada recalled two early papers about HMS—first reported in 1965—that led him to suspect Debabneh’s patients had that syndrome.
An estimated one in a million people have PLS, and fewer than 100 people with HMS have been reported in the medical literature, according to Orphanet.com.
Most of the information about PLS or HMS focuses on the unusual skin condition. Yet HMS involves the additional symptoms of white skin patches on elbows, knees and around the lips.
Such syndromes originated within a family tree of a small community of Jewish people from Cochin, India, on the Malabar Coast who later migrated to Israel. Only two cases have been reported that are unrelated to this family, but both cases have family ties to India.
The sisters’ parents are double cousins who have origins in the Cochin family and also migrated from India to Israel.
Bissada contributed to the study with Dababneh and Sami E. Jebrin, from King Hussein Medical Center, and Antwan T. Khouri, a private practice dentist in Jordan.