How Do You Cope When a Disease Causes Your Body To Attack its Own Skin

Higher Incidence of Pemphigus In Ashkenazi Jews

Months To Diagnose: Rebecca Oling enjoys symptom-free weeks with husband Scott Oling.
Courtesy of Mary Lou Johnson
Months To Diagnose: Rebecca Oling enjoys symptom-free weeks with husband Scott Oling.

By Maia Efrem

Published August 06, 2013, issue of August 16, 2013.
  • Print
  • Share Share
  • Single Page

About once a month, Rebecca Oling develops painful lesions inside her mouth, a symptom of a rare skin disease she’s been battling since 2004. With medication it takes the lesion roughly a week to dissipate. But Oling, in remission, considers herself one of the lucky ones.

Feeling stressed by the deaths of her stepfather and her husband’s grandfather months apart, Oling, 44, developed her initial symptoms. “I thought they were canker sores that wouldn’t go away, and it was very painful,” said Oling, a librarian and faculty member at the State University of New York at Purchase. “I went to the dentist; the dentist sent me to my primary care physician. Then the infectious disease doctor could not help, and another dentist didn’t, either.” Oling, who was pregnant during the process, finally went to an oral pathologist, who performed a biopsy before diagnosing her with a condition known as pemphigus. “I was very lucky. I got it in my mouth and my nasal cavity, and it stayed there,” she said.

Click to see the rest of the section, Click for more stories about genetics.

Pemphigus — a Greek word meaning “bubble”— is a rare, autoimmune blistering disease that affects 1 person out of every 100,000, with a higher incidence among Ashkenazi Jews and those of Mediterranean descent. According to the International Pemphigus & Pemphigoid Foundation, in Sacramento, Calif., it is estimated that 30,000 to 40,000 people in the United States are currently living with pemphigus and 49,000 more are diagnosed worldwide each year.

Pemphigus vulgaris, the most common form of the disease, often starts in the mouth as a fragile sore or a lesion. It can be limited to the mouth, but in most cases patients go on to have blisters and erosions elsewhere on the body. From the first sign of the disease to diagnosis, a patient will typically see about five different doctors over many months.

Pemphigus is not a genetic disease in the traditional sense of Mendelian inheritance. where those with inherited genes have higher chances of developing a particular disease. Carriers of pemphigus have a variant of an HLA gene, essential in producing proteins that initiate the immune system’s response to invasive pathogens.

“Not everyone who carries the protein develops the disease,” explained Dr. Animesh Sinha, an expert on pemphigus at the University at Buffalo’s School of Medicine and Biomedical Sciences “but those who do essentially have their immune system turn on itself, attacking healthy skin cells. The disease onset is usually in the fourth or fifth decade, but can occur in childhood.

“Often the story in autoimmune disease is that something traumatic happens and this person was predisposed to a disease and something tipped them over,” said Sinha. A death in the family, or major surgery, could be just such an environmental factor.

A report published in the March 2012 issue of the Journal of Investigative Dermatology and spearheaded by Israeli scientists from Tel Aviv Sourasky Medical Center found that those of Jewish descent were 40 times more likely to develop the disease than the rest of the world’s general population.

“Your immune system attacks the cells that keep the skin together, and eventually the skin starts coming apart, like mortar in the wall,” said Will Zrnchik, CEO of the IPPF. “Massive blistering makes some people look like they’ve been dragged behind a car. On other people it looks like chicken pox with dots. It may be everywhere. If untreated, those little dots start playing connect-the-dots. Four or five one day may look like 15 or 20 the next.”


The Jewish Daily Forward welcomes reader comments in order to promote thoughtful discussion on issues of importance to the Jewish community. In the interest of maintaining a civil forum, The Jewish Daily Forwardrequires that all commenters be appropriately respectful toward our writers, other commenters and the subjects of the articles. Vigorous debate and reasoned critique are welcome; name-calling and personal invective are not. While we generally do not seek to edit or actively moderate comments, our spam filter prevents most links and certain key words from being posted and The Jewish Daily Forward reserves the right to remove comments for any reason.





Find us on Facebook!
  • "Israel should not let captives languish or corpses rot. It should do everything in its power to recover people and bodies. Jewish law places a premium on pidyon shvuyim, “the redemption of captives,” and proper burial. But not when the price will lead to more death and more kidnappings." Do you agree?
  • Slate.com's Allison Benedikt wrote that Taglit-Birthright Israel is partly to blame for the death of American IDF volunteer Max Steinberg. This is why she's wrong:
  • Israeli soldiers want you to buy them socks. And snacks. And backpacks. And underwear. And pizza. So claim dozens of fundraising campaigns launched by American Jewish and Israeli charities since the start of the current wave of crisis and conflict in Israel and Gaza.
  • The sign reads: “Dogs are allowed in this establishment but Zionists are not under any circumstances.”
  • Is Twitter Israel's new worst enemy?
  • More than 50 former Israeli soldiers have refused to serve in the current ground operation in #Gaza.
  • "My wife and I are both half-Jewish. Both of us very much felt and feel American first and Jewish second. We are currently debating whether we should send our daughter to a Jewish pre-K and kindergarten program or to a public one. Pros? Give her a Jewish community and identity that she could build on throughout her life. Cons? Costs a lot of money; She will enter school with the idea that being Jewish makes her different somehow instead of something that you do after or in addition to regular school. Maybe a Shabbat sing-along would be enough?"
  • Undeterred by the conflict, 24 Jews participated in the first ever Jewish National Fund— JDate singles trip to Israel. Translation: Jews age 30 to 45 travelled to Israel to get it on in the sun, with a side of hummus.
  • "It pains and shocks me to say this, but here goes: My father was right all along. He always told me, as I spouted liberal talking points at the Shabbos table and challenged his hawkish views on Israel and the Palestinians to his unending chagrin, that I would one day change my tune." Have you had a similar experience?
  • "'What’s this, mommy?' she asked, while pulling at the purple sleeve to unwrap this mysterious little gift mom keeps hidden in the inside pocket of her bag. Oh boy, how do I answer?"
  • "I fear that we are witnessing the end of politics in the Israeli-Palestinian conflict. I see no possibility for resolution right now. I look into the future and see only a void." What do you think?
  • Not a gazillionaire? Take the "poor door."
  • "We will do what we must to protect our people. We have that right. We are not less deserving of life and quiet than anyone else. No more apologies."
  • "Woody Allen should have quit while he was ahead." Ezra Glinter's review of "Magic in the Moonlight": http://jd.fo/f4Q1Q
  • Jon Stewart responds to his critics: “Look, obviously there are many strong opinions on this. But just merely mentioning Israel or questioning in any way the effectiveness or humanity of Israel’s policies is not the same thing as being pro-Hamas.”
  • from-cache

Would you like to receive updates about new stories?




















We will not share your e-mail address or other personal information.

Already subscribed? Manage your subscription.