Solve the Medical Riddle: She Has Blisters in Her Mouth and on Her Body, Fourth Week

By Marie Savard MD

Editor’s note: Welcome to our ThirdAge feature that gives you a chance to play medical sleuth as we share the details of what happened when a patient presented with a problem that stumped the physician at first.

The first week of this riddle, the patient reported her symptoms. The doctor referred her to a dermatologist associated with a university. The second week, the dermatologist proceeded with the examination. Both physicians used the components of the classic S-O-A-P notes, which are as follows:

S=Symptoms or Chief Complaint

O=Objective Findings

A=Assessment or Analysis

P=Treatment Plan or Recommendation

Last week, we let you know what some people had suggested as possible diagnoses. This week, the doctor will reveal the actual diagnosis. Then we’ll begin a new riddle for the following month!

The Doctor Reveals the Diagnosis

Congratulations, Athena L.! All of the guesses were really good, but you nailed it. Zoe did indeed have pemphigus vulgaris. This is the other dermatological emergency that Mille R.’s cousin couldn’t remember.

One clue to the actual diagnosis was that the IDIF test revealed circulating intercellular antibodies that are detected in 80% to 90% of patients with pemphigus vulgaris. The titer (amount) of circulating antibody correlates with disease course.

Pemphigus vulgaris presents with oral lesions in 50% to 70% of patients, and almost all patients have mucosal lesions at some point in the course of their disease. Mucosal lesions may be the sole sign for an average of five months before skin lesions develop, or they may be the sole manifestation of the disease. Her scalp lesions were called vegetating lesions. This type of crusting or thickened area tends to occur more frequently where two skin areas rub together, such as the groin areas and on the scalp or face.

Treatment:

The goal in treating pemphigus vulgaris is to reduce blister formation, encourage healing of existing blisters, and decide on the lowest dose of medication necessary to keep the disease under control. Therapy should be individualized, taking into account each patient’s health history. Even so, patients may continue to have blisters even when undergoing the best possible treatment. Zoe wasn’t happy to hear that considering the upcoming wedding, yet it is tough reality.

Zoe’s doctor started her on corticosteroids, which can keep patients with pemphigus vulgaris alive. Before the advent of steroids, most patients with pemphigus vulgaris died! However, serious side effects from steroids such as osteoporosis and susceptibility to infections are possible.

Many experts now use rituximab, the anti-CD20 antibody, as first- or second-line therapy, or steroid–sparing Dapsone, but Zoe’s doctor decided that he would only consider prescribing one of those medications it if she didn’t have a quick response to the steroids.   

Intravenous immunoglobulin is another effective treatment for pemphigus vulgaris in patients who don’t respond to systemic steroids. Photodynamic therapy is another possibility.

Fortunately, Zoe did respond well to the steroids. Her dermatologist and her PCP coordinated her care with an ophthalmologist to rule out ocular problems because Zoe was taking high-dose steroids for a long time.

Zoe did have oral blisters so she needed regular care from her dentist.

Zoe was warned that patients on systemic steroids should be sure to have adequate vitamin D and calcium intake, but no dietary restrictions were recommended other than staying away from spicy foods, tomatoes, and orange juice, as well as hard foods such as nuts, carrots, and apples that could injure the inside of her mouth.

The doctor also advised Zoe to avoid anything that could traumatize her skin and cause blisters, including tight or scratchy clothes. He recommended nontraumatic exercises such as swimming to minimize Zoe’s stress.

The vegetating type of response on Zoe’s scalp can be more resistant to therapy, so her dermatologist warned her the scalp could take more time than the skin lesions to go away.

Prognosis is usually better in childhood than in adulthood, but the doctor held out hope that Zoe’s condition would be adequately controlled.

As Zoe said, “I got over the vanity issue and just felt thankful to alive! Actually, I’ve been lucky in so many ways regarding my appearance. I’ve had no involvement with my hands or nails so my daughter and I are going to go for a pre-wedding mani-pedi. And I can still wear my contact lenses because my eyes haven’t been affected at all. My daughter helped me pick out an attractive mother-of-the-bride dress with a high neck and long sleeves that will cover any remaining blisters. I also discussed with my wonderful hairstylist how to deal with the scalp issues so that no one will be the wiser. I did gain weight because of the steroids and I have a puffy face. I can’t complain, though! I’m still here to celebrate my daughter’s wedding and look forward to the possibility of becoming a grandmother. Life is good!”

Come back to ThirdAge.com next Thursday when we’ll introduce a new medical riddle!

Marie Savard, M.D., a former Medical Contributor for ABC News and a frequent keynote speaker around the world, is one of the most trusted voices on women’s health, wellness, and patient empowerment. She is the author of four books, including one that made the Wall Street Journal list of the best health books of 2009: “Ask Dr. Marie: What Women Need to Know about Hormones, Libido, and the Medical Problems No One Talks About.” Dr. Marie earned a B.S. in Nursing and an M.D. degree at the University of Pennsylvania. She has served as Director of the Center for Women’s Health at the Medical College of Pennsylvania, technical advisor to the United Nations’ Fourth World Conference on Women in Beijing, advisor to the American Board of Internal Medicine Subcommittee on Clinical Competency in Women’s Health, health columnist for Woman’s Day magazine, and senior medical consultant to Lifetime Television’s Strong Medicine. Please visit DrSavard.com.