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