A Nodular Rash
I recently saw a patient who presented to the clinic with itchy nodules of both arms for a period of weeks. She was not taking any new medications or creams. She did not have a subjective fever or otherwise feel ill. She had used over the counter antihistamines and a prescription steroid cream that she had at home- all did not benefit her.
She was previously seen by a dermatologist and allergist for a “sun rash”. The patient had a diagnosis of Sjogren’s Disease. Please note: “syndrome” changed to “disease”. As an older physician, I have always called it a “syndrome”, as per the previous literature. This was not mentioned in either the dermatologist’s or allergist’s reports.
On exam, the patient had no objective fever, and her other vital signs were normal. Two firm nodules were noted on both upper arms. The remainder of the physical exam was normal.
When I saw the diagnosis of Sjogren’s Disease, an autoimmune abnormality associated with various manifestations, including rashes and light sensitivity, I had my “aha” moment. I ordered blood work and placed the patient on a week of oral steroid therapy.
The blood work showed a mildly active inflammatory disorder, and positive markers for Sjogren’s Disease. The markers are auto-antibodies directed against proteins in the cell nucleus. Blood tests for Systemic Lupus Erythematosus (SLE), which is an autoimmune disease that also causes light sensitivity, were negative.
Her rash was nearly completely resolved in one week. She was alerted to the fact that the rash may recur off steroids, and that she should let us know if that happened.
Sjogren’s Disease is associated with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). This is collectively known as the sicca syndrome, which the patient had. Sjogren adversely affects the salivary glands, reducing the amount of saliva production. It can be associated with other autoimmune diseases such as rheumatoid arthritis or SLE. It is also a risk factor for developing lymphoma.
Sjogren’s Syndrome is associated with dermatologic disease, although this is not a prominent abnormality, and can therefore be overlooked. https://sjogrens.org/blog/2020/the-sun-sjogrens-how-to-protect-yourself#:~:text=Share,refer%20only%20to%20UVB%20rays.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8303716/
The prevalence of Sjogren’s Disease is between 0.01 to 0.09 percent of the European and Asian population.
With this patient we have an uncommon manifestation of an uncommon disease, which is why it was initially overlooked with her previous dermatologic problem. Diagnosis in medicine frequently involves looking for possible connections of different diseases. Always look for it.