Sjögren’s and Rare Skin Disorder Found in Chinese Woman, 69

Case study reports on patient with 2 rare autoimmune diseases

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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Two doctors use a giant magnifying glass to examine a patient in this case study illustration.

A 69-year-old woman in China was diagnosed with not one but two rare autoimmune diseases, according to researchers, who reported the case of Sjögren’s syndrome occurring alongside the skin disorder Lichen sclerosus as a reference for clinicians.

“Given the rarity of such cases, we report this case to provide a reference for dermatologists,” the team wrote.

Titled “Sjögren’s Syndrome with Lichen Sclerosus: A Case Report,” it was published in the journal Clinical, Cosmetic and Investigational Dermatology.

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Sjögren’s syndrome is an autoimmune disease mostly characterized by dry eyes and mouth, joint pain, and fatigue. While skin dryness is one of its most common symptoms — estimated to be present in 67% of patients — other organs and systems, such as the liver, lungs, and the nervous system, also can be affected by the condition.

Lichen sclerosus (LS) is a rare autoimmune disorder whose hallmark is itchy white skin patches, most commonly found in the genitals. In rarer cases, such patches may be found on other parts of the body.

The disease is more common among women, particularly those over age 50, than in men. If left untreated, LS can lead to skin scarring and also increase the risk for squamous cell carcinoma, a common form of skin cancer.

Now, researchers in China reported the case of an older woman with concomitant or co-occurring Sjögren’s and Lichen sclerosus.

The patient was treated at Beijing Chaoyang Hospital, Capital Medical University, for two completely different types of skin rashes. Her medical history included complaints of dry mouth for six years and dry eyes for more than three years. More than a year earlier, she had developed skin redness, called erythema, on the inner right thigh, but had no itching. The rash turned darker and larger, but resolved spontaneously within a week.

However, soon after, similar rashes erupted near the original location. In the previous months, she also had developed hardening of the skin on her back that shrunk with touch. The rash also moved and started enveloping the back of her neck.

During this time, she observed scattered dot-like white rashes on both arms, which gradually increased and blended into flakes.

She reported daily use of sodium hyaluronate eye drops, also known as artificial tears. Upon physical examination, she showed loss of pigmentation accompanied by thinning of the skin around the hairline, as well as on the forearms and face.

Additionally, she had finely wrinkled patches, mild redness, shrinkage, and some skin shedding in the back of the head and upper back. Multiple white patches of different sizes were present in the front of the neck, chest, and back. Several red ring-like patches were seen on the right shoulder blade, inner right thigh, and right buttock.

Closer examination of the genital area revealed pale red plaques around the labia majora (vulva outer lips) and clitoris. The skin surface was hardened and flaky.

Tissue analysis of the skin patches on the patient’s back confirmed increased thickness of the outer skin layer, accompanied by several changes, including the infiltration of immune cells, called lymphocytes.

A biopsy and follow-up analysis of a leg patch also showed these alterations. Fungus infection of the patient’s skin lesions was ruled out. Blood work showed she was positive for Sjögren’s anti-nuclear antibodies and SSA antibodies, which are typically found in people with Sjögren’s.

The Schirmer test, which evaluates whether the tear glands are producing enough tears, confirmed she had an impairment in tear production. She also showed low salivary production.

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The woman’s platelet and white blood cell counts dropped, and she showed signs of liver damage and impaired blood clotting. A high-resolution CT scan also showed signs of mild interstitial lung disease, which is characterized by lung scarring and inflammation, and liver scarring (cirrhosis).

Based on this clinical scenario, and following the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2016 criteria, she was diagnosed with LS combined with Sjögren’s syndrome.

She received into-the-vein treatment with glycyrrhizin, the primary active ingredient of the licorice root, along with daily oral tablets of compound danshen and glucosidorum tripterygll totorum. These two herb-extracted compounds are commonly used in traditional Chinese medicine.

However, as her white blood cell counts decreased, glucosidorum tripterygll totorum was stopped after one day. Instead, she was prescribed a topical tacrolimus ointment and mometasone furoate cream, which are commonly used to treat skin conditions. She applied both to the face and vulva. Other types of creams also were used for the trunk and legs, and for the remaining skin rashes.

The patient was discharged from the hospital one month later. Her skin rashes disappeared and the area of white thickened patches also were reduced.

However, new skin lesions appeared. Three months later, she was treated with hydroxychloroquine, a  disease-modifying anti-rheumatic drug (DMARD).  Within one month, her skin lesions had become smaller.

Although “her symptoms were controlled” following treatment, the researchers noted that “the patient’s condition [will require] long-term management.”

Such cases of co-occuring autoimmune diseases are rare, the team noted.

“Once suspected, a detailed patient medical history, systematic examinations are necessary,” the researchers concluded.