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Sjögren’s Syndrome

  • Chapter
Autoimmune Diseases of the Skin

Summary

Cutaneous manifestations of Sjogren’s syndrome (SS) include:

  • • Dry skin (xerosis)

  • • Vasospastic disorders ranging from Raynaud’s to acrocyanosis

  • • Macular, papular and vesicular rashes

  • • Infections such as varicella zoster

  • • Palpable and non-palpable purpura due vasculitis, and

  • • Embolic lesions and thrombotic lesions

  • • Acute or chronic thrombosis with lymphedema

  • • Other associated skin conditions including urticaria or allergic skin eruptions

Complaints of dry skin occur in about 50% of SS patients. It is unclear whether or not the xerosis is due to infiltrate of the eccrine or sebaceous glands, or dysfunctional response of the residual glands. In many biopsies from SS patients, dryness of the skin has been associated with lymphocytic infiltrates in the eccrine glands. Similar to SLE patients, antibody and complement fixation is often detected clinically “normal” skin.

However, the extent of dryness of the skin and the clinical appearance termed “xerosis” is often more severe than that expected for the degree of lymphocytic infiltration (and glandular destruction).

A common finding on deeper skin biopsy is “non-specific perivascular lymphocytic infiltrates.” Immuno-histologic studies have also indicated an increase in peri-vascular dendritic cells of both the mesenchymal and Plasmacytoid types. These histologic findings on SS skin biopsy are so common that the pathologist may often only mentions them “in passing,” while they emphasize that no leukocytoclastic vasculitic changes were present. But these “perivascular” lymphocytic (and dendritic) cell infiltrates may be the crucial factor in xerosis of SS.

Treatment of SS is generally symptomatic, with most patients requiring treatment only for dryness. Adequate explanation is essential; many subjects, for example, may not realize that their central heating or air conditioning creates a drying environment or that a windy day is likely to make their eyes dryer. Simple measures such as humidifiers, sips of water, chewing gums, and simple replacement tears will be adequate in the majority of subjects. The rest should be told of the wide range of artificial fluids available and encouraged to try several different formulations.

Treatment of other manifestations of SS has been influenced by our treatment of other connective tissue diseases. The most serious (and fortunately rare) complications such as vasculitis and neurologic disease probably require immunosuppression with drugs such as cyclophosphamide, as in systemic lupus erythematosus.

Because many lupus symptoms mimic other illnesses, are sometimes vague and may come and go, lupus can be difficult to diagnose. Diagnosis is usually made by a careful review of a person’s entire medical history coupled with an analysis of the results obtained in routine laboratory tests and some specialized tests related to immune status. Currently, there is no single laboratory test that can determine whether a person has lupus or not. To assist the physician in the diagnosis of lupus, the American Rheumatism Association issued a list of 11 symptoms or signs that help distinguish lupus from other diseases. A person should have four or more of these symptoms to suspect lupus. The symptoms do not all have to occur at the same time

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  1. Allergy and Rheumatology Clinic, Scripps Memorial Hospital and Research Foundation, 9850 Genesee Ave, #860, La Jolla, CA, 92037, USA

    Robert I. Fox MD, PhD

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  1. Robert I. Fox MD, PhD
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  2. Carla M. Fox
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Michael Hertl

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Fox, R.I., Fox, C.M. (2011). Sjögren’s Syndrome. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_13

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