The immune system is a complex protective mechanism of the human body on constant guard against an arsenal of invaders that threaten the body’s ability to thrive. Autoimmune disorders occur when the body’s immune system turns against itself, attacking it, as if it were a foreign pathogen—the body becomes allergic to itself.
In this phase, the immune response causes excessive inflammation with a destructive effect on cells and tissues. No tissue or organ is exempt from autoimmune disease, and there are vast implications in the mismanagement of these diseases that could possibly trigger the further development of associated autoimmune diseases, and other systemic or cutaneous diseases. The effects and symptoms of autoimmune diseases may also vary greatly from one individual to another with regard to the severity and responsiveness in symptomology, the disease progression and treatment.
There are many theories now postulated regarding the offenders for the increase in autoimmune diseases, however common delineations include both the environment and genetic connections. A close genetic relationship exists among autoimmune diseases, explaining dispersing in individuals and families, as well as a common pathways of disease.
Prevalence of autoimmune diseases
According to a recent study, the prevalence and incidence of autoimmune diseases, such as lupus, celiac disease and type 1 diabetes, is on the rise—and researchers at the Center for Disease Control and Prevention are not certain of the influx. Between 2001 and 2009, the incidence of type 1 diabetes increased by 23%, according to the American Diabetes Association.1
There is a staggering increase in the number of cases of autoimmune diseases, and many studies persuade that environmental factors may play a significant role in the development and progression of diseases.
There are several studies emerging that implicate autoimmune diseases may share a common root by hiding in the intestinal lining even years before the symptoms appear and manifest.2 This appears with great confidence by the nature of the staggering increase of celiac disease and other gut-related disorders. The National Institutes of Health and the University of Chicago Celiac Disease Center estimate that 1 in 133 people are affected by celiac disease.1
Researchers at Ben-Gurion University of the Negev in Beersheba, Israel have shown that chronic stress increases the susceptibility to an autoimmune disease similar to multiple sclerosis, which is characterized by damage to the nervous system in the brain,3 identifying the probability of stress triggers and immunity displacement.
Research and statistics have demonstrated a commonality in the link between autoimmune disease and the correlation of skin symptoms or some degree of dermal manifestation, such as rashes, plaques, lesions and general inflammation of the skin. This factor implicates the enormous impact the immune system has on the skin’s presentation, functionality and immunity—advising the esthetic community to be keenly observant of all aspects of heath issues facing clients, as subclinical conditions undetected by the eyes may greatly impact the services skin care professionals provide—no matter an individual’s skills or experience.
The immune system—no simple task
The immune system is a vast and intricate network that requires an in-depth explanation of its many components, functions and relationships beyond what may be contained in this article.
For the intent of this feature, the following key points are expressed with regard to abridging the primary components of the immune system, how autoimmunity evolves and its correlation with lupus.
Lymphoid tissue. Lymphoid tissue is an integral part of the immune system, representing a ratio of approximately 3% of a person’s body weight. This specific tissue includes the lymph nodes, circulating lymphocytes and fixed lymphoid tissue—the spleen.
Bone marrow. Bone marrow is a source of thymus cells (T cells) and bursa-derived cells (B cells)—lymphocyte cells that migrate to the thymus, which is the organ that is responsible for the production of T cells. T cells provide cellular immunity and account for approximately 70% of lymphocytes. T cells are highly significant because they are equipped to recall memory of foreign antigens and proteins, and then transmit alerts to the body to formulate protective responses. T cells—natural cells, and B cells—killer cells, account for approximately 10–15% of the lymphocytes that eliminate what is foreign to the body. Natural killer cells have the ability to destroy target invaders without requiring memory.
Immunoglobulin G (IgG). IgG is a gamma globulin antibody, which is a major contributor to antibody responses if the body is being attacked. There are five gamma globulins that protect the body, however the IgG is of prime consideration. These antibodies characterize the majority of autoimmune diseases, and the IgG study is one of the primary lab tests ordered when a severe allergy, chronic illness or autoimmune disease is suspected. The immunoglobulin E (IgE) is also significant, as it attaches to mast cells and mediates the aspect of allergic reactions.
Cytokines. Cytokines are hormone-like substances that contribute immensely in the growth and development of cells. One of the most important relationships of cells is that of cluster of differentiation 4 (CD4) cells that boost cytokines and promote inflammation—they are also referred to as T-1 or T helper cells, interleukin-2 and tumor necrosis factor. Cytokines are of great importance in lupus due to the fact that they are altered in the presence of this disease.
Inflammation assault—lupus
In healthy individuals, the key fighters for the body’s defense include neutrophils, lymphocytes, macrophages and monocytes, which are all involved to protect against inflammation and the immune process. The development of lupus occurs when there is faulty signaling, communication and initiation in the immune system, and if individuals are subjected to certain factors.
Neutrophils. Neutrophils are white blood cells responsible for mediating inflammation, and may increase exponentially in the individual with lupus because of a reaction with cytokines and blood plasma. Chemicals that the individual may be exposed to are drawn closer to the site of inflammation due to the development of adhesion-type molecules from the chemicals themselves. This factor also involves the weakened status and relationship of all immune cells coming to the aid to combat the antagonist.
Lymphocytes. Lymphocytes— also white blood cells—go through an altered function in lupus. T helper cells become more active and the body becomes less responsive to T suppressor cells. This results in the body’s B cells creating antibodies in an individual’s own tissues. Thereby initiating a highly accelerated signaling of an inflammatory response. The cells in turn create autoantibodies and attack its own cells and tissues. Autoantibodies are one of the key signs of the lupus patient and other autoimmune diseases. The immune system overall is impaired with a decrease in the numbers of white blood cells.
Macrophages. Macrophages are key regulators of inflammation and immune response. They are considered resident phagocytic cells in both lymphoid and non-lymphoid tissue. Macrophage cells have receptors that render them very effective at the process of phagocytosis and induce the activity of cytokines.
Monocytes. Monocytes circulate in bone marrow, blood and the spleen, and house pathogen receptors that mediate infection from blood to tissue in the presence of infection.
Lupus overview and skin signs
Skin involvement is one of the most prevalent and recognizable presentations of lupus such as the malar or “butterfly” rash and other non-specific skin lesions. Lupus may also affect multiple organs and systems in the body, as an individual’s immune system may attack various cells causing a wide variety of signs and symptoms. The activity of lupus presents an increase of antibodies against various protein fractions and a resultant scenario, inflammation of connective tissue—where the skin is often involved. (See Autoimmune Diseases That Affect Skin on Page 51.)
The integumentary system is constantly exposed to external and internal sources of antigens, bacteria and free radicals that can affect the skin’s immune system (SIS). The SIS is located throughout the various dermal layers and is able to detect foreign proteins, such as bacteria and viruses. When an antigen comes in contact with immune cells, the cells produce antibodies that position themselves around the antibody in a locking fashion.
The other immune cells recognize this new development and advance to destroy the invader. Mast cells then send out powerful chemical signals to release histamines, which then activate other aspects and components of the immune system. Histamines have a range of activity that may produce a reaction from mild to severe that presents inflammation, erythema, irritation and possibly edema. These key observations identify the uniqueness of lupus and autoimmune disease in general, and give insight to the understanding of the complexities of how the immune system governs the body’s defenses. With regard to dermal immunity, it should be apparent that the skin of the lupus patient presents specific requirements to maintain barrier function, disease prevention, protection and vitality.
Chronic cutaneous lupus (CCLE). The most common form is discoid lupus erythematosus (DLE), in which 70–80% of patients exhibit skin signs.4
CCLE may present coin-shaped lesions on the cheeks, face, and sometimes the ears and neck. Once the lesions have healed after a flare, the skin may atrophy and appear thinner, and possibly present obvious pigmentation irregularities.
Fatty lesions called lupus profundus, may also appear under the skin—they feel somewhat hard to the touch, and may leave scars after destroying fat cells.
Subacute cutaneous lupus erythematosus (SCLE). SCLE may present distinct-shaped lesions that resemble psoriasis plaques, which present a scaling at the edge of the lesions. SCLE is considered a non-scarring rash that can coexist with both discoid and systemic lupus. SCLE is not often associated with systemic disease, but the patient may experience associated joint disease.
Acute cutaneous lupus (ACLE). Primarily seen in patients with active systemic disease, the hallmark “butterfly” or malar rash characterizes this most common form of ACLE. The malar rash is often treated in dermatology with triamcinolone or clobetasol—fluorinated steroid salves. Obstacles in this therapy include patient compliance regarding the overuse of medicaments, which in turn increases exasperation of the rash and thinning of the skin. Traditional topical treatment calls for the use of occlusive steroidal gels and ointments, and abstaining from sun exposure. Lesions may also appear on the legs, trunk or arms, and are extremely sensitive to sunlight. These lesions generally do not present scarring, however may present hypo- or hyperpigmentation. Other manifestations of ACLE include mouth ulcers, temporary hair loss and hives—all that occur during a disease phase or flare.
Systemic lupus erythematosus (SLE). SLE may affect organs of the body—heart, lungs, joints, brain, blood cells and blood vessels. If there is damage to the blood vessels, small violet red-colored lesions may appear on the lower legs called vasculitis.
Related cutaneous involvement
Other conditions that present in the skin of lupus patients include the following.
Calcinosis cutis. These are milia-like lesions that are calcium deposits under the skin. These deposits can be painful and may extrude a discharge. Calcinosis can develop from a reaction to steroid injections and are to be treated by a physician.
Alopecia and hair loss. Severe systemic lupus may instigate a temporary pattern of hair loss that is then replaced by new hair growth. A severe lupus flare can result in fragile hair that breaks easily and may present a characteristic frayed appearance—termed “lupus hair.”
Raynaud’s syndrome. Raynaud’s is a condition in which the blood vessels in the hands and feet initiate constricting spasms resulting in restricted blood flow. Lupus-related Raynaud’s usually results from inflammation of nerves and blood vessels, and occurs more frequently in cold climates and temperatures. Raynaud’s may be identified by the tips of the fingers or toes appearing red, white or even blue with accompanying pain, numbness or tingling. These patients should be advised to wear warm socks and gloves regularly in compromising temperatures.
Livedo reticularis and palmar erythema. These conditions are caused by abnormal rates of blood flow through the capillaries and small arteries. A bluish, lace-like mottling will appear beneath the skin, especially on the legs, presenting a fine net-like appearance. Like Raynaud’s syndrome, this condition tends to be worse in cold weather and temperatures.
Mucosal ulcerations. These are ulcerated lesions in the mouth or nose that can be caused by both cutaneous lupus and systemic lupus. It is important to differentiate lupus ulcers from herpes simplex virus 1 (HSV-1) cold sores, which may be brought on by the use of immunosuppressive drugs.
Petechiae. Petechiae are tiny red spots on the skin that occur from low platelet numbers in the blood count, resulting from a condition called thrombocytopenia, typically appearing on the legs. Although thrombocytopenia is common in lupus, serious bleeding as a result of the low number of platelets usually does not occur.
Contributing factors
There is a long list of various factors that are attributed to contributing to the development of lupus and lupus flare-ups.
Alfalfa, also known as lucerne. Alfalfa contains the amino acid L-canavanine, which has been found to trigger lupus.
Crystalline silica. Crystalline silica creates pulmonary inflammation and fibrosis, and has well-known inflammatory effects.5
Early life exposures. Health events early on, such as asthma, may program the immune system.
Echinacea. Echniacea may act as an immune system stimulant. Individuals with lupus have an overactive immune system, and several studies have shown that echinacea can induce human immune cells to secrete pro-inflammatory cytokines, which are known to play a role in lupus disease.6
Epstein–Barr virus (EBV). This virus is closely associated with lupus—studies have illustrated a higher EBV viral load in patients with lupus.
Ineffective phagocytosis. Ineffective phagocytosis and poor cell clearance may contribute to the development of lupus. As cells come to the end of their function they are cleared through the body efficiently involving certain immune cells. Monocytes and macrophage cells have been shown to exhibit abnormalities in lupus patients. Other factors, such as glycoproteins—which play a critical role in immune response—have been found to be deficient, missing entirely or functioning abnormally in lupus patients. It has been postulated that autoimmunity may develop as a result of the immune system being exposed for longer periods to material from dead and dying cells that is not disposed of efficiently.7
Medications. Typically, the lupus skin flare resolves when the drug is discontinued that is contributing to the development. These drugs may include: non-steroidal anti-inflammatory drugs; oral contraceptives; procainamide; hydralazine; minocycline and other antibiotics; and some biologics.4
Ultraviolet light. A gene-environment relationship and susceptibility is suggested. UV light is shown to be a potent inhibitor of DNA methylation, which has been shown to cause changes in gene expression that may contribute to lupus.4
Additional factors include:
- Aromatic amines—hair color products, tobacco smoke;
- Aspartame8, 9;
- Beta blockers;
- Cadmium exposure;
- Cigarette smoking, secondhand smoking;
- Estrogen;
- Food coloring;
- Hydrocarbons—pristine;
- Lead exposure;
- Medication preservatives;
- Mercury exposure;
- Pesticide exposure;
- Silicone; and
- Xenoestrogens.
Medical treatment of lupus
It is important to note that certain medications may precipitate lupus as in the case of drug-induced lupus (DIL), as well as other skin sensitivities. Many of the medications listed are not approved by the FDA for cutaneous lupus.10 Medications for the Treatment of Lupus represents possible medications that are used for the treatment of lupus and is not a complete list in its entirety.
The esthetic approach
The esthetic treatment of the lupus patient may be aligned with many of the same treatment approaches and protocols to that of the oncology patient with regard to sensitivity and inflammation thresholds. As with any sensitive or compromised skin, treatment should be directed at maintaining barrier function, reducing sensitivity, preventing transepidermal water loss (TEWL), controlling pruritus, preventing infection and ensuring protection and comfort. Esthetic treatments are advised to be performed with a practical holistic approach recognizing the functioning of the SIS, and the acknowledgement that the skin will behave and perform as compromised because of immune status.
Pharmacology assessment is also a key aspect of client evaluation regarding the esthetic treatment of any kind for the lupus patient. The observation of the immune relationship of the body and the skin cannot be stressed enough, factoring in the variables of pharmacological behavior on a subclinical level. Esthetic treatment of lupus should be discussed with the client’s rheumatologist, dermatologist and or general physician to identify possible markers of medication reactions in conjunction with skin care treatments.
Esthetic care practice recommendations include:
- Perform soothing and anti-inflammatory protocols;
- Introduce red light-emitting diode (LED) therapy10;
- Avoid chemical peels, microdermabrasion and physical exfoliation;
- Avoid heated instruments, hot steaming and aggressive extractions;
- Apply soothing masks that contain zinc or titanium dioxide;
- Apply alginate or collagen sheet-type masks that are easy to remove;
- Utilize cool spray technology;
- Select products used for rosacea, sensitive skin or oncology patients;
- Use cool water-soaked cotton compresses, beauty globes or similar comfort cooling devices with appropriate temperatures to help reduce inflammation;
- Recommend SPF 30 or above with titanium and zinc;
- Avoid glycolic and other alpha hydroxy acids unless cleared with physician;
- Avoid heavily fragranced products and artificial coloring;
- Avoid enzymes with lipase that may interact with medications;
- Select soothing occlusive moisturizers with anti-inflammatory components;
- Offer mineral and camouflage makeup instruction for malar rash concealment;
- Ensure client education regarding their home-care regime and compliance—keep home care simple and direct; and
- Use clinical protocol by communicating with the client’s physician with regard to progress and treatment questions.
The lupus patient may benefit greatly from both traditional and progressive products and treatments. (See Topical Ingredients—Products for Esthetic Treatment.) Estheticians should encourage alliances with physicians that treat lupus patients, and receive training in specialty, holistic and integrative esthetics to offer lupus patients a noninvasive approach to the care and management of the their skin, self esteem and overall wellness.
REFERENCES
- www.medicalnewstoday.com/articles/246960.php
- www.todaysdietitian.com/newarchives/021313p38.shtml
- nocamels.com/2013/05/lets-relax-researchers-show-stress-leads-to-increase-in-autoimmune-diseases
- www.niams.nih.gov/about_us/Mission_and_Purpose/lupus_plan.asp#Appendix_A
- www.ncbi.nlm.nih.gov/pubmed/17075811
- www.lef.org/protocols/immune-connective-joint/lupus/page-02
- www.ei-resource.org/illness-information/related-conditions/lupus/
- www.thelupussite.com/practical_information/aspartame.html
- articles.mercola.com/sites/articles/archive/2005/10/06/aspartame-killing-us-by-degrees-part-i.aspx
- www.rheumatologynews.com/fileadmin/content_pdf/san/scms_pdf/vol27_i4_Light-Emitting_Diodes.pdf
(All websites accessed Nov 21 2014)
General References
- www.nature.com/nbt/journal/v18/n10s/full/nbt1000_IT7.html
- JD Bros, Skin Immune System: Cutaneous Immunology and Clinical Immunodermatology, CRC Press, Boca Raton, FL (2004)
- RE Jordon, Immunologic Diseases of the Skin, Appleton & Lange (1991)
- S Moore, Lupus: Alternative Therapies That Work, Healing Arts Press, Rochester, VT (2000)
- DJ Wallace, The Lupus Book: A Guide For Patients & Their Families, Oxford University Press, New York, NY (2012)
- www.americanskin.org/resource/lupus.php
- articles.mercola.com/sites/articles/archive/2005/10/06/aspartame-killing-us-by-degrees-part-i.aspx
- lpi.oregonstate.edu/sp-su98/immune.html
- www.healthy.net/scr/Article.aspx?Id=3349
- www.merryclinic.com/lupus/treatment.htm
- www.dermnetnz.org
- www.hopkinslupus.org
- www.nlm.nih.gov/medlineplus/lupus.html
- www.lef.org//Protocols/Immune-Connective-Joint/Lupus/Page-01?source=search&key=lupus
- www.lupusny.org/about-lupus/fight-lupus-body-and-mind/lupus-and-your-skin
- www.ncbi.nlm.nih.gov/pmc/articles/PMC2887389/
- www.lupus.org/answers/entry/how-lupus-affects-skin
(All websites accessed Nov 21 2014)
