SKIN ISSUES

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ITH
L
UPUS

 

 

Skin LESIONS and SLE
Skin disorders are very common in Lupus

  • Cutaneous manifestation of lupus are as follow:
    • Discoid lupus: lesions present as urticarial patch, scaly patch (follicular plugging), scarred patch, with pigmentary changes.
    • The palms can be involved with atrophy, erosion or hyperkeratosis.
    • During acute flare up of systemic lupus erythematous, patient often develops transient maculopapular butterfly malar rash with slerash affecting both cheeks. (commonly known as the malar rash or mask)
    • In subacute lupus (SCLE), the eruption can be urticarial (annular), papulosquamous (psoriasiform).
    • Mucosal involvement is also commonly seen and these include ulceration or nosebleed.
    • Scalp involvement is not uncommon and both diffuse or scarring alopecia can be seen.
    • Vascular lesions seen in lupus include: Raynaud's phenomenon, nail fold telangiectasia and infarct, splinter haemorrhages, chilblain LE, acquired C1 esterase deficiency, vasculitis, urticarial vasculitis, purpura, thrombophlebitis, livedo reticularis, antiphospholipid syndrome, Degos syndrome and calcinosis.
    • Others less common cutaneous manifestations are Bullous LE, LE profundus, erythema multiforme.

 

DISCOID Lesions

  • The term discoid is a very confusing term which, unfortunately, is inappropriately used by many people, including physicians.
  • The term discoid simply means coin-shaped. cutaneous cheek rash
  • The scarring coin-shaped lupus lesion commonly seen on areas of the skin that are exposed to light has been termed discoid lupus erythematosus.
  • This term refers only to the description of the lupus lesion on the skin and should not be employed to distinguish cutaneous lupus from systemic lupus erythematosus.
  • A physician cannot determine whether or not a discoid lupus lesion on the skin is occurring in the presence or absence of systemic features just by examining the shape of the lesion.
  • This can only be done by taking a complete history and physical examination and interpreting the results of appropriate blood tests.

 

DISCOID vs SYSTEMIC LUPUS

  • Lupus erythematosus should be viewed as a continuum of a spectrum of this autoimmune disease.
  • At one end of the spectrum, in its most mild form, it is characterized by coin-shaped, scarring, skin lesions which we term discoid lesions. lesions on ears of lupus patient
  • At the other end of the spectrum are those systemic lupus erythematosus patients who have no skin lesions, but have systemic features.
  • People with only discoid lesions and no systemic features commonly have no auto-antibodies in their serum and their antinuclear or anti-DNA tests will be negative.
  • On the other hand, people with systemic lupus erythematosus are characterized by the presence of one or more types of auto-antibodies in their blood.
  • From reviewing the literature, it has been estimated that between 5 and 10% of patients initially presenting with only the coin-shaped lesions of discoid lupus will, with time, develop systemic features.
  • As noted above, approximately 20% of people with systemic lupus erythematosus will at the time of the initial presentation of their disease have discoid lupus lesions.
  • Therefore indicating that with time, a small percentage of those patients who only have discoid lupus lesions will eventually develop systemic disease
  • In addition to these coin-shaped, scarring lesions, there are several different types of discoid lupus lesions with which patients should be familiar.
  • Occasionally, the discoid lupus lesions may occur in the scalp producing a scarring, localized baldness termed alopecia.
  • At times, these discoid lesions may appear over the central portion of the face and nose producing the characteristic butterfly rash or 'malar rash'. sle facial rash
  • This type of lupus obviously has significant cosmetic implications.
  • The discoid lupus lesions may develop thick, scaly (hyperkeratotic) formations and are termed: hyper-keratotic or hyper-trophic, cutaneous lupus lesions.
  • Discoid lupus lesions may also occur in the presence of thickening of the layers of underlying skin, a condition which is termed lupus profundus.
  • sle rash on handsAt the present time, research indicates that discoid lupus lesions are the result of an inflammatory process in the skin in which the patients' lymphocytes play a major role.
  • This is in contrast to systemic lupus erythematosus, where auto-antibodies and immune complex formation are responsible for many of the clinical symptoms.

 

SUBACUTE CUTANEOUS Lesions

  • This type of specific lupus lesion was most recently described by Sontheimer and Gilliam during the late 1970's. scle rash on hands
  • This lesion is characterized as a non-scarring, erythematosus, or red, coin-shaped lesion which is very photosensitive, meaning it gets worse when exposed to UV light.
  • This type of lesion, which is characteristic of subacute cutaneous lupus, occurs in lupus patients who, approximately half of the time, demonstrate features of systemic lupus erythematosus.
  • Renal disease, however, is unusual in these patients.
  • These skin lesions also occur in people who only have clinical evidence of skin disease (discoid lupus), and do not show any symptoms of systemic lupus.
  • Approximately 70% of people with these lesions have anti-Ro antibodies. scle lesion on chest
  • scle rash on armThe subacute cutaneous lupus lesion can sometimes mimic the lesions of psoriasis or they can appear as non-scarring, coin-shaped lesions much like hives.
  • These lesions can occur on the face in a butterfly distribution, or can cover large areas of the body.
  • Unlike the discoid lupus lesions, these lesions do not produce permanent scarring, but can be of major cosmetic significance.

 

Non-Specific Lupus Lesions - ALOPECIA

hair loss in child

  • The non-specific lupus lesions include several forms of alopecia, or hair loss, which are not related to the presence of discoid lupus lesions in the scalp.
  • Systemic lupus patients who have been severely ill with their disease may over a period of time, develop a transient hair loss in which large amounts of hair evolve into a resting phase and fall out, being quickly replaced by new hair.
  • In addition, a severe flare of systemic lupus erythematosus can result in defective hair growth which causes the hair to be fragile and to break easily.
  • The hair is broken off above the surface of the scalp, especially at the edge of the scalp, giving the characteristic appearance termed "lupus hair".

 

VASCULITIS

  • Systemic lupus erythematosus patients may develop inflammatory disease of the blood vessels (vasculitis).
  • The cutaneous manifestations of vasculitis are varied. vasculitis on  arm
  • The lesions may appear as red welts involving large areas of the body.
  • These lesions can also present as small red lines in the cuticle nail fold or on the tips of the fingers or as red bumps on the legs.
  • vasculitis on the legIn addition, these red bumps may ulcerate.
  • At times, the blood vessels that are involved in this inflammatory process may be deep in the skin producing painful, red nodules.
  • These are usually found on the legs.

 

PHOTOSENSITIVITY ISSUES Malar Rash from Sun Exposure

  • Photosensitivity is a common feature of lupus erythematosus.
  • The overwhelming majority of specific lupus lesions, occur on sun-exposed areas.
  • In addition, approximately 40-70% of people with lupus will note that their disease process, including the skin disease, is aggravated by sun exposure.
  • Furthermore, people with subacute cutaneous lupus erythematosus, especially those who have anti-Ro (SSA) antibodies demonstrate pronounced photosensitivity.
  • It has been estimated that 90% of patients with systemic or discoid lupus who have anti-Ro (SSA) antibodies are photosensitive.
  • Furthermore, a number of these patients are so photosensitive that they will burn through window glass.
  • Window glass filters out sunlight in the sunburn spectrum and protects normal people from developing a sunburn.
  • However, window glass will not filter ultraviolet light of longer wavelengths and these wavelengths are capable of exacerbating the skin lesions in people with lupus with anti-Ro (SSA) antibodies.
  • Also there is clinical evidence that shows that ultraviolet light can induce flares in people with sle.
  • The way that ultraviolet light triggers these systemic flares, or leads to the development of skin lesion, is not known.
  • However, there is evidence that suggests that ultraviolet light is capable of leading to an increase in the number of auto-antigens to which the person is reacting.

 

TREATMENTS AVAILABLE

Treatment options
Treatment for skin issues with lupus may include:

  • Topical steroid creams.
  • The use of plastic wrapping over the skin to increase the absorption of steroid creams.
  • Injections of medication in the case of exceptionally thick skin lesions that don't respond to creams.
  • Anti-malarial drugs.
  • Other medications, such as those used for psoriasis.
  • Oral steroids if SLE is also present.

Avoid sun exposure
Staying out of the sun is perhaps the most crucial strategy for the management of discoid lupus. The ultraviolet radiation in sunlight can trigger or worsen an attack. Suggestions include:

  • Avoid exposing yourself to direct sunlight whenever possible.
  • Cover as much of your skin as you can with clothes such as long-sleeved shirts, trousers, gloves, broad brimmed hat and so on.
  • Always wear sunscreen lotion on all exposed areas of skin when you go outside.
  • Choose sunscreens that protect against both UVA and UVB.
  • Wear sunscreen even in winter or on cloudy days - any degree of ultraviolet radiation on the skin should be avoided.
  • Remember that ultraviolet radiation is not stopped by window glass, and is reflected off surfaces like concrete, snow and water.
  • Some fluorescent tubes emit ultraviolet radiation.
  • Wind and cold temperatures may affect some people with discoid lupus.

Where to get help

  • Your doctor

  • Dermatologist

  • Support Groups (lupus patients)

    Things to Remember

    • Discoid lupus is a chronic skin condition characterised by reddened scaly patches that develop in sun-exposed areas of the body such as the face and hands.
    • It is unclear whether discoid lupus is a separate disease, or a milder version of systemic lupus.
    • Staying out of the sun is perhaps the most crucial strategy for the management of discoid lupus.
    • Treatment options include topical steroids and anti-malarial medication. (however, be very careful with your research on meds, as some have irreversible occular side effects *for some patients* -i.e.: chloroquin)