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Old 11-30-2012, 10:54 AM   #1
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Lupus-specific Rashes

Lupus-specific Rashes (list isn’t exhaustive)


The CCLE rashes (Chronic cutaneous lupus erythematosus). Lesions favor photo-exposed skin. Most of the time labs are negative. Of patients presenting with CCLE lesions, only 5-10% progress to systemic lupus (SLE).

1. Discoid lupus erythematosus (DLE). Lesions are red, round (disc-like), thick, and adherent. Favors face, ears and scalp. Typically don’t itch or hurt. While DLE lesions are most associated with “cutaneous-only” lupus (meaning fewer than 4 criteria are met, so overall disease is viewed as “mild”), cosmetic damage can be profound, including pitting, scarring, depigmentation, and permanent hair loss.

2. Variants include---
  • Acral DLE (palmar/plantar). Targets palms of hands and/or feet. Painful and erosive.
  • Chilblains LE. Purplish or red swollen nodules most often on toes and fingers; often very painful or itchy. Cold is the most common trigger.
  • Hypertrophic lupus erythematosus (HLE), aka Verrucous LE, aka Hyperkeratotic LE. Resembles viral warts or skin cancer, are crusty and itchy. Favors face, arms and upper trunk.
  • Lupus erythematosus profundus (LEP), aka Lupus erythematosus panniculitis, aka Lobular panniculitis. Rare inflammation of subcutaneous fat below skin surface causing outer skin to thicken and raise. Most often on face. Kills fat cells and leaves residual dimpling.
  • Tumid lupus erythematosus (TLE). Marked red background on which hive-like bumps and patches may appear. Usually on trunk (V-neck is common), rarely on face. Typically very photosensitive. Heals without scarring or depigmentation.

The SCLE rashes (Subacute cutaneous lupus erythematosus). Lesions tend to be extremely photosensitive, and favor photo-exposed areas of arms, shoulders, neck, trunk and upper back (facial lesions are less common). 40% to 50% of patients with SCLE lesions meet 4 or more ACR criteria for systemic disease. Positive labs are common: ANA is positive in roughly 70%, anti-Ro is found in a high percent, anti-La less often. Tend not to itch. Scarring and depigmentation are rare.

1. SCLE annular, aka SCLE polycyclic. Immature form resembles red raised papules; this limited stage can occur for many cycles. Eventually evolves to the mature stage, in which new papules expand into annular (ring-shaped) circles with clear centers and slight scale (flakiness) on borders.

2. SCLE psoriasiform, aka SCLE papulosquamous. Lesions are red elevated areas of skin with distinct margins. Resembles psoriasis.

3. Neonatal lupus erythematosus (NLE). Temporary annular (ring-shaped) rash seen only in infants. Symptoms clear without treatment in 4-6 months. Babies are at small risk for congenital heart block.

4. Variants include---
  • Annular erythema associated with Sjogren’s syndrome. Annular (ring-shaped) lesions seen in Polynesian and Japanese patients.
  • Rowell syndrome. Erythema multiform-like lesions coupled with speckled-pattern ANA, positive RF, and sometimes positive anti-La.

The ACLE rashes (Acute cutaneous lupus erythematosus). ACLE lesions occur in 50% or more of those with systemic disease at some point in time; of them, malar is the most common. ANA is almost always positive, and anti-ds-DNA is positive in 60% to 80%.

1. Malar, aka butterfly. In this most “classic” of lupus rashes, skin over cheeks and bridge of nose reddens, forming a butterfly shape but sparing the nasal folds. Typically occurs at start of disease, thereafter can accompany flares. May be UV-triggered or appear spontaneously. Typically doesn’t itch. Can last hours, days, weeks, or longer. From appearance alone is difficult to distinguish from acne rosacea.

2. Other ACLE rashes---
  • Widespread photosensitive erythema. ACLE lesions can also occur on forehead, around eye sockets, sides of neck, arms, legs, and trunk.
  • Bullous LE, aka Pemphigoid LE. Raised fluid-filled blisters that can reach the size of large chickenpox. Can be widespread. Can cause dehydration (requires prompt medical intervention).

The DILE rashes & skin problems (Drug-induced lupus erythematosus). Unlike other forms of lupus, DILE lupus rarely requires treatment; it’s almost always “treated” only by identifying and discontinuing the offending drug(s). Skin manifestations in DILE align pretty closely with the CCLE, SCLE, and ACLE rashes already described above.

1. Drug-induced CCLE. May cause Discoid lupus erythematosus (DLE) lesions and Tumid lupus erythematosus (TLE). See remarks in CCLE section.

2. Drug-induced SCLE. May cause SCLE annular (polycyclic) rashes and SCLE psoriasiform (papulosquamous) rashes. See remarks in SCLE section.

3. Drug-induced SLE. May cause photosensitivity. Also purpura [see remarks below]. Also erythema nodosum, red painful lumps, typically on front of legs below the knees. Also urticarial vasculitis, painful, itching burning lesions lasting > 24 hours. Also necrotizing vasculitis, which causes tissue death.


Other Skin Issues that aren’t specific to Lupus (list isn’t exhaustive)


1. Alopecia (hair loss). In lupus, occurs in approx. 70% of patients at some point. May occur as patches or overall thinning. General hair loss from flares is followed by new hair growth, but loss from discoid lesions is often permanent. Breakage along scalp edge is called “lupus hair” or “lupus fringe”.

2. Calcinosis. Calcium deposits under the skin. Can be painful and may leak a white liquid. Can occur in other conditions. In lupus, can occur after steroid injections or due to kidney failure.

3. Hives (urticaria) or welts. In lupus, occur in approx. 10% of patients at some point. Red raised lesions that typically itch, often greatly. Will turn white when pressed. Causes other than lupus include food allergies, vasculitis, medications, etc.

4. Livedo reticularis. Red-to-violet lacy fishnet pattern just under skin surface, caused by abnormal blood flow. More prominent on extremities, especially legs. Tends to worsen in cold. Usually isn’t cause for alarm. Is also seen in antiphospholipid syndrome (APS), but may occur without lupus or APS.

5. Mucosal ulcerations. In lupus, occurs in approx. 25% of patients at some point. May affect mouth, nose, more rarely eyes and vaginal tissue. Occurs in both cutaneous and systemic lupus. Not considered dangerous. Pain is possible, ranging from extreme discomfort to none.

6. Nail changes. Nails may crack or fall off. Skin at base of nail may swell and turn blue or red.

7. Pigmentation changes. Lightening or darkening occurs in approx. 10% of patients. An extreme example is seen when vitiligo is present: it causes total whitening.

8. Purpura, petechiae, and ecchymoses. Purpura is the general name for red or purple areas just below skin surface caused by leaking blood vessels. In lupus, occurs in approx. 15% of patients at some point. Small spots are petechiae, larger areas are ecchymoses. Favor the lower legs. Also may be caused by low blood platelets (thrombocytopenia), medications, and other conditions.

9. Raynaud’s phenomenon. Affects approx. 1/3 of lupus patients but also occurs without lupus. Blood vessels in hands and feet spasm, restricting blood flow. Main trigger is cold, also stress. Color change may be red, white, blue, or any combination of the three. Pain, numbness and tingling are possible; and in extreme cases, gangrene.

10. Vasculitis. In cutaneous vasculitis, skin manifestations vary widely from small red-purple spots, to hive-like bumps, to purplish-reddish lines, to black spots, to red welts. All tend to be painful and may be widespread. Lesions don’t turn white when pressed. Favors lower legs, but can appear on hands, fingertips, or nail folds. Cutaneous vasculitis isn’t serious, except if ulceration or gangrene occurs. In systemic vasculitis, eyes, heart, brain, GI tract, or kidneys may be affected; by definition, systemic vasculitis is serious. Both cutaneous vasculitis and systemic vasculitis can occur with or without lupus.


Pre-approved by Administrator

Last edited by Administrator; 11-30-2012 at 12:01 PM.

 
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