Skin by SLE, Lupus in the skin 4.58/5 (24)

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Lupus in the skin: Butterfly rash. Illustration: City Doctoral Internet - Own work, CC BY-SA 4.0

Definition

Different types of skin rash (skin symptoms) and oral cavity occur in most people Systemic lupus erythematosus (SLE) and are, along with symptoms of joints, muscles and tendons, the most common signs of disease of SLE.

Occurrence

Skin symptoms: 20% at onset, 5070% later (reference: Sontheimer RD 1996)

Different types of skin manifestations in lupus

Mucocutaneous rash

In the oral cavity, mucosal changes occur at 25-45%. Oral ulcerations (ulcers of the mouth) in 28%. These can be very different in size, location and shape.

  • Lips, gums, insides of the cheeks and palate may be attacked

Butterfly rash (malar rash)

Butterfly antler at SLE. Uva L, Autoimmune Dis (2012). CC BY 3.0

Eczema on both cheeks and over the nose. The eczema is reminiscent of the shape of a butterfly.

  • About 50% with SLE experiences a butterfly rash, most commonly after exposure to sun or bright light or with increasing disease activity. The skin feels warm and slightly swollen
  • The rash lasts for hours or days and often flares up

Diseases that are not associated with SLE, but which cause similar changes in the face are:

Sun rash

Rash on exposed areas. The rash may have different shape and intensity and occur at about 60% with SLE. Blonde, blue-eyed people have little pigment in the skin and are most at risk. The antibody SSA (Ro) is the predominant factor

  • Also Roseacea (differential diagnosis) is exacerbated by sun

Subacute cutaneous LE (SCLE)

SCLE occurs in approximately 10% with SLE, but the condition exists, similar to discoid lupus (see below), even without SLE

  • Photosensitivity (sun rash)
  • SSA (Ro) antibody
  • Typical symptom onset is small reddish, easily scaly dots that develop into either psoriatic-like or circular forms. The edges of the rash are typically red, sometimes crust-like. The skin above the shoulders, forearms, neck and upper body is most commonly attacked while the face is spared
  • SCLE is described in more detail here

Diskoid Lupus

Diskoid lupus. Ranginwala AM, J Oral Maxillofac Pathol (2012) CC BY-NC-SA 3.0

Diskoid lupus is detected in up to 25% at SLE, but also occurs as a separate skin disease without simultaneous SLE. In these cases, 5 - 10% will develop SLE over time

  • Characteristics of discoid lupus are reddish, flaky changes in the skin or in the scalp
  • The most commonly found changes in the face, neck and scalp
  • Often left scars or pigment changes
  • Diskoid lupus is ten times more common than SLE

Different types of Diskoid Lupus

  • Mucosal (mucosa)
  • Verrusous (wart-like)
  • Hypertrophic
  • Telangiectasia-like

Round (Annular) plaques (circular, dry spots) at Lupus can be similar Psoriasis

Lupus profundus / Lupsus panniculitis

Lupus panniculitis. Bednarek A, Postepy Dermatol Alergol (2015). CC BY-NC-ND 3.0

Panniculitis with abcess-like, necrosis of fatty tissue under the skin

  • Both face body and arms can be attacked
  • Leaves deep soaks that resemble fat loss after injection with cortisone
  • Associated with diskoid lupus at 70%
  • ANA detected at 75%
  • DNA antibodies are rare
  • Some receive treatment with Thalidomide

Chilblain lupus erythematosus

Chilblain lupus in young girl. Bansal S, Goel A - Indian Dermatol Online J (2014). CC BY-NC-SA 3.0

Blue-livid plaque / noduli on fingers

  • Antibodies that often occur: ANA, SSA (Ro)
  • Occurs at 20% with SLE
  • Similar changes are seen Aicardi-Goutieres Syndrome (AGS)
  • Treatment
    • Cortisone creams during periods
    • CellCept (mycophenolate) has been reported to work in some cases (reference: Gouillon L, 2017)

Lupus Tumidus

Lupus tumidus. Muylate BPB, An Bras Dermatol (2016). CC BY 4.0

Erythematous plaques, most often on the body, middle of the arms and legs

  • Photosensitivity (sun eczema) in light-exposed areas
  • "Intermittent cutaneous lupus" (coming and going)
  • Unspecified eczema:
    • Urticaria / hives-like,
    • Non-itchy, chronic urticaria 44%
  • Rarely typical of Systemic Lupus Erythematosus (SLE)
  • Antibody: ANA of 30%, SSA (Ro) / SSB (La) 5%, non-DNA
  • Tissue sample (Histological): Mucin deposition
  • Associated with Hypocomplement urticaria vasculitis (HUVS)
  • Treatment with hydroxyclorokine (Plaquenil)
  • The state can be identical or overlapping with Jessner's disease

STING mutation

  • SLE-like changes in skin and vasculitis in children

Lupus Pernio

Lupus Pernio is not related to Lupus, but is a form of Sarcoidosis of the skin

Hair and scalp

  • Hair loss (Alopecia) 20-60%, different degree
    • Diffuse hair loss most common, reddish scalp, not scar, but discoid lupus gives scars
  • "Lupus Hair": Rough, dry 30%. The reason may be the disease itself, but medications and other factors may also play a role

Nail disorders

The nails can be attacked at 25% with SLE

  • Splint Bleeding
  • Hyperkeratosis
  • Dilated vessels in nail-fold
  • Pigment changes

Leg ulcers

Can be detected at approx. 10% at SLE

Tissue test Skin biopsy (Punch Biopsy)

  • Light-exposed area:
    • Positive (Impact of immunoglobulin and compliment in dermo-epithelial transition zone at 90%, but many "false positive"
  • Non-light-exposed:
    • Less sensitive, but more specific for SLE. In the case of discoid lupus, a complement of unaffected skin is not expected

Prevention and treatment of skin reaction

The purpose of treatment is to prevent permanent damage to the skin. Such damage may be dark spots with too much pigment or pigment loss, visible blood vessels (teleangial lesions), hair loss or scars.

  • Effective prevention will prevent permanent damage to most people
    • Sensitive persons must protect the skin from the sun. Protection is important on the beach, sea, lakes, especially in the middle of the day (at 10-15) when the sun is strongest.
    • High factor sunscreen (30 or more) should be used.
    • Sunscreen clothing may also be applicable.

Medical treatment is a specialist task and is often done in collaboration between rheumatologists and dermatologists. Proper use of various medications on the skin is relevant, but adjustment of the other SLE treatment must be considered.

  • Avoid nicotine
  • Sunscreen
  • Hydrochlorochloroquine (Plaquenil)
  • Steroids (Prednisolone)
  • Dapson is used in rare cases against bully SLE
  • Thaliodomide in rare cases against lupus profundus
  • Retinoids in rare cases against hypertrophic discoid lupus
  • Treatment directly on the skin
    • Steroids are the first choice, possibly with occlusion
    • Tacrolimus (Protopic)
      • Protopic ointment is immunosuppressive, (Do not use if liver infection, Note: herpes zoster risk, Alcohol intolerance), Thin layer outside of mucous membranes. Improvement by 1 week. Adults 0,1% x twice daily (children 0,03%)
    • Pimecrolimus (Elidel)
    • Imiquimod
  • Drugs that are being tested against skin lupus: rituximab, ruxolitinib, belimumab
  • Vitamin Ddeficiency (often at lupus) should be corrected

CLASI index

The degree of skin damage and severity of skin lupus can be assessed by CLASI index (reference: Kuhn A, 2010)


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