Lupus erythematosus (LE) is an autoimmune connective tissue disorder that can affect one or several organs. Circulating autoantibodies and immune complexes are due to loss of normal immune tolerance and are pathogenic. Clinical features of LE are highly variable. LE nearly always affects the skin to some degree.

1. LUPUS ERYTHEMATOSUS
ACHU EDISON PUSHPAKUMARI
4th Year Medical Student
BSMU Chernivtsi Ukraine

2. Lupus Erythematosus
• Lupus erythematosus (LE) is an autoimmune connective
tissue disorder that can affect one or several organs.
• Circulating autoantibodies and immune complexes are due to loss
of normal immune tolerance and are pathogenic.
• Clinical features of LE are highly variable.
• LE nearly always affects the skin to some degree

3. Signs & Symptoms
• Symptoms vary from person to person, and may come and go.
• Almost everyone with lupus has joint pain and swelling.
• Some develop arthritis.
• Frequently affected joints are the
fingers, hands, wrists, and knees.

4. Signs & Symptoms (continue…)
Common symptoms include:
• chest pain when taking a deep breath
• Joint pain
• Oral ulcer
• fatigue
• fever with no other cause

5. Signs & Symptoms (continue…)
• general discomfort, uneasiness, or ill feeling (malaise)
• hair loss
• sensitivity to sunlight
• skin rash – a "butterfly" rash in about half people with SLE
• swollen lymph nodes

6. Signs & Symptoms (continue…)
Other symptoms depend on which part of the body is affected:
• Brain and nervous system: Headaches, numbness, tingling, seizures,
vision problems, and personality changes
• Digestive tract: Abdominal pain, nausea, and vomiting
• Heart: Abnormal heart rhythms (arrhythmias)
• Lung: Coughing up blood and difficulty breathing
• Skin: Patchy skin colour and fingers that change colour when cold
(Raynaud phenomenon)
• Kidney: Swelling in the legs, weight gain

7. Classification
• Lupus erythematosus may manifest as systemic disease
or in a purely cutaneous form also known as incomplete
lupus erythematosus.
• Lupus has four main types:
• systemic
• discoid
• drug-induced
• neonatal

8. • Chronic, multisystem, inflammatory disorder of
autoimmune etiology, occurring predominantly in young
women.
• Common manifestations may include arthralgias and
arthritis, malar and other rashes, pleuritis or
pericarditis, renal or CNS involvement, and hematologic
cytopenias.
Systematic Lupus Erythematosus (SLE)

10. Discoid lupus erythematosus (DLE)
• Chronic skin condition of sores with inflammation and
scarring favouring the face, ears, and scalp and at times on
other body areas.
• These lesions develop as a red, inflamed patch with a
scaling and crusty appearance. The centre areas may appear
lighter in colour with a rim darker than the normal skin.
• Discoid lupus erythematosus can be divided into localized,
generalized, and childhood discoid lupus erythematosus

11. Discoid Lupus Erythematosus

12. Drug-induced lupus erythematosus (DILE)
• Autoimmune disorder (similar to systemic lupus erythematosus
[SLE]) caused by chronic use of certain drugs.
• These drugs cause an autoimmune response (the body attacks its
own cells) producing symptoms similar to those of SLE.
• There are 38 known medications to cause DIL but there are three
that report the highest number of cases:
• hydralazine,
• procainamide, and
• isoniazid.

13. Neonatal lupus erythematosus
• Neonatal lupus erythematosus is the occurrence of systemic lupus
erythematosus (SLE) symptoms in an infant born from a mother with
SLE, most commonly presenting with a rash resembling discoid lupus
erythematosus, and sometimes with systemic abnormalities such as
complete heart block or hepatosplenomegaly.
• The infants have no skin lesions at birth, but develop them during the
first weeks of life. Neonatal lupus is usually benign and self-limited.

14. Neonatal Lupus Erythematosus

15. Cutaneous Lupus Erythematosus
• Cutaneous LE comprises several chronic and relapsing LE-specific and LE-
nonspecific inflammatory conditions. There can be some overlap.
LE-specific cutaneous LE has been classified as acute, subacute,
intermittent and chronic. Lesions may be localised or generalised. In LE-
specific cutaneous LE, lesions are often induced by exposure to sunlight.
LE-nonspecific cutaneous LE may relate to systemic LE or other
autoimmune disease.

16. Who gets Cutaneous LE?
• Cutaneous LE most often affects young to middle-aged adult women
(aged 20–50 years) but children, the elderly, and males may be
affected.
• Important predisposing factors for cutaneous LE include:
Female gender
Genes: ≥ 25 risk loci have been identified and there are HLA
associations
Skin of colour

17. Etiology : Lupus Erythematosus
• LE is classified as autoimmune, as it is associated with
pathogenic antibodies directed against components of cell
nuclei in various tissues.
• UVB irradiation causes keratinocyte necrosis, immune system
activation and antibody formation.
• Factors that aggravate LE include:
oSun exposure
oCigarette smoking
oHormones
oViral infection
oCertain drugs

18. Specific features of Cutaneous LE
• There are various types of cutaneous LE, classified as
 acute,
 subacute,
 intermittent and
 chronic.

19. Acute cutaneous lupus erythematosus
• Affects at least 50% of patients with systemic lupus
erythematosus (SLE).
• Many are sick, young, fair-skinned females.
• Specific features of acute cutaneous LE may include:
Malar eruption or ‘butterfly rash’ (erythema and oedema of cheeks,
sparing nasolabial folds) lasting hours to days
Erythematous papular rash on arms, sometimes forming large plaques
and spreading widely
Photosensitivity (a rash on all recently sun-exposed skin)
Cheilitis and mouth ulcers
Blisters (bullous LE) and erosions

20. Acute cutaneous lupus erythematosus
Vasculitis

21. Subacute cutaneous lupus erythematosus
• About 15% of patients with cutaneous LE have subacute cutaneous LE. One-third of
cases are due to previous drug exposure.
• Features of subacute cutaneous LE include:
Precipitation or aggravation by sun exposure
Non-itchy psoriasis-like papulosquamous rash on the upper back, chest and
upper arms
Annular or polycyclic plaques that clear centrally
Absence of scarring on resolution
• Up to 50% of patients with subacute cutaneous LE may also have a mild form of
SLE, resulting in arthralgia (painful joints) or arthritis (joint disease) and low blood
counts. Severe SLE is rare in patients with subacute cutaneous LE.

22. Subacute cutaneous lupus erythematosus

23. Drug-induced subacute cutaneous LE
• Many drugs have been associated with the onset of subacute
cutaneous LE. They include:
Terbinafine
Tumour necrosis factor-alpha (TNF-α) inhibitors (biologics)
Anticonvulsants
Proton-pump inhibitors

24. Drug-induced subacute cutaneous LE

25. Neonatal cutaneous lupus erythematosus
• Neonatal cutaneous LE arises within 2 months of birth to mothers with known or
subclinical subacute cutaneous LE.
• Features of neonatal cutaneous LE may include:
An annular erythematous rash, which slowly resolves over 6 months
Rash is most often periorbital
Photosensitivity
Blood count abnormalities: haemolytic anaemia, leukopenia, thrombocytopenia
Hepatobiliary disease
Persistent congenital heart block
• A paediatrician should assess all babies born to mothers with subacute LE at birth.
Mortality in babies with heart block is up to 20%, despite pacemaker implantation.

26. Neonatal cutaneous lupus erythematosus

27. Intermittent cutaneous lupus erythematosus
• Intermittent cutaneous LE, more often known as lupus tumidus, is a
dermal form of lupus.
• Features of lupus tumidus include:
Affects sun-exposed sites such as cheeks, neck, anterior chest
Erythematous, urticaria-like patches and plaques with a smooth
surface
Round or annular shapes
Clears during the winter months
Non-scarring

28. Intermittent cutaneous lupus erythematosus

29. Chronic cutaneous lupus erythematosus
• Chronic cutaneous LE accounts for 80% of presentations with
cutaneous LE. About 25% of patients with chronic cutaneous LE
also have systemic LE.
• Types:
Discoid LE
Hypertrophic LE
Mucosal LE
Lupus profundus

30. Discoid LE
• Discoid LE is the most common form of chronic cutaneous LE. It is more prevalent
in patients with skin of dark colour, who are at greater risk of postinflammatory
hyperpigmentation and hypertrophic scarring.
• Discoid LE is confined to the skin above the neck in most patients, but can spread
below the neck to affect upper back, V of neck, forearms and backs of hands.
• Scalp, ears, cheeks, nose are the most common sites.
• Most patients have photosensitivity.
• New lesions are destructive, erythematous scaly plaques with follicular
prominence.
• Scalp discoid LE presents as red, scaly and bald plaques.
• Slow healing leads to postinflammatory pigmentation and white scars.
• Hair growth may partially or completely recover with treatment. Cicatricial
(scarring) alopecia can be permanent.

31. Discoid LE

32. Hypertrophic LE
• Hypertrophic LE is a variant of discoid LE in which there are
thickened and warty plaques resembling viral warts or skin
cancers.
• Hypertrophic LE can occur on palms and/or soles.
• This is also called palmoplantar LE, and is a form of acquired
keratoderma.

33. Hypertrophic LE
Erythematous-
hyperkeratotic
lesions on the
periungual areas and
on the proximal
joints of the fingers
Sharply marginated
painful erosions on
the palate
Haematoxylin-eosin
stain of lesional skin
of the extensor
surface of the first
finger showing
marked acanthosis
and hyperkeratosis
and pronounced cell
infiltrate in the
upper dermis

34. Mucosal LE
• Mucosal LE presents with plaques, ulcers and scaling.
• Mucosal lesions may predispose to squamous cell carcinoma.
• Features:
Lips and inside the mouth
Lower eyelid with madarosis (loss of eyelashes)
Rarely, vulva/penis

35. Mucosal LE

36. Lupus profundus
• Lupus profundus affects subcutaneous tissue and may also be
called lupus panniculitis.
• Lupus profundus may develop at any age, including childhood.
• It may involve face, buttocks, limbs or anywhere.
• Firm deep and tender nodules persist for some months.
• Lesions resolve leaving dented, atrophic scars (lipoatrophy).

37. Lupus profundus

38. Nonspecific cutaneous features of LE
LE nonspecific cutaneous features are most often associated with SLE. They include:
 Diffuse hair thinning
 Urticaria
 Raynaud phenomenon: abnormal blanching of fingers and toes in response to cold weather,
followed by numbness and slow rewarming by the fingers which go blue then red.
 Lupus chilblains: painful erythematous nodules on fingers and toes during cooler months.
 Dilated periungual telangiectasia, ragged cuticles and nail dystrophy
 Digital ulcers and pitting scars
 Thrombophlebitis
 Papular and nodular mucinosis on cheeks, upper chest, upper arms or back.
 Vasculitis: small vessel vasculitis, urticarial vasculitis and less often, vasculitis of medium and
large vessels
 Livedo reticularis and antiphospholipid syndrome

39. Chilblain lupus
Raynaud
phenomenon
Thrombophlebitis
Telangiectasia
Urticaria Livedo reticularis

40. Complications of Cutaneous LE
• Chronic cutaneous LE causes facial deformity and scarring. Active
and burned-out disease can lead to social isolation and
depression.
• Systemic LE may involve heart, lung and brain with significant
morbidity and mortality.
• Vasculitis and antiphospholipid syndrome involving internal organs
can be serious.

41. Diagnosis: Cutaneous LE
• SLE is associated with high titre antinuclear antibodies
• About 70% of patients with subacute LE have Anti-Ro/La
(antibodies found in autoimmune diseases) extractable nuclear
antigens (ENA)
• The severity of LE may be reflected in the titre of ANA and/or
ENA.
• ANA and ENA are often negative in a patient with chronic
cutaneous LE.
• Mild anaemia or leukopenia may be present in patients that do not
have SLE

42. Diagnosis (continue….)
• Skin biopsy may be diagnostic, showing a lichenoid tissue reaction
and features specific to the kind of cutaneous LE.
• Direct immunofluorescence tests may show positive antibody
deposition along the basement membrane (lupus band test).
• Diagnostic features on biopsy are more likely to be found in LE-
specific skin lesions than in LE-nonspecific cutaneous LE.

43. Prevention: Cutaneous LE
• Carefully protect all exposed skin from sun exposure with covering
clothing and SPF50+ broad-spectrum sunscreen
• Smoking cessation is essential – it is best to avoid nicotine
replacement as nicotine in any form may exacerbate cutaneous LE
• If subacute LE is drug-induced, stop the responsible medication.

44. Treatment: Cutaneous LE
• The aim of treatment for
cutaneous LE is to prevent
flares, improve appearance and
to prevent scarring.

45. Treatment: Local therapy
• Potent or ultrapotent topical steroids are applied to chronic
discoid LE plaques
• Calcineurin inhibitors, pimecrolimus cream or tacrolimus ointment
can be used instead of topical steroids
• Intralesional corticosteroid can be injected into small lesions
resistant to topical therapy
• Topical retinoids, calcipotriol and imiquimod have also been
reported to be helpful in a few patients.
• Cosmetic camouflage may be used to disguise unsightly plaques.

46. Treatment: Systemic therapy
• Treatment for cutaneous and systemic LE may include:
Antimalarials especially hydroxychloroquine
Immune modulators such as methotrexate, mycophenolate,
dapsone, ciclosporin
Retinoids, acitretin, isotretinoin
Systemic corticosteroids
If sun protection is strict, vitamin D supplementation.

47. Treatment: Systemic therapy (continue…)
• Severe disease may require more aggressive treatment:
Cyclophosphamide
Thalidomide
Photopheresis
Intravenous immunoglobulin
Monoclonal antibodies targeting T and B cells and cytokines:
rituximab

48. Treatment: Procedures
• Phototherapy using UVA1 may be useful to treat skin lesions of
cutaneous LE
• Photodynamic therapy (PDT) has been reported to clear chronic
cutaneous LE
• Vascular laser can reduce telangiectasia
• Surgery may improve appearance of disfiguring scars

49. Outlook: Cutaneous LE
• The prognosis for cutaneous LE is variable.
• The skin involvement in SLE tends to mirror systemic involvement.
• Drug-induced Subacute Cutaneous LE clears within a few weeks of
withdrawal of the responsible drug.
• Untreated chronic cutaneous LE tends to persist, but severity is
lessened by strict sun protection and avoidance of nicotine.

50. THANK YOU