Dermatoses Morphology Etiology Pathogenesis Treatment/Management

LUPUS ERYTHEMATOSUS

A. CHRONIC CUTANEOUS LE – most often have skin-only or skin-predominant disease

1. Discoid LE -Characterized by dull red -F>M; Young adults -Do biopsy and DIF (75%
macules or indurated plaques of cases positive)
-MOST COMMON
-Presence of adherent scales +/- FORM
atrophy, scarring, and pigment
changes -Course of DLE is variable
(95% of cases confined to
-Carpet tack-like spines under skin at the outset will remain
the scale localized)

-In darker skinned individuals: -Purely cutaneous DLE to

areas with hyperpigmentation SLE is uncommon. SLE
and depigmentation with discoid lesions are
common.
-In lighter-skinned patients: grey
or have little pigment alteration -Fever and arthralgia are
common in patients with
SLE and discoid lesions.
a) Localized DLE -Usually localized above the
neck: scalp, bridge of the nose,
malar areas, lower lip, and ears
(concha of the ear and external
canal)

-Some patients with periorbital

edema and erythema

-Scalp: erythematous patches or

plaques to white, often
depressed, hairless patches

-Lips: may be grey or red and

hyperkeratotic; and are usually
surrounded by a narrow, red
inflammatory zone

-Signs of active disease:

Perifollicular erythema and
easily extractable anagen hairs
b) Generalized DLE -Predilection sites: head, neck, -Less common the localized
thorax and upper extremities DLE

-Scalp: quite bald with striking -Laboratory abnormalities:

patterns of hyperpigmentation  Elevated erythrocyte
and depigmentation sedimentation rate
(ESR)
-Diffuse scarring may involve  Elevated anti-
the face and upper extremities nuclear antibodies
(ANA)
 Single stranded (ss)
DNA antibodies
 Leukopenia
 Dermatoses Morphology Etiology Pathogenesis Treatment/Management

CHRONIC CUTANEOUS LE

2. Hypertrophic LE -Non-pruritic papulonodular -Resembles

lesions keratoacanthoma or
hypertropic lichen planus
-Predilection sites: arms, hands,
scalp, and lips -Seen in SLE while some
have only cutaneous
involvement

3. LE – LP Overlap -Lesions are large, atrophic, -Potent topical

Syndrome hypopigmented, red or pink corticosteroids
patches and plaques -Dapsone
-Thalidomide
-Plus fine telangiectasia and -Isotretinoin
scaling -Mycophenolate
-Mofetil
-Predilection sites: Extensor -Azathioprine
aspects of the extremities and
midline back -Response to treatment is
poor
-Prominent palmoplantar
involvement is characteristic
and most troublesome feature

-Nail dystrophy and anonychia

-Scarring alopecia and oral

involvement
 Dermatoses Morphology Etiology Pathogenesis Treatment/Management

CHRONIC CUTANEOUS LE

4. Chilblain LE -Predilection sites: fingertips, -F>M -Diagnostics:

(Hutchinson) rims of ears, calves, and heels  Cryoglobulins
-Chronic and unremitting  Antiphospholipid
-It is usually preceded by DLE form of LE antibody
on the face
-Systemic involvement is
sometimes seen

-Differential diagnosis:
Sarcoidosis

5. Tumid LE -Present with edematous -Rare but distinctive entity -Antimalarials

erythematous plaques

-Predilection site: Trunk

-Resembles reticular
erythematous mucinosis
 Dermatoses Morphology Etiology Pathogenesis Treatment/Management

CHRONIC CUTANEOUS LE

6. Lupus Panniculitis (LE -Firm, sharply defined an non- -Kaposi Irgang Disease
Profundus) tender subcutaneous nodules
-F>M; 20-45 years old
-Predilection site: Proximal
extremities -May heal with deep
depressions from loss of the
-May have DLE at other sites panniculus

-Differential diagnosis:
Subcutaneous panniculitis-
type lymphoma
 Dermatoses Morphology Etiology Pathogenesis Treatment/Management

B. SUBACUTE -Presents as transient red to -Clinically distinct subset of cases -Sun protection
CUTANEOUS LE pink with faint violet of LE -Antimalarial
polycyclic annular lesions or
psoriasiform plaques -F>M; 15-40 years old; whites

-With thin easily detach scale -Approximately 10-15% of the

and telangiectasia or LE population
dyspigmentation
-20% has concomitant DLE
-Follicles are NOT involve:
NO SCARRING -Associated with arthralgia or
arthritis, autoimmune thyroid
-Common in sun-exposed disease
areas: face and neck, the V
portion of the chest and back; -80% positive ANA test, 20% has
arms leukopenia

-Majority has antibodies to

-Photosensitivity is prominent Ro/SSA antigen, and most are
positive for Human Leukocyte
Antigen (HLA)-DR3

-Drug induced SCLE is related

to:
 Hydrochlorothiazide
 ACE-inhibitors
 Calcium channel blockers
 Interferons
 Anticonvulsants
 Griseofulvin
 Glyburide
 Piroxicam
 Penicillamine
 Spironolactone
 Terbinafine
 Statins
 Dermatoses Morphology
SUBACUTE CUTANEOUS LE
1. Neonatal LE -Annular erythematous
macules and plaques
-Predilection site: head and
extremities
-Associated with periocular
involvement (Raccoon eyes)
-Resolve spontaneously by 6
months of age and heal
without significant scarring
+/- atrophy
-Dyspigmentation and
persistent telangiectasias may
remain from months to years
2. Complement Deficiency -Presents as photosensitive
Syndrome annular SCLE lesions
-Patients with C4 deficiency
often have hyperkeratosis of
the palms and soles
Etiology Pathogenesis Treatment/Management
-F>M; born to mothers who carry
the Ro/SSA antibody
-NO skin lesions at birth but
develop them during the first few
weeks of life
-Associated with:
 Congenital Heart Block
 Thromboocytopenia
 Hepatic disease
-Strong association with Ro/SSA
autoantibody
-Deficiencies of the early
components, especially C2 and
C4
-+ Ro/SSA antibody formation
-Heterozygous deficiency of
either complement component
C4A or C4B has a frequency of
approximately 20% in white
populations
-Homozygous deficiency of both
is rare
 Dermatoses Morphology Etiology Pathogenesis Treatment/Management

C. ACUTE CUTANEOUS -Commonly affects the malar

LE areas of the face (Butterfly
distribution)

-Skin lesions are transient and

heal with less pigmentary
change

-Well-demarcated patches of
erythema with fine overlying
scale on the dorsal aspect of the
hands, fingers and periungual
areas

-Characteristic sparing of the

knuckles (which are
preferentially involve in DM)
 Dermatoses Morphology Etiology Pathogenesis
D. SYSTEMIC LE Criteria for classification of SLE: (should fulfil 4 out of
11)
 Malar rash: fixed erythema, flat or raised, over the
malar eminences, tending to spare the nasolabial
folds
 Discoid rash: erythematous raised patches with
adherent keratotic scaling and follicular plugging;
atrophic scarring
 Photosensitivity: skin rash as a result of unusual
reaction to sunlight, by patient history or physical
examination
 Oral ulcers: oral or nasopharyngeal ulceration,
painless
 Arthritis: non-erosive arthritis involving two or
more peripheral joints, characterized by tenderness,
swelling or effusion
Immunologic findings in SLE
 ANA test: positive in 95% of cases of SLE
 Lupus erythematosus cell test: specific but not
very sensitive
 Double-stranded DNA/ Anti-dsDNA: specific but
not very sensitive; indicates high risk of renal
disease
 Anti-ssDNA antibody: sensitive but not specific;
An IgM isotope seen in DLE may identify a subset
of patients at risk for developing systemic symptoms
 Anti-Sm antibody: very high specificity but only
10% sensitive
 Antinuclear ribonucleic acid protein (anti-
nRNP): very high titers are present in mixed
connective tissue disease, lower titers may be seen
in SLE
 Anti-La antibodies: common in SCLE and Sjogren
syndrome, and occasionally found in SLE
 Anti-Ro antibodies: 25% of SLE and 40% of
Sjogren cases. They are more common in patients
Treatment/Management
Local treatment
-The single most effective local
treatment is the injection of
corticosteroids into the lesions
-Application of potent or
superpotent topical
corticosteroids
-Topical calcineurin inhibitors
(topical macrolactams) may also
be useful as second-line topical
therapy
-Photodynamic therapy has been
reported as effective
Systemic treatment
-The safest class of systemic
agent for LE is the
Antimalarials
-Retinoids: second line agents
and are particularly helpful on
treating Hypertrophic LE
-Systemic immunosuppressive
agents: often required to
manage the systemic
manifestations of LE and are
third-line systemic agents for
cutaneous LE
-Thalidomide: can be effective
but is used in limited by the risk
of teratogenicity and neuropathy
-Dapsone: drug of choice for
 with SCLE (70%), Neonatal LE (95%), C2 and C4 bullous systemic LE, and may
deficient LE (50-75%), Late onset LE (75%), and be effective in some cases of
Asian patients with LE (50-60%) SCLE and DLE
 Serum complement: low levels indicate active
disease and often with renal involvement. -Oral prednisone: generally
 Antiphospholipid antibodies: may occur in reserve for acute flares of
association with lupus and other connective tissue disaese
disorder
1. Childhood SLE -Typical butterfly -Onset of childhood occurs between the ages of 3 and 15
eruption on the face and
photosensitivity -F>M; 4:1

-Involvement of the -May be associated with joint, renal, neurologic and

oral mucosa gastrointestinal disease, weight loss, fatigue,
hepatosplenomegaly, lymphadenopathy, and fever

2. SLE and Pregnancy -A flare of SLE may -Women with LE may have successful pregnancies
occur during the
postpartum period -Difficult in conceiving; greater frequency of miscarriage

-Course of pregnancy may be entirely normal (Remission of

the LE or the symptoms of LE may become worse)

-Risk of fetal death is increased in women with a previous

history of fetal loss and anticardiolipin or antii-Ro antibodies