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Chronic cutaneous lupus erythematosus introduction
Chronic cutaneous lupus erythematosus, also known as discoid
lupus erythematosus (DLE), is the most familiar of all the primary
cicatricial alopecia conditions. Chronic cutaneous lupus erythematosus
is a term used by many authors and dermo-pathologists, to define
a form of photosensitive dermatosis characterized by pronounced
erythema, scale and pigment changes in the skin. Its is hoped
that the continuous and untiring research undertaken so far by
researchers in the field of hair follicle cycling and genetic
anomalies of hair production will pave the way for the development
of a targeted therapy for cutaneous lupus erythematosus, but currently
no fully effective treatment is available.
Chronic cutaneous lupus erythematosus occurs more predominantly
in the female sex compared to the male sex, and is more commonly
seen in adults rather than in children. There is no clear racial
tendency in the condition, although reports in the USA indicate
that it may be somewhat more prevalent among African Americans
as compared to Caucasians.
As is the situation in most forms of cicatricial alopecia, the
exact cause of chronic cutaneous lupus erythematosus remains an
enigma. A complex interplay of genetic, environmental, and a host
of other, factors play a role in the manifestation of the disease.
In some susceptible individuals, exposure to ultra violet light
is suspected to trigger the condition. Another consideration in
the etiology of this form of alopecia is koebnerization, which
is development of the disease in areas of skin damaged due to
wounds. Mundane trauma such as scratching or strong hair care
practices that can damage the hair and scalp skin may aggravate
the scalp disease in affected individuals.
Onset of the disease characteristically occurs between 20 and
40 years of age. Approximately 5-10% of the adults with cutaneous
lupus erythematosus can eventually develop systemic lupus erythematosus
(SLE) in due course. SLE is an autoimmune disease in which the
individual’s own immune system is directed against the body's
own tissues and multiple organs are affected. The course of the
systemic disease is often severe, and renal or neurological involvement
cannot be ruled out.
Chronic
cutaneous lupus erythematosus clinical features
The diagnosis “cutaneous lupus erythematosus” actually
encompasses at least 10 to 15 slightly different clinical presentations.
Some of the common types include localized and generalized discoid
lupus erythematosus (DLE), lupus panniculitis and lupus tumidus.
The names of the different variants reflect the predominant component
of the lesion. As maintained by Gilliam and Sontheimer, DLE is
one particular type of chronic cutaneous lupus erythematosus.
The term discoid simply means coin-shaped, and the scarred, coin-shaped
lupus lesions commonly appearing on the skin that is exposed to
light has been termed as discoid lupus erythematosus. Subacute
cutaneous lupus erythematosus and systemic lupus erythematosus
each have several different cutaneous presentations.
The clinical features vary from case to case and in general range
from typical erythematosus, hardened and hyperkeratotic or thickened
plaques to patches that appear non-inflammatory. These apparently
non-inflammatory patches can be deceptive as they belie the presence
of deep dermal inflammation beneath. The inflammation is too deep
in the skin to be seen at the skin surface, but a skin biopsy
reveals the intense inflammation.
Early lesions of this type of alopecia are erythematous, slightly
elevated papules or plaques. In older lesions, follicular plugging
characterized by small round areas of hyperkeratosis is noted.
Lesions can spread centrifugally and may merge together. These
lesions usually heal leaving behind scars and pigmentary changes.
The lesions of chronic cutaneous lupus erythematosus are characteristically
well circumscribed, slightly scaly and atrophic, and can be tender
or pruritic. Follicullar plugging with keratinous debris is distinct,
and hair follicular ostia or openings can be distended with laminated
keratin. Scaling is limited to perifollicular areas. Sebaceous
glands are atrophied or absent. If the scalp lesions are erythematous
and scaly, the hair usually grows back, but if the lesion heals
with scarring the alopecia in that area is permanent.
The pull test may be positive for anagen hairs. In this test,
the dermatologist takes a few strands between their thumb and
forefinger and pulls on them gently. Anagen or growing hairs remain
rooted in place while hairs in the telogen or so-called resting
state come out easily. By ascertaining the ratio of determining
how many hairs were pulled and the number that came out, dermatologists
roughly work out the percentage of hair follicles in a telogen
state.
Patients suffering from this condition often complain of increased
shedding of hair, pruritis and a stinging or burning sensation.
Symptoms of scalp tenderness are commonly experienced. Patients
with systemic lupus erythematosus may have discoid scalp lesions
and short frontal lupus hairs. Follicular orifices are usually
damaged.
Chronic
cutaneous lupus erythematosus differential diagnosis
When lesions are present on both the scalp and elsewhere on the
skin, as well as when extra-cranial DLE is present, the diagnosis
is simplified. However, many patients with scalp lesions have
no other manifestations of lupus erythematosus elsewhere on their
body.
In these cases, early scalp changes can be confused with psoriasis,
tinea capitis (ringworm), dermato-myositis, and other forms of
scarring alopecia including; lichen planopilaris, alopecia mucinosa
and subacute folliculitis decalvans. In active disease, the center
of the lesion is affected rather than the hair bearing periphery.
This differentiates DLE from lichen planopilaris and folliculitis
decalvans. By examining skin biopsies, peri-vascular inflammation
or inflammation around blood vessels and peri-eccrine chronic
inflammation are also characteristics that aid in distinguishing
chronic cutaneous lupus erythematosus from lichen planopilaris.
Non-inflammatory alopecic patches can be confused with alopecia
areata or pseudopelade, or other end stage primary cicatricial
alopecia conditions. However, in such cases, follicular plugging,
atrophy or breakdown of skin and mottled pigmentation would be
the relevant pointers towards DLE and away from these other diagnoses.
Chronic
cutaneous lupus erythematosus pathology
As the clinical findings can mimic other forms of alopecia, scalp
biopsy as a tool for diagnosis becomes crucial in evaluation of
the alopecia concerned. In chronic cutaneous lupus erythematosus,
there is vacuolar degeneration of the basal layer of the follicular
epithelium. The inflammation at the interface tends to be mild.
Plasma cells in the infiltrate and presence of dermal mucin (a
substance that shows up as regions without any obvious structure
in skin biopsies) are indicative of chronic cutaneous lupus erythematosus.
Regrettably, more often than not, biopsy specimens from chronic
cutaneous lupus erythematosus only demonstrate vacuolar interface
change of the follicular epithelium, confusing the condition with
lichen planopilaris. At this juncture, if direct immuno-fluorescence
demonstrates deposition of immunoreactants at the dermo-epidermal
junction, it is indicative of chronic cutaneous lupus erythematosus.
The downside is that most often immuno-fluorescence fails to play
a confirmatory role in the diagnosis of chronic cutaneous lupus
erythematosus.
When differentiation between lichen planopilaris and chronic
cutaneous lupus erythematosus proves difficult, histological findings
on the nature of the inflammatory infiltrate are a help in making
a diagnosis. In contrast to pseudopelade and lichen planopilaris
where perifollicular lymphocytic infiltrates usually involve the
upper or one-third of the follicle, the lymphocytic infiltrates
of lupus erythematosus are typically found throughout the full
thickness of the reticular dermis. In other words, the inflammation
is less focused on hair follicles as compared to other forms of
scarring alopecia.
Chronic
cutaneous lupus erythematosus treatment
Detailed history, thorough physical examination and interpretation
of appropriate tests are necessary before reaching diagnostic
deductions and commencement of therapy. Patients with chronic
cutaneous lupus erythematosus must be treated aggressively to
prevent permanent scarring and scarring alopecia, and it may be
noted that the acute or early lesions are the most responsive
to treatment. Exposure to sunlight (or ultraviolet light) should
be minimized in such people.
In patients with early stage manifestations and / or limited
active disease, first line therapy is by way of class I or class
II corticosteroids applied topically. Intralesional topical steroids
alone may be also be effective. Patients are given a high, medium
and low potency steroid, starting with the most potent. With improvement
in the condition, treatment tapers to the medium and low potency
preparations in sequence. Potent topical steroids should be used
with caution, as they may suppress natural corticosteroid production
in the adrenal glands.
If the above treatment options fail to produce a favorable response
within 8 weeks, anti malarial drugs are started. Antimalarials
remain the cornerstone of treatment because of their effectiveness
and safety in cases of rapidly progressive or extensively active
chronic cutaneous lupus erythematosus. Cigarette smoking has been
proven to hamper the therapeutic response to these drugs, so patients
are advised to decrease, and if possible, discontinue smoking
to abet improvement. Clinical improvement is normally perceived
within 4-8 weeks after starting anti-malarial drug use, but the
full extent of benefit is not apparent until several months after
commencement of treatment. Oral prednisone administered in tapering
dosages can help as bridge therapy in very severe cases. When
using Antimalarials, the potential side effects must be taken
into serious consideration and patient monitoring must be carried
out on a periodic basis.
Alternative therapies encompass the use of retinoids (vitamin
A derived drugs), dapsone and experimentally thalidomide has been
used in some patients. In those patients with resistance to anti
malarial drugs, oral retinoids have provided positive results
ranging from good to excellent. Oral retinoids can be used for
stabilization of acute disease, as a combination approach until
control of disease is established, or to maintain disease remission.
Once again, any of these lines of treatment may produce harmful
side effects and patients should be closely monitored. In many
instances, it has been found that patients respond to combination
therapy. Once there is visible progress, it is advisable to taper
the drugs with undesirable side effects.
End stage cutaneous lupus erythematosus can be best treated by
surgery. The surgical procedures involve excision, scalp reduction,
hair transplantation, and grafting and sometimes laser hair removal.
Surgical intervention is advised judiciously as dormant lesions
may flare up after surgery, and may require subsequent aggressive
systemic therapy.
When supported by clinical and histological findings, chronic
cutaneous lupus erythematosus is found to be a distinct condition
with features that has overlapping clinical features with other
forms of scarring hair loss. Because the cutaneous lesions of
discoid lupus erythematosus and systemic lupus erythematosus may
be identical, a patient presenting with typical discoid lesions
must be evaluated to determine whether systemic involvement is
present. A medical history and physical examination are required
to rule out a possible early cutaneous manifestation of systemic
lupus erythematosus.
Chronic
cutaneous lupus erythematosus
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