Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an
autoimmune
In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". ...
skin condition on the
lupus erythematosus
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blo ...
spectrum of illnesses.
It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp.
[James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.) Saunders. Chapter 8. .] The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin.
These lesions can last for years without treatment.
Patients with
systemic lupus erythematous develop discoid lupus lesions with some frequency.
However, patients who present initially with discoid lupus infrequently develop systemic lupus.
Discoid lupus can be divided into localized, generalized, and childhood discoid lupus.
The lesions are diagnosed by biopsy.
Patients are first treated with
sunscreen and
topical steroids.
If this does not work, an oral medication—most likely
hydroxychloroquine
Hydroxychloroquine, sold under the brand name Plaquenil among others, is a medication used to prevent and treat malaria in areas where malaria remains sensitive to chloroquine. Other uses include treatment of rheumatoid arthritis, lupus, an ...
or a related medication—can be tried.
Signs and symptoms
Morphology of lesions
Discoid lupus erythematosus (DLE) skin lesions first present as dull or purplish red, disc-shaped flat or raised and firm areas of skin.
These lesions then develop increasing amounts of white, adherent scale.
Finally, the lesions develop extensive
scar
A scar (or scar tissue) is an area of fibrous tissue that replaces normal skin after an injury. Scars result from the biological process of wound repair in the skin, as well as in other organs, and tissues of the body. Thus, scarring is a n ...
ring and/or atrophy, as well as pigment changes.
They may also have overlying dried fluid, known as
crust.
On darker skin, the lesions often lose skin
pigmentation
A pigment is a colored material that is completely or nearly insoluble in water. In contrast, dyes are typically soluble, at least at some stage in their use. Generally dyes are often organic compounds whereas pigments are often inorganic compoun ...
in the center and develop increased, dark skin pigmentation around the rim.
On lighter skin, the lesions often develop a gray color or have very little color change.
More rarely, the lesions may be bright red and look like
hives.
Location of lesions
The
skin lesion
A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of th ...
s are most often in sun-exposed areas localized above the
neck, with favored sites being the
scalp
The scalp is the anatomical area bordered by the human face at the front, and by the neck at the sides and back.
Structure
The scalp is usually described as having five layers, which can conveniently be remembered as a mnemonic:
* S: The ski ...
, bridge of the
nose
A nose is a protuberance in vertebrates that houses the nostrils, or nares, which receive and expel air for respiration alongside the mouth. Behind the nose are the olfactory mucosa and the sinuses. Behind the nasal cavity, air next passes ...
,
upper cheeks, lower
lip
The lips are the visible body part at the mouth of many animals, including humans. Lips are soft, movable, and serve as the opening for food intake and in the articulation of sound and speech. Human lips are a tactile sensory organ, and can be ...
, and
ear
An ear is the organ that enables hearing and, in mammals, body balance using the vestibular system. In mammals, the ear is usually described as having three parts—the outer ear, the middle ear and the inner ear. The outer ear consists of ...
and hands
24% of patients also have lesions in the
mouth (most often the palate),
nose
A nose is a protuberance in vertebrates that houses the nostrils, or nares, which receive and expel air for respiration alongside the mouth. Behind the nose are the olfactory mucosa and the sinuses. Behind the nasal cavity, air next passes ...
,
eye, or
vulva
The vulva (plural: vulvas or vulvae; derived from Latin for wrapper or covering) consists of the external female sex organs. The vulva includes the mons pubis (or mons veneris), labia majora, labia minora, clitoris, vestibular bulbs, vulv ...
, which are all
mucosal parts of the body.
More rarely, patients may have lesions on the head and neck as well as the arms and trunk.
Special characteristics of some lesions
Scalp lesions
When discoid lupus is on the scalp, it starts as a red flat or raised area of skin that then loses hair and develops extensive scarring.
The lesions often lose skin
pigment
A pigment is a colored material that is completely or nearly insoluble in water. In contrast, dyes are typically soluble, at least at some stage in their use. Generally dyes are often organic compounds whereas pigments are often inorganic compou ...
and become white with areas of increased skin pigment, with or without areas of redness, and have a sunken appearance.
They can have a smooth surface or have visible, dilated
hair follicles on the surface.
Lip lesions
When discoid lupus is on the lip, it often has a grey or red colour with a thickened top layer of skin (known as
hyperkeratosis
Hyperkeratosis is thickening of the stratum corneum (the outermost layer of the epidermis, or skin), often associated with the presence of an abnormal quantity of keratin,Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) ''Robbins & Cotran Pathologi ...
), areas where the top layer has worn away (known as
erosion
Erosion is the action of surface processes (such as water flow or wind) that removes soil, rock, or dissolved material from one location on the Earth's crust, and then transports it to another location where it is deposited. Erosion is dis ...
), and a surrounding rim of redness.
Other symptoms
Patients may state that their lesions are itchy, tender, or asymptomatic.
In addition to their skin lesions, they may also have swelling and redness around their eyes, as well as
blepharitis.
Complications
Darker-skinned patients are often left with severe
scar
A scar (or scar tissue) is an area of fibrous tissue that replaces normal skin after an injury. Scars result from the biological process of wound repair in the skin, as well as in other organs, and tissues of the body. Thus, scarring is a n ...
ring and skin color changes even after the lesions get better.
In addition, these patients have an increased, though still small, risk for aggressive skin
squamous cell carcinoma.
Causes
Sun exposure triggers lesions in people with discoid lupus erythematous (DLE).
Evidence does not clearly demonstrate a genetic component to DLE; however, genetics may predispose certain people to disease.
Mechanism
Most experts consider DLE an
autoimmune disease since
pathologists
A list of people notable in the field of pathology.
A
* John Abercrombie, Scottish physician, neuropathologist and philosopher.
* Maude Abbott (1869–1940), Canadian pathologist, one of the earliest women graduated in medicine, expert in co ...
see
antibodies when they biopsy the lesions and look at the
tissue under the microscope.
However, scientists do not understand the connection between these antibodies and the lesions seen in discoid lupus.
Possibly,
UV light damages skin cells, which then release material from their
nuclei.
This material diffuses to the
dermoepidermal junction
The dermoepidermal junction or dermal-epidermal junction (DEJ) is the area of tissue that joins the epidermal and the dermal layers of the skin. The basal cells in the stratum basale of the epidermis connect to the basement membrane by the anchor ...
, where it binds to circulating antibodies, thereby leading to a series of inflammatory reactions by the
immune system
The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splint ...
.
Alternatively, dysfunctional
T cell
A T cell is a type of lymphocyte. T cells are one of the important white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell r ...
s may lead to the disease.
Diagnosis
When a patient initially presents with discoid lupus, the doctor should ensure that the patient does not have
systemic lupus erythematosus.
The doctor will order tests to check for
anti-nuclear antibodies
Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are autoantibodies that bind to contents of the cell nucleus. In normal individuals, the immune system produces antibodies to foreign proteins (antigens) but not to human prote ...
in the patient's serum,
low white blood cell levels, and
protein
Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, res ...
and/or
blood in the urine
Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. “Gross hematuria” occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable w ...
.
In order to help with diagnosis, the doctor may peel off the top layer of
scale from a patient's lesions in order to look at its underside.
If the patients do indeed have discoid lupus, the doctor may see tiny spines of keratin that look like carpet tacks and are called ''langue au chat.''
Diagnosis is confirmed through biopsy.
Typical biopsy findings include deposits of
IgG and
IgM
Immunoglobulin M (IgM) is one of several isotypes of antibody (also known as immunoglobulin) that are produced by vertebrates. IgM is the largest antibody, and it is the first antibody to appear in the response to initial exposure to an antig ...
antibodies at the dermoepidermal junction on
direct immunofluorescence
A direct fluorescent antibody (DFA or dFA), also known as "direct immunofluorescence", is an antibody that has been tagged in a direct fluorescent antibody test. Its name derives from the fact that it directly tests the presence of an antigen with ...
.
This finding is 90%
sensitive; however,
false positives can occur with biopsies of facial lesions.
In addition,
pathologists
A list of people notable in the field of pathology.
A
* John Abercrombie, Scottish physician, neuropathologist and philosopher.
* Maude Abbott (1869–1940), Canadian pathologist, one of the earliest women graduated in medicine, expert in co ...
often see groups of
white blood cells, particularly
T helper cell
The T helper cells (Th cells), also known as CD4+ cells or CD4-positive cells, are a type of T cell that play an important role in the adaptive immune system. They aid the activity of other immune cells by releasing cytokines. They are consider ...
s, around the
follicles and
blood vessel
The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...
s in the
dermis.
The
epidermis appears thin and has effaced
rete ridges as well as excess amounts of
keratin
Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. Alpha-keratin (α-keratin) is a type of keratin found in vertebrates. It is the key structural material making up scales, hair, nails, feathers, ho ...
clogging the openings of the follicles.
The
basal layer of the epidermis sometimes appears to have holes in it since some of the cells in this layer have broken apart.
The remains of skin cells that have died through a process called
apoptosis are visible in the upper layer of the dermis and the basal layer of the epidermis.
The differential diagnosis includes
actinic keratoses
Actinic keratosis (AK), sometimes called solar keratosis or senile keratosis, is a pre-cancerous area of thick, scaly, or crusty skin.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . Actinic k ...
,
sebborheic dermatitis,
lupus vulgaris
Lupus vulgaris (also known as tuberculosis luposa) are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck. It is the most common ''Mycobacterium tuberc ...
,
sarcoidosis
Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly a ...
,
drug rash,
Bowen's disease
Squamous-cell skin cancer, also known as cutaneous squamous-cell carcinoma (cSCC), is one of the main types of skin cancer along with basal cell cancer and melanoma. It usually presents as a hard lump with a scaly top but can also form an ulcer. O ...
,
lichen planus
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, fla ...
,
tertiary syphilis,
polymorphous light eruption,
lymphocytic infiltration,
psoriasis
Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by raised areas of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small, localized patches to complete ...
, and systemic lupus erythematosus.
Classification
Discoid lupus can be broadly classified into localized discoid lupus and generalized discoid lupus based on the location of the lesions.
Patients who develop discoid lupus in childhood also have their own sub-type of disease.
Hypertrophic lupus and lupus profundus are two special types of discoid lupus distinguished by their characteristic morphological findings.
Finally, many patients with systemic lupus also develop discoid lupus lesions.
Localized
Most people with discoid lupus only have lesions above the neck and therefore have localized discoid lupus erythematosus.
Generalized
Rarely, patients may have lesions above and below the neck; these patients have generalized discoid lupus erythematosus.
In addition to lesions in the typical above-the-neck locations, patients with generalized discoid lupus often have lesions on the
thorax
The thorax or chest is a part of the anatomy of humans, mammals, and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main divisions of the cre ...
and the
arm
In human anatomy, the arm refers to the upper limb in common usage, although academically the term specifically means the upper arm between the glenohumeral joint (shoulder joint) and the elbow joint. The distal part of the upper limb between th ...
s.
These patients are often
bald
Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarrin ...
, with abnormal
skin pigment on their scalp, and have severe
scar
A scar (or scar tissue) is an area of fibrous tissue that replaces normal skin after an injury. Scars result from the biological process of wound repair in the skin, as well as in other organs, and tissues of the body. Thus, scarring is a n ...
ring of the
face
The face is the front of an animal's head that features the eyes, nose and mouth, and through which animals express many of their emotions. The face is crucial for human identity, and damage such as scarring or developmental deformities may aff ...
and arms.
Patients with generalized discoid lupus often have abnormal lab tests, such as an elevated
ESR or a
low white blood cell count.
They also often have
auto-antibodies
An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies.
Pr ...
, such as
ANA or
anti-ssDNA antibody.
Childhood
When patients develop discoid lupus in childhood, it differs from typical discoid lupus in several ways. Boys and girls are equally affected, and these patients later develop
SLE more often.
These patients also typically do not have any abnormal sensitivity to the sun.
Special types of discoid lupus lesions
= Hypertrophic lupus
=
Some experts consider
hypertrophic lupus erythematosus—which consists of lesions covered by a very thick,
keratin
Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. Alpha-keratin (α-keratin) is a type of keratin found in vertebrates. It is the key structural material making up scales, hair, nails, feathers, ho ...
-filled scale—an unusual subset of discoid lupus.
Others consider it a distinct entity.
= Lupus profundus
=
If a patient has discoid lupus lesions on top of
lupus panniculitis, they have lupus profundus.
These patients have firm, nontender nodules with defined borders underneath their discoid lupus lesions.
Systemic lupus erythematosus with discoid lupus lesions
In general, patients with discoid lupus who have only skin disease and no systemic symptoms have a genetically distinct disease from patients with
SLE.
However, 25% of patients with SLE get discoid lupus lesions at some point as part of their disease.
Treatment
Treatment for discoid lupus erythematosus includes smoking cessation and a sunscreen that protects against both
UVA and
UVB
Ultraviolet (UV) is a form of electromagnetic radiation with wavelength from 10 nm (with a corresponding frequency around 30 PHz) to 400 nm (750 THz), shorter than that of visible light, but longer than X-rays. UV radiation i ...
light as well as very strong topical steroids or steroids injected into the lesions.
Other topical treatments,
tacrolimus
Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...
or
pimecrolimus
Pimecrolimus is an immunomodulating agent of the calcineurin inhibitor class used in the treatment of atopic dermatitis (eczema). It is available as a topical cream, once marketed by Novartis (however, Galderma has been promoting the compound i ...
can also be used.
If this does not help the patient, his or her physician can prescribe an antimalarial medication such as oral hydroxychloroquine or
chloroquine
Chloroquine is a medication primarily used to prevent and treat malaria in areas where malaria remains sensitive to its effects. Certain types of malaria, resistant strains, and complicated cases typically require different or additional medi ...
.
Other oral medications used to treat discoid lupus include
retinoids (
isotretinoin
Isotretinoin, also known as 13-''cis''-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers ( squamous-cell carcinoma), and in t ...
or
acitretin
Acitretin (trade names Soriatane and Neotigason) is a second-generation retinoid. It is taken orally, and is typically used for psoriasis.
Acitretin is an oral retinoid used in the treatment of severe resistant psoriasis. Because of the potential ...
),
dapsone,
thalidomide
Thalidomide, sold under the brand names Contergan and Thalomid among others, is a medication used to treat a number of cancers (including multiple myeloma), graft-versus-host disease, and a number of skin conditions including complications o ...
(teratogenic, side effects include peripheral neuropathy),
azathioprine
Azathioprine (AZA), sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus erythematosus, ...
,
methotrexate, or
gold
Gold is a chemical element with the symbol Au (from la, aurum) and atomic number 79. This makes it one of the higher atomic number elements that occur naturally. It is a bright, slightly orange-yellow, dense, soft, malleable, and ductile me ...
.
The topical steroid
fluocinonide
Fluocinonide (sold under various brand names) is a potent glucocorticoid used topically as an anti-inflammatory agent for the treatment of skin disorders such as eczema and seborrhoeic dermatitis. It relieves itching, redness, dryness, crusti ...
is more effective than
hydrocortisone
Hydrocortisone is the name for the hormone cortisol when supplied as a medication. Uses include conditions such as adrenocortical insufficiency, adrenogenital syndrome, high blood calcium, thyroiditis, rheumatoid arthritis, dermatitis, asthm ...
in the treatment of discoid lupus erythematosus.
For oral treatment, hydroxychloroquine and acitretin are equally effective; however, acitretin was associated with more adverse effects.
Pulsed dye laser is also an effective treatment for patients with localized discoid lupus. For patients with scalp disease, hair transplantation can help with their hair loss.
Prognosis
Discoid lupus erythematosus is a
chronic condition, and lesions will last for several years without treatment.
50% of patients will eventually get better on their own.
If a patient does not have any signs of
systemic lupus erythematosus, such as generalized hair loss,
ulcers
An ulcer is a discontinuity or break in a bodily membrane that impedes normal function of the affected organ. According to Robbins's pathology, "ulcer is the breach of the continuity of skin, epithelium or mucous membrane caused by sloughing o ...
in the mouth or nose,
Raynaud's phenomenon
Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, is a medical condition in which the spasm of small arteries cau ...
,
arthritis, or
fever
Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...
at the time that they develop discoid lupus, they will most likely only have discoid lupus and will never develop systemic lupus erythematosus.
Epidemiology
Discoid lupus has an unknown
incidence, although it is two to three times more common than systemic lupus erythematosus.
The disease tends to affect young adults, and women are affected more than men in a 2:1 ratio.
Society and culture
The musician
Seal
Seal may refer to any of the following:
Common uses
* Pinniped, a diverse group of semi-aquatic marine mammals, many of which are commonly called seals, particularly:
** Earless seal, or "true seal"
** Fur seal
* Seal (emblem), a device to imp ...
has this skin condition.
Singer
Michael Jackson
Michael Joseph Jackson (August 29, 1958 – June 25, 2009) was an American singer, songwriter, dancer, and philanthropist. Dubbed the "King of Pop", he is regarded as one of the most significant cultural figures of the 20th century. Over a ...
was reportedly diagnosed with discoid lupus in 1984; the condition might have damaged his nasal cartilage and led to some of his cosmetic surgery.
In animals
Dogs and horses can also get discoid lupus.
See also
*
List of cutaneous conditions associated with increased risk of nonmelanoma skin cancer
*
List of people with lupus
This is a categorised, alphabetical list of notable people who have been diagnosed with lupus.
Lupus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Symptoms of these ...
References
External links
{{Localized connective tissue diseases
Autoimmune diseases
Cutaneous lupus erythematosus