Subacute Cutaneous Lupus Erythematosus
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Subacute cutaneous lupus erythematosus (SCLE ) is a clinically distinct subset of cases of
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, blood cells, heart, ...
that is most often present in white women aged 15 to 40, consisting of
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 this ...
s that are scaly and evolve as ''poly-cyclic annular'' lesions or plaques similar to those of plaque
psoriasis Psoriasis is a long-lasting, noncontagious autoimmune disease characterized by patches of abnormal skin. These areas are red, pink, or purple, dry, itchy, and scaly. Psoriasis varies in severity from small localized patches to complete b ...
.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 160. . Characteristically the lesions appear in sun-exposed areas such as the vee of the
neckline The neckline is the top edge or edges of a clothing, garment that surrounds the neck, especially from the front view. Neckline also refers to the overall visual line between all the layers of clothing and the neck and Shoulder, shoulders of a per ...
or the
forearm The forearm is the region of the upper limb between the elbow and the wrist. The term forearm is used in anatomy to distinguish it from the arm, a word which is used to describe the entire appendage of the upper limb, but which in anatomy, techn ...
s, but not the
face The face is the front of the 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 affect th ...
. It may be brought on by sun-sensitizing medications, but is usually associated with
autoimmune disorders An autoimmune disease is a condition that results from an anomalous response of the adaptive immune system, wherein it mistakenly targets and attacks healthy, functioning parts of the body as if they were foreign organisms. It is estimated tha ...
such as
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects synovial joint, joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and h ...
and Sjögren's syndrome. Therapy generally involves sun avoidance and protection and topical
corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...
s. Sometimes systemic drug treatment is necessary. Besides corticosteroids other
immunosuppressant Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent the activity of the immune system. Classification Immunosuppressive drugs can be classified ...
s such as
methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...
are also used. Lesions of SCLE may have an ''annular'' (shaped like a ring) configuration, with raised red borders and central clearing.


Signs and symptoms

The lesions of systemic
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, blood cells, heart, ...
are characterized by their distribution, which can be either annular with central clearing or papulosquamous. The lesions normally heal without
atrophy Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), malnutrition, poor nourishment, poor circulatory system, circulation, loss of hormone, ...
or scarring, and these two forms can happen at the same time. While
telangiectasia Telangiectasias (), also known as spider veins, are small dilated blood vessels that can occur near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. These dilated blood vessels can develop anywhere ...
or
hypopigmentation Hypopigmentation is characterized specifically as an area of Human skin, skin becoming lighter than the baseline skin color, but not completely devoid of skin pigment, pigment. This is not to be confused with depigmentation, which is characterize ...
may occur, most patients' skin returns to normal. Serologic abnormalities, musculoskeletal complaints, and mild illness are common in patients with SCLE. SCLE typically avoids the face and is more common in sun-exposed areas such as the neck, shoulders, chest, and extensor surfaces of the arms.


Causes

The etiology of systemic
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, blood cells, heart, ...
is not well understood. Sunlight exposure is the traditional precipitating factor in patients with immune dysregulation and an aberrant milieu of genetic predisposition. There have also been reports of drug-induced SCLE.
Angiotensin-converting enzyme inhibitors Angiotensin-converting-enzyme inhibitors (ACE inhibitors) are a class of medication used primarily for the treatment of high blood pressure and heart failure. This class of medicine works by causing relaxation of blood vessels as well as a decr ...
,
anticonvulsants Anticonvulsants (also known as antiepileptic drugs, antiseizure drugs, or anti-seizure medications (ASM)) are a diverse group of pharmacological agents used in the treatment of epileptic seizures. Anticonvulsants are also used in the treatment ...
,
beta-blockers Beta blockers, also spelled β-blockers, are a class of medications that are predominantly used to manage abnormal heart rhythms (arrhythmia), and to protect the heart from a second heart attack after a first heart attack (secondary prevention). ...
, and immune modulators, such as TNF alpha inhibitors, are frequently used medications that have been linked to SCLE. Case reports of SCLE developing in cancers have been published.


Diagnosis

Like other forms of
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, blood cells, heart, ...
, systemic lupus erythematosus is primarily diagnosed clinically based on clinical features. When there is doubt about the diagnosis, a
skin biopsy Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 day ...
combined with a confirmatory histopathologic examination is recommended. Anti-SSA/Ro and anti-SSB/La laboratory studies are also recommended. In cases where
histopathology Histopathology (compound of three Greek words: 'tissue', 'suffering', and '' -logia'' 'study of') is the microscopic examination of tissue in order to study the manifestations of disease. Specifically, in clinical medicine, histopatholog ...
is unclear, direct
immunofluorescence Immunofluorescence (IF) is a light microscopy-based technique that allows detection and localization of a wide variety of target biomolecules within a cell or tissue at a quantitative level. The technique utilizes the binding specificity of anti ...
can be utilized to help confirm the diagnosis. At the dermal-epidermal junction, immunofluorescence reveals a granular pattern of
immunoglobulin An antibody (Ab) or immunoglobulin (Ig) is a large, Y-shaped protein belonging to the immunoglobulin superfamily which is used by the immune system to identify and neutralize antigens such as pathogenic bacteria, bacteria and viruses, includin ...
deposition.


See also

*
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, blood cells, heart, ...
*
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the Human body, body and composed of Human skin, skin, hair, Nail (anatomy), nails, and related muscle and glands. The major function o ...
*
List of human leukocyte antigen alleles associated with cutaneous conditions There are many human leukocyte antigen (HLA) alleles associated with conditions of or affecting the human integumentary system See also * List of allergens *List of cutaneous conditions associated with increased risk of nonmelanoma skin ca ...


References


Further reading

* *


External links


DermNet

Cleveland Clinic
{{Localized connective tissue diseases Cutaneous lupus erythematosus