What are the other Names for this Condition? (Also known as/Synonyms)

• HLE (Hypertrophic Lupus Erythematosus)
• Hypertrophic Variant of Chronic Cutaneous Lupus Erythematosus
• Verrucous Lupus Erythematosus

What is Hypertrophic Lupus Erythematosus? (Definition/Background Information)

• Hypertrophic Lupus Erythematosus (HLE) is described as a rare manifestation of Chronic Cutaneous Lupus Erythematosus (CCLE) skin diseases that are subtypes of Cutaneous Lupus Erythematosus disorders primarily affecting the skin
• Hypertrophic Lupus Erythematosus appears in the lesional skin of Discoid Lupus Erythematosus DLE), a subclass of CCLE. The name “discoid” stems from the circular or ‘disc-shaped’ skin lesions on the head and neck region
• Cutaneous Lupus Erythematosus (CLE) constitutes a wide group of autoimmune disorders. Autoimmune disorders cause the body to mistake normal healthy tissues and organs for dangerous foreign material and attack them leading to long-term chronic inflammation
• In many cases, Hypertrophic Lupus Erythematosus may be misdiagnosed as other skin conditions, such as hypertrophic lichen planus or keratoacanthoma, due to a similarity between these conditions and relative rarity of HLE
• Hypertrophic Lupus Erythematosus usually affects middle-aged women and is characterized by thickened skin plaques with or without scaling, usually on the arms or face. In many cases, flatter, more typical  discoid skin lesions of DLE are also present
• The exact cause of Hypertrophic Lupus Erythematosus is not yet known. In a few cases, it is associated with systemic lupus erythematosus (SLE), which is a serious disorder that can potentially involve several organs and systems of the body
• The condition may be diagnosed through physical examination, complete medical history, and specialized blood tests. The treatment of Hypertrophic Lupus Erythematosus may involve topical medications, oral medication, and systemic corticosteroids for severe symptoms
• The prognosis depends on the severity of the disorder and its response to therapy. In case of delayed diagnosis and/or treatment, Hypertrophic Lupus Erythematosus can result in complications such as scarring and severe emotional stress

Who gets Hypertrophic Lupus Erythematosus? (Age and Sex Distribution)

• Individuals of any age may be affected by Hypertrophic Lupus Erythematosus; but it is more commonly seen in young and middle-aged adults
• Women are more prone to be affected than men
• Both fair-skinned and dark-skinned individuals can be affected, although darker-skinned Africans and Asians have a higher risk than fair-skinned Caucasians for HLE

What are the Risk Factors for Hypertrophic Lupus Erythematosus? (Predisposing Factors)

Risk factors associated with Hypertrophic Lupus Erythematosus may include:

• Presence of systemic lupus erythematosus (SLE), an autoimmune disorder affecting several systems and organs of the body. It is reported that about 25% of chronic cutaneous lupus erythematosus cases are associated with SLE
• Female gender
• Genetic predisposition: A family history of cutaneous lupus erythematosus, which may be related to HLA genetic makeup, may increase one’s risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hypertrophic Lupus Erythematosus? (Etiology)

The exact cause of Hypertrophic Lupus Erythematosus (HLE) is not fully known or understood.

• It is most likely an autoimmune disorder, where antibodies of the body that fight diseases or infections attack one’s healthy tissues instead. Such an attack by the body’s immune cells or antibodies against its tissue can be severe, resulting in significant signs and symptoms
• HLE is usually not associated with systemic lupus erythematosus (SLE). When associated with SLE, it can often result in inflammation in other parts of the body besides the skin
• Some individuals may have a genetic predisposition for the condition; which may then be triggered by infections, certain drugs, or on exposure to sunlight

What are the Signs and Symptoms of Hypertrophic Lupus Erythematosus?

The signs and symptoms of Hypertrophic Lupus Erythematosus (HLE) may vary from one individual to another. It may be mild in some individuals or severe in others and may include:

• The skin patches are in the form of highly-thickened plaques with hardened borders; scaling is usually minimal, but this may vary from one individual to another
• The lesions are non-itchy, well-defined, and may show whitish patches
• Most are present on the forearm, head and neck region (including face), and chest and back; typically on sun-exposed areas
• The skin lesions can resemble viral warts; it may give rise to a suspicion of squamous cell skin cancer, in some cases
• If it affects the palms and soles, it is called palmoplantar lupus erythematosus, a type of palmoplantar keratoderma. These may appear as focal (localized) lesions or diffuse (widespread) lesions
• Some hypertrophic lesions are large (about 5 cm in size), irregular, and may involve the elbows
• Additionally, in HLE, the presence of discoid skin lesions, characteristic of discoid lupus erythematosus are usually noted
• The skin lesions heal with scarring
• Exposure to sunlight makes the condition worse

The signs and symptoms of systemic lupus erythematosus, if present, may be noted. Apart from the skin symptoms, this may include low-grade fever, appetite loss, joint pain, enlarged lymph nodes, and involvement of various body organs. It has been reported that chronic SLE may also show Hypertrophic Lupus Erythematosus skin lesions.

Certain factors can worsen the signs and symptoms (causing flare-ups), and these include:

• Trauma
• Smoking
• Certain viral infections
• Certain medications
• Hormonal influence

How is Hypertrophic Lupus Erythematosus Diagnosed?

Hypertrophic Lupus Erythematosus may be diagnosed using the following tests and exams:

• Complete physical examination with a comprehensive evaluation of one’s medical history
• Assessment of the signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood tests that include:
  • Routine blood testing (sedimentation rate/C-reactive protein)
  • Antinuclear antibody (ANA) levels blood test to look for antibodies in blood
  • Serological blood test for anti-double-stranded DNA (ds-DNA)
  • Extractable nuclear antigen antibodies panel blood test to detect anti-Ro antibodies and anti-La antibodies (also known as SSA and SSB antibodies)
  • Blood chemistry panels
• Body fluid analysis, if needed
• Skin/tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed), and microscope findings, the pathologist arrives at a definitive diagnosis
• Direct immunofluorescence studies on tissue biopsies
• A differential diagnosis is undertaken to exclude hypertrophic lichen planus, keratoacanthoma, or tuberous xanthoma that show symptoms similar to HLE

In order to diagnose systemic lupus erythematosus (SLE), a set of criteria established by the US-based organization American College of Rheumatology may be used.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hypertrophic Lupus Erythematosus?

The complications of Hypertrophic Lupus Erythematosus may include:

• Secondary bacterial and fungal infection due to scratching, bleeding, and ulceration
• The hair follicles may be completely destroyed, causing permanent hair loss
• With time, scarring and atrophy of skin are usually reported
• Cosmetic concerns causing emotional and psychological stress
• About 5-10% of the individuals with HLE are known to have an associated systemic lupus erythematosus, which can involve the entire body
• Severe systemic lupus erythematosus (SLE) causing involvement of vital organs, such as the brain, lungs, heart, liver, and kidneys, resulting in organ dysfunction
• Longstanding ulcers can result in the development of squamous cell carcinoma (SCC)

How is Hypertrophic Lupus Erythematosus Treated?

The treatment undertaken for Hypertrophic Lupus Erythematosus consists of decreasing inflammation and autoimmune activity in the body and minimizing the occurrence of flare-ups. The treatment measures may include:

• Topical medication and ointments
• Topical/oral retinoid therapy
• Intralesional corticosteroid injections, when drugs are injected into the skin lesions
• Laser therapy for cosmetic skin lesions
• Cosmetic surgery to remove disfiguring scars
• Systemic therapy using antimalarial drugs are a first line of treatment
• Systemic steroid therapy
• Avoid visible and ultraviolet light, using time-of-day precautions, clothing, and sunscreen
• In those who limit sun exposure, vitamin D supplements may be administered
• Immunosuppressive medication for severe cases
• Intravenous immunoglobulin (IVIG) therapy
• Anti-inflammatory monoclonal antibody therapy

Treatment measures for underlying systemic lupus erythematosus, if present, may be instituted.

It is essential to note that Hypertrophic Lupus Erythematosus in pregnant women should be carefully evaluated by a qualified healthcare provider before starting and/or performing any treatment. This is because some of the treatment measures may have the potential to affect the developing fetus, and hence, the guidance of a healthcare expert is crucial.

How can Hypertrophic Lupus Erythematosus be Prevented?

Currently, there is no effective preventive method available for Hypertrophic Lupus Erythematosus (HLE).

• Flare-ups can be prevented by managing medications, avoiding sun exposure, utilizing suitable sunscreens and clothing (cover-up skin exposed to sunlight), and close monitoring of the symptoms
• Avoiding factors that aggravate the condition through the following measures:
  • Avoidance of smoking
  • Avoid scratching or picking the skin lesions
  • Undertaking early treatment of viral infections
  • Addressing issues causing hormonal imbalance in the body
• In pregnant women with HLE, careful and periodic monitoring of the pregnancy is advised and recommended
• Educating the individual and keeping them well-informed about lupus is important and beneficial

What is the Prognosis of Hypertrophic Lupus Erythematosus? (Outcomes/Resolutions)

The prognosis of Hypertrophic Lupus Erythematosus depends on the severity of the signs and symptoms, its association with systemic lupus erythematosus, and the response to treatment.

• Individuals with mild cases of HLE, who respond well to therapy, have a better prognosis than those with severe symptoms and poor response to treatment
• In many cases, the prognosis is good, and with proper treatment and monitoring of the condition; many individuals are able to lead normal, healthy lives while managing flares
• It is important that appropriate treatment be provided during pregnancy and close monitoring of both the mother and developing fetus be undertaken

Additional and Relevant Useful Information for Hypertrophic Lupus Erythematosus:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/systemic-lupus-erythematosus/