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

• Lupus Erythematosus Panniculitis
• Lupus Erythematosus Profundus
• Lupus Profundus

What is Lupus Panniculitis? (Definition/Background Information)

• Lupus Panniculitis is a rare and chronic skin condition that causes the formation of painful subcutaneous nodules and thickened plaques on the body. The term ‘panniculitis’ indicates an inflammation of fat (adipose) tissue
• In some cases, Lupus Panniculitis is associated with cutaneous lupus erythematosus (CLE). Also, the condition may be seen before or after the onset of systemic lupus erythematosus (SLE), which is a condition that affects several parts of the body. Thus, Lupus Panniculitis is reportedly an uncommon type of lupus erythematosus
• The condition can occur in all ages, although most cases are observed in young-to-middle-aged adults. The cause of Lupus Panniculitis is presently unknown
• In many cases, the nodules are known to ulcerate and bleed, leaving behind scars on healing. In some individuals, Lupus Panniculitis can present severe cosmetic challenges due to disfigurement
• The treatment of Lupus Panniculitis is mostly undertaken using antimalarial medications. In case of severe symptoms, systemic corticosteroids may be necessary. Additionally, treatment of the associated lupus erythematosus, if any noted, is also undertaken
• The outcome of Lupus Panniculitis depends upon the severity of the condition. Generally, individuals with mild symptoms have a better prognosis than those presenting severe symptoms and complications from systemic lupus erythematosus

Who gets Lupus Panniculitis? (Age and Sex Distribution)

• Lupus Panniculitis is a rare condition that is mostly observed in young, middle-aged and older adults; most cases arise in the 20-60 years’ age range
• Both males and females are affected, although a female predominance is reported (female-male ratio is 9:2)
• No racial or ethnic group predilection is observed and the condition is observed worldwide

What are the Risk Factors for Lupus Panniculitis? (Predisposing Factors)

The risk factors for Lupus Panniculitis may include:

• Longstanding cutaneous lupus erythematosus, which is observed in nearly 33% of the individuals with Lupus Panniculitis
• Between 2-5% of the cases are associated with systemic lupus erythematosus (SLE)

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 Lupus Panniculitis? (Etiology)

• The cause of development of Lupus Panniculitis is presently unknown. It occurs from the presence of fat necrosis within the subcutaneous tissue
• The condition is known to be associated with the ‘skin form’ or ‘systemic form’ of lupus erythematosus, in some cases
• Research is currently on to identify the relevant causative factors

What are the Signs and Symptoms of Lupus Panniculitis?

The signs and symptoms of Lupus Panniculitis may include:

• The presence of firm, well-defined nodules and plaques causing thickened skin
• The size of the nodular lesions may range from 1 to 4 cm
• The lesions are noted subcutaneously, meaning they arise beneath the skin layers; many are painful
• The skin over the nodules may have a slightly reddish or purplish discoloration
• Any part of the body skin may be involved, but most are noticed on the head and neck region, arms, thighs, lower back, buttocks, and abdomen
• The lesions may also appear at sites of an injury or trauma
• These nodules and plaques are known to commonly ulcerate and bleed
• Other symptoms of the underlying conditions, if any, may be noted

How is Lupus Panniculitis Diagnosed?

Lupus Panniculitis may be diagnosed using the following tests and exams:

• Complete physical examination with comprehensive evaluation of medical history
• Assessment of the signs and symptoms
• Radiographic studies of the affected region, if needed
• 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
• Skin or tissue biopsy: A 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 with microscope findings, the pathologist arrives at a definitive diagnosis
• A differential diagnosis to exclude the following conditions (mostly other panniculitis types) may be necessary prior to a definitive diagnosis:
  • Cold panniculitis
  • Post-steroid panniculitis
  • Sclerema neonatorum
  • Subcutaneous fat necrosis of the newborn
  • Subcutaneous panniculitis-like T-cell lymphoma
  • Traumatic panniculitis

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 Lupus Panniculitis?

The complications of Lupus Panniculitis may include:

• The nodules may ulcerate and bleed, resulting in the formation of scars on healing
• Bacterial and fungal infections: The condition may providing a suitable environment for bacteria and fungi to grow and thrive, resulting in secondary infections
• Emotional stress and psychological trauma, due to cosmetic concerns; the condition can cause severe disfigurement in some. This is because of damage and loss of fat tissue underneath the skin resulting in a condition called lipoatrophy
• In 10-15% (and sometimes, even up to 50%) of the cases, Lupus Panniculitis can become either systemic lupus erythematosus affecting various parts of the body, later in life; or, it may also develop into discoid lupus erythematosus with more severe skin symptoms

How is Lupus Panniculitis Treated?

The following treatment measures may be considered for Lupus Panniculitis:

• Administration of antimalarial drugs, such as hydroxychloroquine, is known to be effective in treating the condition
• Systemic steroid medication; intralesional steroid injections
• Surgical excision of the nodules may be considered; surgical debridement
• Undertaking treatment of any associated condition, such as lupus erythematosus
• Symptomatic and supportive therapy

In pregnant women, antimalarial medication should not be used. If a woman is pregnant, it is always essential to ensure that a careful evaluation by a qualified healthcare provider is undertaken, prior to starting and/or performing any treatment.

How can Lupus Panniculitis be Prevented?

Current medical research has not established a method of preventing the occurrence of Lupus Panniculitis.

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

• The prognosis of Lupus Panniculitis is dependent upon the severity of the signs and symptoms and associated complications, if any present
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• In many individuals, the condition may be longstanding, and it is known to often relapse. Lupus Panniculitis may also develop into systemic lupus erythematosus with an involvement of the entire body

Additional and Relevant Useful Information for Lupus Panniculitis:

There are many different types of panniculitis and it has been difficult to obtain an accurate picture of their true incidence, since they may be associated with so many conditions. Some of the different types include:

• Lupus panniculitis
• Pancreatitis-associated panniculitis
• Alpha-1 antitrypsin deficiency associated panniculitis
• Infective panniculitis