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

• Atypical Xanthoma Disseminatum
• Diffuse Normolipemic Plane Xanthoma (DNPX)
• Diffuse Plane Xanthoma

What is Diffuse Plane Xanthomatosis? (Definition/Background Information)

• Diffuse Plane Xanthomatosis (DPX) is the widespread occurrence of flat yellow-orange lesions (known as xanthomas) on skin, mostly due to an underlying systemic disorder
• In nearly 50% of the cases, the systemic illness is a blood-related disorder such as leukemia or multiple myeloma. In some individuals, an association with a metabolic condition, such as familial hyperlipemia or biliary cirrhosis is noted
• It is important to note that the presence of xanthomas may be reported several years before other significant symptoms of the underlying condition are manifested. Thus, a diagnosis of Diffuse Plane Xanthomatosis needs to be followed through with other suitable tests and procedures to diagnose the underlying systemic condition
• Diffuse Plane Xanthomatosis is generally observed in adults. The lesions are usually painless and numerous, appearing in patches. They can be present anywhere in the body; commonly on the back, chest, shoulders, face and neck
• The main treatment involves treating the underlying systemic disorder causing Diffuse Plane Xanthomatosis. The skin (xanthomatous) lesions may be treated through laser therapy, abrasion therapy, or surgery
• The prognosis for Diffuse Plane Xanthomatosis is good with suitable treatment of the underlying condition causing the skin lesions. However, in some individuals, DPX is not known to respond well to therapy

Who gets Diffuse Plane Xanthomatosis? (Age and Sex Distribution)

• Diffuse Plane Xanthomatosis is an uncommon condition that is usually observed in adults; although, rarely, children are known to be affected
• Both males and females may be affected
• Individuals of all racial and ethnic background may be affected. Worldwide, no geographical localization has been reported

What are the Risk Factors for Diffuse Plane Xanthomatosis? (Predisposing Factors)

The predisposing factors for Diffuse Plane Xanthomatosis may include a wide variety of disorders such as the following:

• Blood-associated disorders and malignancies:
  • Multiple myeloma
  • Leukemia including chronic myelomonocytic leukemia, chronic granulocytic leukemia, etc.
  • Lymphoma including Waldenström's macroglobulinemia, Sézary syndrome, etc.
  • Monoclonal gammopathy
  • Langerhans' cell histiocytosis
  • Cryoglobulinemia
  • Dysglobulinemia
  • Castleman's disease
  • A variety of other paraproteinemias
  • Other reticuloendothelial disorders
• Certain skin conditions such as:
  • Actinic reticuloid
  • Photosensitive eczema
  • Exfoliative dermatitis
• Budd-Chiari syndrome
• Eosinophilic granulomatosis
• Familial hyperlipemia
• Conditions causing biliary obstruction or bile duct obstruction; biliary cirrhosis
• Rheumatoid arthritis

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 Diffuse Plane Xanthomatosis? (Etiology)

The cause of development of Diffuse Plane Xanthomatosis (DPX) is unknown.

• It is caused by abnormal accumulation of lipids in macrophages, which are a type of white blood cell. Macrophages phagocytize (ingest into their interior) certain materials in their role as immune cells
• The condition has been seen in individuals with a variety of diseases and conditions including multiple myeloma, monoclonal gammopathy, leukemias, lymphoma, and certain autoimmune disorders
• Some researchers consider DPX to be a type of non-X histiocytosis, a group of skin syndromes that share localized collections of histiocytes which are non-aggressive in behavior, and sometimes self-heal. The condition can also be seen in individuals without an abnormality in lipids (normal lipid levels) such as dyslipidemia and dyscholesterolemia
• Research is being performed to better understand the cause of Diffuse Plane Xanthomatosis

What are the Signs and Symptoms of Diffuse Plane Xanthomatosis?

The signs and symptoms of Diffuse Plane Xanthomatosis may include:

• Presence of large cutaneous plaques distributed all over the body
• The body sites mainly affected include the head and neck region (including the eyelids), shoulders, armpits, upper back and chest, along  the bends of joints, groin and buttocks
• The skin lesions appear as yellow brown-orange patches that may be arranged symmetrically on the body
• Even though the lesions may cover large areas on the body, they typically do not present any pain and are non-inflammatory
• Some individuals may present urticaria (itchy skin rashes) and angioedema (swelling due to fluid accumulation)

Additional signs and symptoms of the underlying/associated condition may be noted.

How is Diffuse Plane Xanthomatosis Diagnosed?

A diagnosis of Diffuse Plane Xanthomatosis (DPX) may involve the following tests and exams:

• A thorough medical history and physical examination
• Liver function test
• Lipid profile test
• 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 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 with microscope findings, the pathologist arrives at a definitive diagnosis

Diffuse Plane Xanthomatosis can be associated with systemic conditions. Sometimes, DPX appears months/years before the underlying systemic condition is apparent. Hence, individuals diagnosed with DPX should have a thorough checkup to rule out underlying or associated conditions. If no underlying conditions are noted, then close follow-up should be performed for early detection of any disorder that may develop (or manifest with symptoms) in future.

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 Diffuse Plane Xanthomatosis?

The complications of Diffuse Plane Xanthomatosis may include:

• Severe cosmetic concerns and emotional stress
• Scratching the lesions may lead to bleeding and ulceration, which may result in secondary infections
• Formation of new lesions at other body sites, or recurrence of xanthomas after treatment
• Complications that arise from the underlying disorder

How is Diffuse Plane Xanthomatosis Treated?

The primary treatment of Diffuse Plane Xanthomatosis involves treating the underlying disorder. The treatment measures for the skin condition may involve:

• Application of topical trichloroacetic acid (TCA)
• Ablative laser therapy
• Dermabrasion 
• Electrodessication
• Surgical excision and removal

Follow-up care with regular screening and check-ups are important post-treatment.

How can Diffuse Plane Xanthomatosis be Prevented?

• Currently, there are no known methods to prevent the occurrence of Diffuse Plane Xanthomatosis (DPX)
• Treating the underlying condition appropriately may help prevent recurrence of DPX

What is the Prognosis of Diffuse Plane Xanthomatosis? (Outcomes/Resolutions)

• The prognosis of Diffuse Plane Xanthomatosis (DPX) is good with appropriate treatment of the underlying condition in some individuals
• The skin lesions are known to regress with adequate management of the underlying condition. However, in some individuals, DPX is persistent and resists adequate treatment

Since, in many cases, the skin symptoms (xanthomas) appear before symptoms of the underlying disorder are manifested, it is important to maintain close follow-up with regular testing for several years, if needed, until the underlying condition causing DPX is definitively diagnosed.

Additional and Relevant Useful Information for Diffuse Plane Xanthomatosis:

• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse