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

• Bazex Syndrome (Acrokeratosis Neoplastica)
• Paraneoplastic Acrokeratosis

What is Acrokeratosis Neoplastica? (Definition/Background Information)

• Acrokeratosis Neoplastica is a rare skin condition which is characterized by plaques and lesions on the fingers, toes, and other external body parts, resembling features of psoriasis (another skin disorder)
• Individuals with an internal cancer have an elevated risk of developing Acrokeratosis Neoplastica, as there is a strong association with squamous cell carcinoma of the upper GI and respiratory tracts. There have also been reported cases involving other tumor types
• The condition is mostly seen in adult males. The affected individuals present with abnormal nails, scaly lesions, and thickening of the palms and soles
• The diagnosis of Acrokeratosis Neoplastica is made on the basis of radiological (computerized) images, blood tests, and tissue biopsies
• The main treatment involves attempting to resolve the underlying cancer, and the use of topical corticosteroid creams to help control and treat skin lesions
• The prognosis is determined by the severity of the underlying condition causing Acrokeratosis Neoplastica and by the response of the affected individual to treatment

Who gets Acrokeratosis Neoplastica? (Age and Sex Distribution)

• Acrokeratosis Neoplastica is a condition that has almost exclusively been found to affect males, typically over the age of 40 years
• No racial or ethnic predilection is reported for the occurrence of the disorder

What are the Risk Factors for Acrokeratosis Neoplastica? (Predisposing Factors)

• An internal malignancy, typically squamous cell carcinoma (SCC), is the biggest risk factor for developing Acrokeratosis Neoplastica. Frequently, the carcinoma involves the upper gastrointestinal tract and chest region, involving the esophagus, larynx, or lung. Other SCC sites include the cervix, skin, thymus, and vulva. In some cases, the site of origin of the primary SCC tumor is unknown
• Other malignancies that may be associated with Acrokeratosis Neoplastica include:
  • Adenocarcinoma of pancreas
  • Carcinoid tumor
  • Cholangiocarcinoma
  • Ductal carcinoma of breast
  • Liposarcoma
  • Lymphoma
  • Multiple myeloma (MM)
  • Poorly-differentiated carcinoma, NOS
  • Small cell carcinoma of lung
  • Tonsil cancer
  • Urothelial carcinoma
  • Well-differentiated carcinoma of thymus

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 Acrokeratosis Neoplastica? (Etiology)

The exact cause and mechanism of Acrokeratosis Neoplastica development is unknown.

• A strong association of squamous cell carcinoma of the upper respiratory or gastrointestinal (GI) tract and Acrokeratosis Neoplastica has been established
• It has been suggested that circulating antibodies to antigens of squamous cell carcinomas may contribute to its development

What are the Signs and Symptoms of Acrokeratosis Neoplastica?

The signs and symptoms of Acrokeratosis Neoplastica may include the following:

• Scaly, symmetrical, red plaques over the peripheral body parts
• Lesions on the fingers, feet, nose, and ears during the early stages of Acrokeratosis Neoplastica
• In more advanced cases, skin lesions may be observed on the cheeks, elbows, knees, palms of the hands and soles of the feet
• Changes in the appearance of the nails: thickening, discoloration, and ridging
• Swelling of the fingers and toes may also be observed

Acrokeratosis Neoplastica is generally categorized into one of the three following stages:

• Stage 1: Presence of lesions on the ears, nails and fingers
• Stage 2: Keratosis of the palms of the hands and the soles of the feet
• Stage 3: Presence of lesions on the knees, elbows, and torso

How is Acrokeratosis Neoplastica Diagnosed?

A healthcare provider diagnoses Acrokeratosis Neoplastica based on the following tests and exams:

• Complete physical examination and assessment of symptoms
• An evaluation of personal and family medical history pertinent to cancers, with emphasis on squamous cell carcinoma
• Tests to ascertain squamous cell carcinoma, which may include:
  • Chest X-ray
  • Computerized tomography (CT) scan, also known as CAT scan, for detailed three-dimensional images of structures inside the body
  • Magnetic resonance imaging (MRI) scan of the affected part
  • Positron emission tomography (PET) of the body, especially to detect spread of cancer (if any)
  • Extensive workup that includes biochemical tests, complete blood count, and physical examination of the chest, pelvis, head and neck region, etc.
  • Tissue biopsies
  • Endoscopy: Endoscopic investigation of the larynx, pharynx, and gastrointestinal tract. Endoscopy is a minimally-invasive procedure that uses an optical instrument to examine the inner linings of the gastrointestinal tract
  • Bronchoscopy: Bronchoscopy is a minimally-invasive procedure that uses an optical instrument to examine the inner linings of the bronchial tubes

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 Acrokeratosis Neoplastica?

Acrokeratosis Neoplastica usually resolves with treatment and no known complications are generally observed. However, complications may arise due the underlying malignancy (and its treatment), which may include:

• Local invasion and spread of cancer to other parts of the body
• Recurrence of the malignancy following treatment/surgery
• Side effects of radiation therapy that may include sunburn-like rashes (at the spot where radiation was targeted), red or dry skin, heaviness of the breasts, and general fatigue
• Side effects of chemotherapy, such as nausea, vomiting, hair loss, decreased appetite, mouth sores, fatigue, low blood cell counts, and a higher chance of developing infections
• The treatments can also cause infertility in men and women. Hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy

How is Acrokeratosis Neoplastica Treated?

The aim of treatment of Acrokeratosis Neoplastica is to address the underlying malignancy. Once the internal malignancy has been successfully treated, the lesions associated with Acrokeratosis Neoplastica, are also known to resolve.

Symptomatic treatment for the skin lesions may include:

• Topical corticosteroids and creams containing urea, lactic acid, or salicylic acid may be used to reduce the thickness of the skin lesions
• Therapy with a type of vitamin D is also reported as being successful
• Acitretin (an oral retinoid) helps with the skin features of the condition

The general treatment for squamous cell carcinomas include:

• Surgery: Depending on the stage of the squamous cell carcinoma, surgery might be a viable option. However, in case the lung is involved, then the location of the tumor in the periphery makes it commonly associated with metastasis
• Chemotherapy: These drugs are typically taken as pills, or injected directly into a vein. The drugs travel through the body to kill any cancer cells
• Radiation therapy: It involves the use of X-rays to kill cancerous cells. High-powered radiation beams are directed to specific body regions to target the cancer. Radiation may be administered by a machine placed externally, or internally, through a device placed at or near the target tissue

How can Acrokeratosis Neoplastica be Prevented?

Currently, there are no methods or guidelines available for the prevention of Acrokeratosis Neoplastica.

• However, the following guidelines may help reduce the risk of developing squamous cell carcinoma, which shows a strong association with Acrokeratosis Neoplastica:
  • Complete smoking cessation and avoiding exposure to secondhand smoke
  • Engaging in regular physical activity
  • Eating adequate amounts of fruits and vegetables: A healthy diet, low in saturated fats and rich in many fruits and vegetables, may help decrease one’s risk for cancer
  • Avoiding exposure to toxic materials and chemicals 
  • Avoiding exposure to radon gas
  • Limiting alcohol consumption
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Acrokeratosis Neoplastica

Regular medical screening at periodic intervals with tests and physical examinations are highly recommended.

What is the Prognosis of Acrokeratosis Neoplastica? (Outcomes/Resolutions)

• In general, the prognosis of Acrokeratosis Neoplastica is dependent on resolution of the underlying internal malignancy. Approximately, 90% of the cases are known to resolve with suitable treatment of the predisposing malignancy
• About 10% of the affected individuals are reported to have recurrent or non-responsive Acrokeratosis Neoplastica

Additional and Relevant Useful Information for Acrokeratosis Neoplastica:

• Squamous cell carcinoma of lung is a type of lung cancer that mostly affects tobacco smokers; there is a very strong association between this cancer type and smoking

The following resource can help you understand scabies:

https://www.dovemed.com/diseases-conditions/squamous-cell-carcinoma-lung/