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

• Idiopathic Alopecia Mucinosa (Acute)
• Primary Acute Pinkus’ Follicular Mucinosis
• Primary Acute Pinkus’ Mucinosis Follicularis

What is Primary Acute Alopecia Mucinosa? (Definition/Background Information)

• Alopecia mucinosa is a rare inflammatory condition of hair-bearing skin, resulting in a thickening of skin that is accompanied by hair loss. It may consist of several types including primary and secondary types
• Primary Acute Alopecia Mucinosa is a type of alopecia mucinosa, in which the cause of the condition remains unknown. It is generally seen in young individuals and tends to resolve within a few years
• The condition most commonly affects the face and neck region; it can involve any part of the body. Some symptoms of the condition include the appearance of tiny pink papules of the skin, loss of hair, and itchy skin. The chief method of diagnosis of Primary Acute Alopecia Mucinosa is a skin biopsy and through evaluation of the presenting signs and symptoms
• Topical, systemic, or intralesional corticosteroids are most commonly used to treat this skin condition; the effectiveness of treatment is generally limited. In a majority of patients, Primary Acute Alopecia Mucinosa slowly gets better with complete resolution in about 2 years

Who gets Primary Acute Alopecia Mucinosa? (Age and Sex Distribution)

• Primary Acute Alopecia Mucinosa is a rare skin condition that is observed in children and young adults. Most of the affected individuals are less than 40 years old
• When the condition is present in infants it is known as Primary Acute Neonatal Alopecia Mucinosa
• The disorder occurs worldwide, without racial, ethnic or gender bias

What are the Risk Factors for Primary Acute Alopecia Mucinosa? (Predisposing Factors)

• Children and adults below 40 years of age develop Primary Acute Alopecia Mucinosa. Presently, this is the only identified risk factor

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 Primary Acute Alopecia Mucinosa? (Etiology)

• The cause of Primary Acute Alopecia Mucinosa is currently unknown. It has been suggested that a reaction to persistent antigens (foreign organisms or substances), such as Staphylococcus aureus, may lead to the development of this disorder
• Alopecia mucinosa results from an overproduction of mucin by cells in the hair follicle. Mucin is a glycoprotein and a component of mucus

What are the Signs and Symptoms of Primary Acute Alopecia Mucinosa?

The signs and symptoms of Primary Acute Alopecia Mucinosa may vary depending on the age of the affected individual. Extensive skin lesions are not observed in this type, unlike other forms of alopecia mucinosa.

The typical signs and symptoms of Primary Acute Alopecia Mucinosa include:

• Red or pink grouped papules (raised bumps of the skin): These may be limited to one location or appear throughout the body
• The number of skin lesions may be few or many
• Common locations of the lesions may include:
  • Face
  • Head and neck region
• Red plaques (large, raised, or thickened patches of skin) with accentuated pores that are hair follicle openings
• Oozing mucin from pores, especially if the skin is touched or squeezed
• Hair loss in the affected (hair-bearing) area of skin, which may result in permanent hair loss
• Itchy skin at the site of lesions (pruritic plaques); scratching can lead to scarring
• There may be scaling over the skin lesions
• Formation of skin nodules

How is Primary Acute Alopecia Mucinosa Diagnosed?

The diagnosis of Primary Acute Alopecia Mucinosa is made through the following tests and exams:

• A complete physical examination and review of the affected individual’s medical history
• Assessment of symptoms and physical inspection of skin lesions
• Skin biopsy: A skin 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 may be performed to eliminate the following conditions prior to arriving at a diagnosis of alopecia mucinosa:

• Alopecia areata
• Seborrheic dermatitis
• Telogen effluvium

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 Primary Acute Alopecia Mucinosa?

Complications of Primary Acute Alopecia Mucinosa may include the following:

• Permanent hair loss at the site of lesions, if scarring has taken place
• Emotional stress due to cosmetic concerns
• Superimposed bacterial, viral, and fungal infections at the affected skin sites

How is Primary Acute Alopecia Mucinosa Treated?

The following are the treatment options for Primary Acute Alopecia Mucinosa:

• Wait and watch approach: Waiting to let the symptoms resolve on their own, based on the judgement of the healthcare provider. The resolution usually occurs anywhere within 2 to 24 months
• Use of topical, intralesional (injected), or systemic (oral) corticosteroids. Corticosteroids are a type of steroid hormone that provides relief at sites of inflammation
• Topical retinoid medications such as tretinoin
• Use of creams containing nitrogen mustard
• Antibiotic, antiviral, or antifungal medications to address infections if detected
• Topical and systemic photochemotherapy (PUVA), which is a type of ultraviolet radiation therapy
• UVA1 phototherapy: The skin is exposed to electromagnetic radiation or wavelengths of light between 340 and 400 nanometers. The therapy works by limiting cell-mediated immune function
• Emotional assistance therapy for depression

How can Primary Acute Alopecia Mucinosa be Prevented?

The exact cause of Primary Acute Alopecia Mucinosa is unknown. Currently there are no guidelines or methods to prevent the condition.

• Early detection and treatment may be beneficial in limiting the complications
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as alopecia mucinosa

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

What is the Prognosis of Primary Acute Alopecia Mucinosa? (Outcomes/Resolutions)

• The prognosis of Primary Acute Alopecia Mucinosa is generally positive in most cases
• A complete resolution of symptoms on their own or through treatment is observed within a maximum period of 2 years (minimum period 2 months)

Additional and Relevant Useful Information for Primary Acute Alopecia Mucinosa:

Alopecia mucinosa belongs to a subset of conditions known as follicular mucinosis. There are two other conditions that belong to the same subset and they include, urticaria-like follicular mucinosis (which is very uncommon), and cutaneous lymphoma related follicular mucinosis.