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

• Follicular Mucinosis
• Pinkus’ Follicular Mucinosis
• Pinkus’ Mucinosis Follicularis

What is 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
• On a skin tissue biopsy, it is characterized by the presence of mucin, a stringy clear or white jelly-like substance in the walls of hair follicles, when examined under a microscope by a pathologist
• The condition most commonly occurs on the scalp, face, and neck, though any part of the body can be affected. Alopecia Mucinosa can affect individuals of all ages
• Alopecia Mucinosa can be classified as acute or chronic, based on the pattern of signs and symptoms observed. It can be also classified as primary and secondary, based on the cause. In the primary form, the cause is unknown; while in the secondary form, the cause of the condition is known
• Some signs and symptoms of the condition include the appearance of tiny pink bumps in the skin, loss of hair, and itchy skin. The chief method of diagnosis of Alopecia Mucinosa is by studying the presenting symptoms and through a skin biopsy
• Topical, systemic, or intralesional corticosteroids are most commonly used to treat this skin condition, though the effectiveness of treatment is generally limited. Additionally, treatment of any associated condition may be undertaken
• The prognosis of Alopecia Mucinosa depends on the type and severity of the condition. The primary forms (both acute and chronic) and secondary forms, when caused by benign conditions, typically have better prognoses than when caused by certain malignancies (such as lymphoma)

Who gets Alopecia Mucinosa? (Age and Sex Distribution)

• Alopecia Mucinosa is a rare inflammatory skin condition that may be seen in both children and adults
• The age demographics generally depends on the type of the condition, whether primary or secondary
• The disorder occurs worldwide, without racial, ethnic, or gender bias

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

Certain risk factors for Alopecia Mucinosa include:

• The secondary type of Alopecia Mucinosa is associated with a variety of benign and malignant conditions
• Use of certain medications are also known to result in the development of the secondary type
• No risk factors are evident for the primary type of Alopecia Mucinosa

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 Alopecia Mucinosa? (Etiology)

Alopecia Mucinosa results from an overproduction of mucin by cells in the hair follicle. Mucin is a glycoprotein and a component of mucus.

• The cause of primary Alopecia Mucinosa is unknown. It has been suggested that a reaction to persistent antigens (foreign organisms or substances), such as Staphylococcus aureus, may lead to the disorder
• Secondary Alopecia Mucinosa may occur due to certain medications, benign conditions, and malignancies (usually lymphomas)

What are the Signs and Symptoms of Alopecia Mucinosa?

The signs and symptoms of Alopecia Mucinosa may vary, depending on the age of the affected individual. It can also vary from one individual to another. The adult type of Alopecia Mucinosa typically presents with more skin lesions on the body and lasts longer than the type found in children.

The typical signs and symptoms include:

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

Additionally, the signs and symptoms of the underlying benign or malignant condition may be noted.

How is Alopecia Mucinosa Diagnosed?

The diagnosis of 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 signs and symptoms and physical inspection of the skin lesions
• A skin biopsy: The affected skin is taken from lesion sites and analyzed in lab to confirm the diagnosis. However, it is difficult to distinguish benign and malignant (lymphoma-associated) Alopecia Mucinosa from this method
• Additional testing to rule out any underlying lymphoma, which may include:
  • Physical examinations
  • Blood tests
  • Imaging studies

Generally, benign and lymphoma-associated cases are distinguished based on the presence of a high number of eosinophils (a type of white blood cell) in skin lesion biopsies of benign occurrences, as opposed to the presence of atypical cells in lesions of lymphoma-associated cases.

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 Alopecia Mucinosa?

Complications of Alopecia Mucinosa may include the following:

• Persistent and chronic skin lesions, especially if the lesions are widespread
• 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
• Complications of the underlying conditions

How is Alopecia Mucinosa Treated?

Currently, there are no standard treatment measures for Alopecia Mucinosa; and, it is treated on a case-by-case basis. Various treatment options have shown little success in treating Alopecia Mucinosa, and most cases resolve on their own within months to few years (usually 2 years).

The following are the treatment options for Alopecia Mucinosa: 

• Waiting to let symptoms resolve on their own, based on the judgement of the healthcare provider. The resolution may take place anywhere within 2 to 24 months
• Use of topical, intralesional (injectable), or systemic (oral) corticosteroids. Corticosteroids are types of steroid hormones that provides relief at sites of inflammation
• Topical steroids combined with tretinoin can be effective in children
• Use of creams containing nitrogen mustard
• Antibiotic, antiviral, or antifungal medications to address infections
• Topical and systemic photochemotherapy (PUVA), 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
• Psychiatric therapy for depression
• Undertaking treatment of the underlying cause of Alopecia Mucinosa is very important

How can Alopecia Mucinosa be Prevented?

The exact cause of Alopecia Mucinosa is unknown, and hence, no guidelines or methods exist presently for the prevention of this condition.

• Early detection and treatment may be beneficial in limiting the complications, as well as to increase the success of treatment for underlying conditions such as lymphoma
• Active research is currently being performed to explore the possibilities for treatment and prevention of conditions such as Alopecia Mucinosa

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

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

The prognosis of Alopecia Mucinosa is generally favorable with appropriate treatment. The potential outcomes of Alopecia Mucinosa include:

• Complete resolution of symptoms on their own over time, or through suitable treatment
• Complications, such as permanent hair loss at the site of skin lesions, with or without proper treatment, if scarring has occurred
• A less favorable prognosis occurs in individuals with an underlying malignancy (such as a lymphoma)

Additional and Relevant Useful Information for Alopecia Mucinosa:

• Benign Alopecia Mucinosa is known as the primary form, while the secondary form is lymphoma-associated. Acute primary Alopecia Mucinosa is the most commonly seen condition
• 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.