What are the other Names for the Condition? (Also known as/Synonyms)
- Idiopathic Alopecia Mucinosa (Chronic)
- Primary Chronic Pinkus’ Follicular Mucinosis
- Primary Chronic Pinkus’ Mucinosis Follicularis
What is Primary Chronic 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 many types including primary and secondary type
- Primary Chronic Alopecia Mucinosa is a type of alopecia mucinosa, in which the cause of the condition remains unknown. It is generally seen in older individuals and can last for several years or even a lifetime
- The condition can involve any part of the body including the face and neck region. Some 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 Primary Chronic Alopecia Mucinosa is a skin biopsy and by evaluating the presenting signs and symptoms. The signs and symptoms may ‘come and go’ in this type
- Topical, systemic, or intra-lesional corticosteroids are used to treat this skin condition, though the effectiveness of treatment is generally limited. Also, in a majority, Primary Chronic Alopecia Mucinosa can persist for a long period
Who gets Primary Chronic Alopecia Mucinosa? (Age and Sex Distribution)
- Primary Chronic Alopecia Mucinosa is a rare skin condition that is observed in adults over the age of 40 years old
- The disorder occurs worldwide, without racial, ethnic or gender bias
What are the Risk Factors for Primary Chronic Alopecia Mucinosa? (Predisposing Factors)
- Adults over 40 years old have the highest risk for Primary Chronic Alopecia Mucinosa. Presently, no other risk factors have been identified
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 Chronic Alopecia Mucinosa? (Etiology)
- The cause of Primary Chronic 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
- 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 Chronic Alopecia Mucinosa?
The signs and symptoms of Primary Chronic Alopecia Mucinosa may vary from one individual to another; they may be mild in some, and severe in others. Also, extensive skin lesions may be observed in this type of alopecia mucinosa.
The typical signs and symptoms of Primary Chronic Alopecia Mucinosa 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
- The skin lesions may be present all over the body
- 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 (hair-bearing) area of skin, which may result in scarring
- Itchy skin at the site of lesions (pruritic plaques); itching can lead to scarring
- There may be scaling over the skin lesions
- Formation of skin nodules
- The skin signs and symptoms may subside and then recur after a certain time interval; this may be repetitive
How is Primary Chronic Alopecia Mucinosa Diagnosed?
The diagnosis of Primary Chronic 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 Chronic Alopecia Mucinosa?
Complications of Primary Chronic 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
- The condition can be chronic and recurrent
How is Primary Chronic Alopecia Mucinosa Treated?
The following are the treatment options for Primary Chronic Alopecia Mucinosa:
- Use of topical, intra-lesional (injectable), or systemic (oral) corticosteroids. Corticosteroids are a type of steroid hormone that provides relief at sites of inflammation
- Use of creams containing nitrogen mustard
- Antibiotic, antiviral, or antifungal medications to address infections
- 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
- Psychiatric therapy for depression
How can Primary Chronic Alopecia Mucinosa be Prevented?
The exact cause of Primary Chronic Alopecia Mucinosa is unknown, and hence, 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 Chronic Alopecia Mucinosa? (Outcomes/Resolutions)
- The prognosis of Primary Chronic Alopecia Mucinosa is variable and may be evaluated on a case-by-case basis
- The disorder can last for a lifetime in some individuals; while in others, it may be present for many years
- Also, the presentations of the disorder (symptoms) can chronically recur and subside, resulting in long-term stress and anxiety
Additional and Relevant Useful Information for Primary Chronic 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.