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

• Secondary Pinkus’ Follicular Mucinosis
• Secondary 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. It may consist of many types including primary and secondary type
• Secondary Alopecia Mucinosa is a type of alopecia mucinosa that is associated with a variety of benign and malignant conditions
  • The benign conditions may include systemic lupus erythematosus and lichen simplex chronicus
  • The malignant conditions may include mycosis fungoides and Hodgkin lymphoma. It is important to note that many individuals with Secondary Alopecia Mucinosa may have mycosis fungoides
  • It may be also caused by certain medications, in which case it is known as drug-induced alopecia mucinosa
• The condition most commonly occurs on the scalp, face, and neck, though any part of the body can be affected. Secondary Alopecia Mucinosa can affect individuals of all ages; it is seen in both children and adults
• Some symptoms of this disorder include the appearance of pink papules (small bumps in the skin), loss of hair in the affected area, and itchy skin. Secondary Alopecia Mucinosa may be diagnosed by studying the presenting symptoms, skin biopsy, and establishing the underlying cause
• 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 is undertaken
• The prognosis of Secondary Alopecia Mucinosa is based on the underlying cause. It has generally better outcomes, if it is caused by a benign condition than by a malignant condition. The prognosis of medication-induced alopecia mucinosa is typically good upon stoppage of the drug causing the condition

Who gets Secondary Alopecia Mucinosa? (Age and Sex Distribution)

• Secondary Alopecia Mucinosa is a rare inflammatory skin condition that is mostly seen in the age group 40-70 years
• However, all age groups may be affected, though
  • Benign cases are more common in children
  • Lymphoma-associated cases of alopecia mucinosa are more common in older adults
• The disorder occurs worldwide, without racial, ethnic or gender bias

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

The risk factors of Secondary Alopecia Mucinosa may include the following:

• Systemic lupus erythematosus (SLE)
• Lichen simplex chronicus (LSC)
• Angiolymphoid hyperplasia
• An underlying lymphoma of the skin, mycosis fungoides, is a frequent cause of Secondary Alopecia Mucinosa. It can also be associated with lymphoma of the skin in approximately 15-30% of the cases
• Hodgkin lymphoma
• Kaposi sarcoma
• Certain medications such as adalimumab and imatinib

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 Secondary 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. Secondary Alopecia Mucinosa may occur due to the following conditions:

• Cancers and malignant tumors including:
  • Lymphoma-associated alopecia mucinosa is typically caused by a form of cutaneous T-cell lymphoma, known as mycosis fungoides. This condition directly involves the hair follicles
  • Other types of lymphoma, such as Hodgkin disease, may also cause alopecia mucinosa
  • Another malignant condition, called Kaposi sarcoma, can also be causative
• Benign skin and systemic conditions including:
  • Systemic lupus erythematosus (SLE)
  • Lichen simplex chronicus (LSC)
  • Angiolymphoid hyperplasia
• The administration of certain medications may also lead to the development of medication-induced alopecia mucinosa

What are the Signs and Symptoms of Secondary Alopecia Mucinosa?

The signs and symptoms of Secondary Alopecia Mucinosa may vary from one individual to another, depending on the cause and age of the affected individual. The adult form of alopecia mucinosa typically presents with more skin lesions on the body and lasts longer than the form found in children.

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 (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

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

How is Secondary Alopecia Mucinosa Diagnosed?

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

• A complete physical examination and review of the affected individual’s medical history (that includes current medications being taken)
• 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
• Additional testing to rule out other underlying conditions:
  • Physical examinations
  • Blood tests
  • Imaging studies

Note: 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 Secondary Alopecia Mucinosa?

Complications of Secondary 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 Secondary Alopecia Mucinosa Treated?

The following are the treatment options for Secondary Alopecia Mucinosa: 

• Use of topical, intralesional (injected into the affected skin area), or systemic (oral) corticosteroids. Corticosteroids are a type of steroid hormone that provides relief at sites of inflammation
• Antibiotic, antiviral, or antifungal medications to address infections
• Topical and systemic photochemotherapy (PUVA), a type of ultraviolet radiation therapy
• Use of creams containing nitrogen mustard
• 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
• Treatment of underlying lymphoma in lymphoma-associated cases of alopecia mucinosa, or undertaking treatment of the underlying benign conditions
• Discontinuation of the drug causing the condition

How can Secondary Alopecia Mucinosa be Prevented?

• It is generally not possible to prevent alopecia mucinosa that occurs secondary to certain underlying disorders or malignancies. However, treating the underlying condition, may help prevent further worsening of alopecia mucinosa
• Secondary Alopecia Mucinosa that occurs due to the intake of certain medications may be avoided by discontinuing usage of the causative medications

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

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

The prognosis of Secondary Alopecia Mucinosa depends on factors (diseases or malignancies) that cause the condition. The potential outcomes of Alopecia Mucinosa include:

• Early detection and treatment may be beneficial in limiting the complications, as well as to increase the success of treatment for underlying conditions
• The prognosis of individuals with malignant conditions, such as lymphoma, is dependent on the stage of the cancer, treatment response, and overall health of the affected individual
• Immediately stopping the use of drugs causing alopecia mucinosa and treating the skin symptoms can result in a good prognosis, when it is a drug-induced condition

Additional and Relevant Useful Information for Secondary 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.