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

• ULFM (Urticaria-Like Follicular Mucinosis)

What is Urticaria-Like Follicular Mucinosis? (Definition/Background Information)

• Follicular mucinosis is a type of skin condition in which an accumulation of mucin is observed around the hair follicles. There are 3 conditions that belong to this group and they include - alopecia mucinosa, cutaneous lymphoma related follicular mucinosis, and Urticaria-Like Follicular Mucinosis
• Urticaria-Like Follicular Mucinosis (ULFM) is a type of alopecia mucinosa with urticaria-like symptoms. It is a very rare condition that is observed in middle-aged men
• The condition can involve any part of the body. Some signs and symptoms of the condition include the appearance of papules and plaques in the skin and itchy skin
• The chief method of diagnosis of Urticaria-Like Follicular Mucinosis is by studying the presenting symptoms, by performing immunofluorescence studies, and a skin biopsy
• Topical, systemic, or intralesional corticosteroids may be used to treat this skin condition, though the effectiveness of treatment is generally limited. In few individuals, exposure to sunlight and antimalarial medications are found to be beneficial
• The prognosis of Urticaria-Like Follicular Mucinosis is generally good with treatment. However, the signs and symptoms may last for several years, even decades in some individuals

Who gets Urticaria-Like Follicular Mucinosis? (Age and Sex Distribution)

• Urticaria-Like Follicular Mucinosis is an extremely rare inflammatory skin condition that may be seen in adults
• It is predominantly noted in middle-aged men, though women may be also affected
• No racial, ethnic, or gender bias has been reported

What are the Risk Factors for Urticaria-Like Follicular Mucinosis? (Predisposing Factors)

• No risk factors are evident for the formation of Urticaria-Like Follicular Mucinosis

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 Urticaria-Like Follicular Mucinosis? (Etiology)

The cause of Urticaria-Like Follicular Mucinosis is unknown.

• It results from an overproduction of mucin by cells in the hair follicle. Mucin is a glycoprotein and a component of mucus
• This causes urticaria-like symptoms of the condition. Urticaria may be described as a type of red skin rash where excess fluid in the skin makes it slightly elevated

What are the Signs and Symptoms of Urticaria-Like Follicular Mucinosis?

The signs and symptoms of Urticaria-Like Follicular Mucinosis may vary from one individual to another. There may be seasonal variation in the signs and symptoms. Some individuals may experience that the symptoms get better during summers.

The signs and symptoms may include:

• Presence of urticaria-like red skin rashes (or weals) on hair-bearing skin
• Fluid accumulation may be observed around the rashes (not blisters)
• Loss of hair at the affected skin regions is not typically noted, even though hair-bearing skin is involved
• In some cases, itchy (pruritic) skin at the site of lesions
• The lesions may be in the form of papules or plaques:
  • A papule is an area of abnormal skin tissue that is less than 1 centimeter around. Usually a papule has distinct borders, and it can appear in a variety of shapes
  • A plaque indicates a broad, raised area on the skin. A skin plaque is usually broader than it is high
• The lesions may form on any part of the body

The signs and symptoms tend to get better and worse over time. They usually last beyond 2 months, or in some cases, for many years.

How is Urticaria-Like Follicular Mucinosis Diagnosed?

The diagnosis of Urticaria-Like Follicular Mucinosis 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
• Immunofluorescence studies on affected skin can be helpful
• Skin biopsy of the affected area: 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 may arrive at a definitive diagnosis

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 Urticaria-Like Follicular Mucinosis?

Complications of Urticaria-Like Follicular Mucinosis may include the following:

• Persistent and chronic skin lesions
• Scarring of skin can occur
• Emotional stress due to cosmetic concerns
• Superimposed bacterial, viral, and fungal infections at the affected skin sites

How is Urticaria-Like Follicular Mucinosis Treated?

Currently, there are no standard treatment measures for Urticaria-Like Follicular Mucinosis, since it is a very uncommon skin condition. The condition is treated on a case-by-case basis. The following treatment options may be considered:

• Waiting to let symptoms resolve on their own, based on the judgement of the healthcare provider
• In some individuals, exposure to sunlight may be helpful
• Use of topical, intralesional (injected just into the active spots), or systemic (oral) corticosteroids
• Use of creams containing nitrogen mustard
• Antibiotic, antiviral, or antifungal medications, to address any superimposed infections
• Topical and systemic photochemotherapy (PUVA), which is a type of ultraviolet radiation therapy
• UVA1 phototherapy: The affected skin is exposed to ultraviolet A light radiation
• Psychological therapy for depression
• Occasionally, the use of antimalarial medications has been found to be beneficial

How can Urticaria-Like Follicular Mucinosis be Prevented?

The exact cause of Urticaria-Like Follicular Mucinosis 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
• Active research is currently being performed to explore the possibilities for treatment and prevention of such conditions

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

What is the Prognosis of Urticaria-Like Follicular Mucinosis? (Outcomes/Resolutions)

• The prognosis of Urticaria-Like Follicular Mucinosis is generally good with appropriate treatment
• A complete resolution of symptoms may take place; although, in some cases, it may take up to 15 years for the symptoms to go away completely
• In many, the symptoms are known to get better during summers (sun exposure is beneficial)

Additional and Relevant Useful Information for Urticaria-Like Follicular Mucinosis:

Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition.