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What Is Alopecia Mucinosa?

Primary alopecia mucinosa is mostly found in children.

Alopecia mucinosa is a rare condition. It is characterized by an abnormal accumulation of mucin in hair follicles and sebaceous glands, and may lead to diffuse or patchy alopecia areata and/or cicatricial alopecia. Occurring in both children and adults, it can cause skin lesions and hair loss.(32, 182, 183)

Regardless of age, those suffering from alopecia mucinosa have an increased risk of lymphoma. Experts recommend regular check-ups to detect cancer. This includes periodic biopsies, especially if the condition doesn't improve or worsens.(23)

Types of Alopecia Mucinosa

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Traditionally, there are considered 3 separate types of the disease:(32, 182, 183)

TYPE CHARACTERISTICS
Primary Acute Alopecia Mucinosa
  • Occurs mostly in children; small percentage under age 40.
  • Most lesions resolve in up to two years.
  • Categorized as benign and of uncertain cause (idiopathic).
Primary Chronic Alopecia Mucinosa
  • Occurs in people older than 40; primarily over age 50.
  • More lesions than in the acute disorder.
  • Can persist or recur.
  • Categorized as benign and of uncertain cause (idiopathic).
Secondary Alopecia Mucinosa
  • Usually occurs between the ages of 40 and 70.
  • Numerous widespread lesions.
  • Mostly occurs in middle-aged men over age 50.
  • Associated with benign inflammatory conditions such as lupus erythematosus as well as certain types of cancer (such as Hodgkin's lymphoma, mycosis fungoides, and Kaposi sarcoma).

However, this strict categorization is questionable since between 9% and 64% (15-40% by other estimates) of all types potentially develop lymphoma, which can precede, follow, or occur at the same time as alopecia mucinosa. Further, it is impossible to predict which cases will progress to malignancy.(23, 182)

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Symptoms of Alopecia Mucinosa

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Clinical symptoms may include any or all of the following:(23)

  • Clear mucinous fluid in the openings of the hair follicles.
  • Groups of or diffusely distributed follicular papules.
  • Hair loss that can resemble many different types, including patchy or diffuse thinning alopecia areata, alopecia associated with tinea capitis, telogen effluvium or even lichen planus types of cicatricial (scarring) alopecias.
  • Hardened scaly plaques (flesh-colored or reddened).
  • Intense itching.

Evidence indicates that it is an autoimmune disorder where triggered immune system cells stimulate follicle keratinocyte cells to produce the destructive mucin. Studies suggest that use of certain medications (e.g., adalimumab and imatinib) and Staphylococcus aureus infection are possible triggers for this immune reaction.(23, 182)

Treatments for Alopecia Mucinosa

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Although there is no treatment standard, a variety of treatments have shown some effectiveness:(182)

  • Radiation therapy
  • Systemic drugs (dapsone, Indomethacin, and interferons)
  • Topical and systemic psoralens plus ultraviolet A light (PUVA) therapy
  • Topical nitrogen mustard
  • Topical, intralesional, and systemic corticosteroids
Hair Regrowth is Possible

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Also known as Pinkus' follicular mucinosis or mucinosis follicularis.(183)
Mucinous material.(182)
Particularly mycosis fungoides, the most common type of cutaneous T-cell lymphoma.(23)
Small rounded bumps without pus.(76)