Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . It is considered to be a clinical variant of

lichen planopilaris Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, fla ...

Presentation

There is loss of both

terminal Terminal may refer to: Computing Hardware * Terminal (electronics), a device for joining electrical circuits together * Terminal (telecommunication), a device communicating over a line * Computer terminal, a set of primary input and output dev ...

and

vellus hair Vellus hair is short, thin, light-colored, and barely noticeable hair that develops on most of a human’s body during childhood. Exceptions include the lips, the back of the ear, the palm of the hand, the sole of the foot, some external genital ...

s that occurs in a bandlike pattern on the frontotemporal

scalp The scalp is the anatomical area bordered by the human face at the front, and by the neck at the sides and back. Structure The scalp is usually described as having five layers, which can conveniently be remembered as a mnemonic: * S: The ski ...

. It is a scarring alopecia that has been associated with facial papules, glabellar red dots, a loss of eyebrows, and prominent venous vasculature in the forehead. Facial

hyperpigmentation Hyperpigmentation is the darkening of an area of skin or nails caused by increased melanin. Causes Hyperpigmentation can be caused by sun damage, inflammation, or other skin injuries, including those related to acne vulgaris.James, William; Ber ...

may occur in dark-skinned patients if association with

lichen planus pigmentosus Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, fla ...

is present.

Associations

Frontal fibrosing alopecia has been most often reported in post-menopausal women with higher levels of affluence and a negative smoking history. Autoimmune disease is found in 30% of patients.

Pathogenesis

Although the pathogenesis of frontal fibrosing alopecia is poorly understood, autoimmune reaction and hormonal factors may play a role.Macdonald A, Clark C, Holmes S. Frontal fibrosing alopecia: a review of 60 cases. J Am Acad Dermatol. 2012;67(5):955-61.

Diagnostic

Perifollicular erythema and scarring white patches are seen on dermoscopy. On scalp biopsy, lymphocytic and granulomatous perifolliculitis with eccentric atrophy of follicular epithelia and perifollicular fibrosis are visualized.

Differential diagnosis

Important diagnoses to consider include

female pattern hair loss Pattern hair loss (also known as androgenetic alopecia (AGA)) is a hair loss condition that primarily affects the top and front of the scalp. In male-pattern hair loss (MPHL), the hair loss typically presents itself as either a receding front ha ...

(FPHL), chronic

telogen effluvium Telogen effluvium is a scalp disorder characterized by the thinning or shedding of hair resulting from the early entry of hair in the telogen phase (the resting phase of the hair follicle).Marks, James G; Miller, Jeffery (2006). ''Lookingbill and ...

(CTE), and

alopecia areata Alopecia areata, also known as spot baldness, is a condition in which hair is lost from some or all areas of the body. Often, it results in a few bald spots on the scalp, each about the size of a coin. Psychological stress and illness are pos ...

(AA). FPHL is a non-scarring progressive miniaturization of the hair follicle with one of three different characteristic patterns. CTE is an idiopathic disease causing increased hair shedding and bi-temporal recession, usually in middle aged women. AA is an autoimmune attack of hair follicles that usually causes hair to fall out in small round patches.

Treatment

Improvement or stabilization of the condition has been reported with topical and intralesional corticosteroids, antibiotics,

hydroxychloroquine Hydroxychloroquine, sold under the brand name Plaquenil among others, is a medication used to prevent and treat malaria in areas where malaria remains sensitive to chloroquine. Other uses include treatment of rheumatoid arthritis, lupus, an ...

, topical and oral

immunomodulator Immunotherapy or biological therapy is the treatment of disease by activating or suppressing the immune system. Immunotherapies designed to elicit or amplify an immune response are classified as ''activation immunotherapies,'' while immunotherap ...

tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...

, and most recently,

5α-reductase inhibitor 5α-Reductases, also known as 3-oxo-5α-steroid 4-dehydrogenases, are enzymes involved in steroid metabolism. They participate in three metabolic pathways: bile acid biosynthesis, androgen and estrogen metabolism. There are three isozymes of ...

s. In one study, the use of

antiandrogen Antiandrogens, also known as androgen antagonists or testosterone blockers, are a class of drugs that prevent androgens like testosterone and dihydrotestosterone (DHT) from mediating their biological effects in the body. They act by blocking the ...

s (finasteride or dutasteride) was associated with improvement in 47% and stabilization in 53% of patients Recently, successful treatment of facial papules in patients with frontal fibrosing alopecia was described with oral

isotretinoin Isotretinoin, also known as 13-''cis''-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers ( squamous-cell carcinoma), and in t ...

.Pirmez R, Duque-Estrada B, Barreto T, Quintella DC, Cuzzi T. Successful Treatment of Facial Papules in Frontal Fibrosing Alopecia with Oral Isotretinoin. Skin Appendage Disord 2017;3:111-113. doi=10.1159/000464334

References

External links

{{Medicine, state=collapsed Conditions of the skin appendages