Presentation
We present a 67-year-old woman with frontal fibrosing alopecia whose daughter was diagnosed to have lichen planopilaris. [ncbi.nlm.nih.gov]
No clinically visible inflammation is noted in most presentations, although histologic inflammation may be present. The most common nonscarring alopecias include alopecia areata and telogen effluvium. [emedicine.medscape.com]
Skin
- Facial Papule
Successful Treatment of Facial Papules in Frontal Fibrosing Alopecia with Oral Isotretinoin. Skin Appendage Disord 2017;3:111-113. doi=10.1159/000464334 External links [ edit ] [en.wikipedia.org]
CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. [ncbi.nlm.nih.gov]
More recently, peculiar facial papules have been reported in FFA representing facial vellus hair involvement. [ijtrichology.com]
Patients with severe forms of frontal fibrosing alopecia had eyelash loss, facial papules and body hair involvement that was stabilized or improved with antiandrogen treatment, according to recent study results. [healio.com]
- Loss of Pubic Hair
loss involving pubic hair, axillary hair, and the eyebrows. [ijdvl.com]
Urogenital
- Incontinence
Histologic evaluation of pigmented macules sometimes exhibited an increased epidermal pigmentation and on occasions, pigmentary incontinence. LIMITATIONS: More patients are needed to determine the prevalence of these lesions in FFA. [ncbi.nlm.nih.gov]
Workup
Other Pathologies
- Lymphocytic Infiltrate
Histopathological examination showed decrease in the number of hair follicles, perifollicular lamellar fibrosis, chronic lymphocytic infiltrate surrounding the infundibular and the isthmus portions of the hair follicles. [revistasrd.ro]
A skin biopsy specimenshowed a perivascular, lymphocytic infiltrate withperiinfundibular fibrosis. These findings establisheda diagnosis of frontal fibrosing alopecia. [ncbi.nlm.nih.gov]
Figure 4: (a) Fibrous tracts replacing ancient hair follicles Some fibrotic tissue can be observed around them admixed with a sparse lymphocytic infiltrate (H and E, x40) (b) Lymphocytic infiltrate localized around the upper portion of the hair follicle [ijdvl.com]
Histopathologically, perifollicular lymphocytic infiltrate and follicular hyperkeratosis are observed at the edge of the affected area, while the rest of the lesion appears pale with loss of the follicular ostia. [orpha.net]
HLA Type
- HLA-DR1
Other associations include HLA-DR1, which seems to be correlated with peptide anchorage and presentation of specific T helper lymphocytes. [14] HLA testing revealed HLA DRB1*(04,13), DQB1*(03:02,06) in both patients, supporting the idea that these diseases [ijdvl.com]
- HLA-B7
Familial lichen planus is uncommon, the prevalence has been reported to be 1.5% [11] and 28 pedigrees have been reported. [12] Copeman et al. [13] found an association of HLA-B7 in familial cases but not in sporadic cases. [ijdvl.com]
Treatment
Currently, no evidence-based treatment is available for FFA. Our aim was to determine the effectiveness of available treatment options for FFA, and to identify promising treatment options for future studies. [ncbi.nlm.nih.gov]
What treatments are available? A number of different treatments have been tried with varying success. There are no large randomized controlled trials (gold standard) to establish whether they are indeed effective. [londonhairspecialists.com]
Prognosis
Pattern III patients have the best prognosis, while pattern II patients have the worst prognosis. © 2017 European Academy of Dermatology and Venereology. [ncbi.nlm.nih.gov]
We will also discuss up-to-date treatment options, ways of monitoring disease activity and prognosis. Click here to download the programme and register to attend Dermfest 2017 [thesynapse.eu]
Prognosis FFA is very stubborn to treat and can progress for years. Once the hair follicles are destroyed, the hair does not grow back and needs either stem cells or hair transplant surgery to provide new growth of hair. [houstonhairlossclinic.com]
Frontal fibrosing alopecia: clinical presentations and prognosis. British Journal of Dermatology. 2009; 160:75-79. http://www.ncbi.nlm.nih.gov/pubmed/18811690. [rarediseases.info.nih.gov]
Key learning points for healthcare professionals include prognosis and the psychological impact of the condition on patients. [mimslearning.co.uk]
Etiology
Etiology It was suggested that the disease could have a hormonal origin, but to date the precise etiology remains unknown. The documents contained in this web site are presented for information purposes only. [orpha.net]
Its etiology remains unknown, and there is no definitive treatment. Information in familial cases of FFA is scarce. [ncbi.nlm.nih.gov]
This has led some to suggest an androgen-driven cause hypothesis, but currently, the etiology is unknown. As the name suggests, it is a scarring alopecia. [journals.lww.com]
Epidemiology
Summary Epidemiology Prevalence is unknown. It most commonly affects postmenopausal women, although it has also been reported in men and premenopausal women. [orpha.net]
Moreover, there is a relative paucity of epidemiological, interventional and basic research studies, and there have been no advances in translational therapeutics, unlike for other inflammatory dermatoses, such as alopecia areata (AA). [ncbi.nlm.nih.gov]
Objective We sought to describe the epidemiology, comorbidities, clinical presentation, diagnostic findings, and therapeutic choices in a large series of patients with FFA. [scinapse.io]
Pathophysiology
Not only can the technology assist in monitoring disease activity in a non-invasive manner, but it may elucidate new pathophysiologic findings. © 2017 European Academy of Dermatology and Venereology. [ncbi.nlm.nih.gov]
Its pathophysiology is unknown. The course is one of spontaneous stabilisation over a number of years. [therapeutique-dermatologique.org]
Other variants are classic LPP and Graham-Little-Piccardi-Lasseur syndrome. 1 The exact pathophysiology of FFA is unknown. [racgp.org.au]
Prevention
However, topical applications can help prevent the condition from progressing. [philipkingsley.co.uk]
How can it be prevented? At this time there is not a method of the prevention of FFA. [houstonhairlossclinic.com]
It is an option for those that couldn’t find an effective solution otherwise, didn’t take preventative measures, or didn’t take action soon enough. And a hair transplant isn’t a solution for everyone. [hishairclinic.com]
These can help to dampen down the immune system and prevent the inflammation around the hair follicles. In some people it can slow or halt hair loss. Antiandrogen treatments e.g. oral finasteride and dutasteride. [bad.org.uk]
Therefore, our aim should be to prevent disease progression. [racgp.org.au]