Cicatricial alopecia — SAQ

Stem

A 58-year-old postmenopausal woman presents with a 2-year history of progressive recession of the frontal and temporal hairline. She has also lost most of her eyebrows. On examination the frontal hairline has receded by about 3 cm, the bald skin is smooth and shiny with no visible follicular openings, and there is a single terminal hair at the advancing edge. The active margin shows white perifollicular scale and mild erythema.

Questions

a) What is the most likely diagnosis, and what clinical features support it? (2 marks)

^[4][6]b) What investigations would you arrange to confirm the diagnosis and classify the disease? (3 marks)

c) Outline a stepwise management plan for this patient. (3 marks)

d) What are the key complications and prognostic factors? (2 marks)

Model answers

a) Diagnosis and supporting features (2 marks)

^[4]- Frontal fibrosing alopecia (FFA), a lymphocytic cicatricial (scarring) alopecia and clinical variant of lichen planopilaris.

• Supporting features: progressive frontal-temporal hairline recession in a postmenopausal woman; smooth, shiny skin with loss of follicular ostia; lonely hair sign (single terminal hair at the advancing edge); perifollicular hyperkeratosis and erythema at the active margin; and eyebrow loss.

^[3]b) Investigations (3 marks)

• Trichoscopy: loss of follicular openings, perifollicular scale and erythema at the active margin, and lonely hair sign. This is the first-line, non-invasive test.
• 4 mm punch scalp biopsy from the active inflammatory margin (not the scarred centre) for histology: lichenoid/perifollicular lymphocytic infiltrate at the follicular isthmus, loss of sebaceous glands, and fibrous tract remnants.
• Autoimmune screen: ANA and anti-dsDNA if discoid lupus is a possibility; baseline FBC, renal function and urinalysis if systemic therapy is planned.

c) Management (3 marks)

• Counsel that the goal is to halt progression and preserve remaining hair; regrowth in scarred areas is not possible.
• Topical treatment: potent topical corticosteroid to the active margin and/or intralesional triamcinolone acetonide at intervals — see the local protocol for the exact preparation, concentration and interval.^[3]
• Systemic first-line: hydroxychloroquine, weight-based with baseline and annual eye screening — see the local protocol for the exact daily dose.^[3]
• Add-on options: a 5-alpha-reductase inhibitor because of FFA pathophysiology; doxycycline for anti-inflammatory effects; or mycophenolate mofetil for refractory disease — see the local protocol for agents and doses.^[3]
• Adjuncts: topical minoxidil to preserve remaining hairs, camouflage/wig if needed, and strict sun protection at the hairline.^[3]

^[2]d) Complications and prognostic factors (2 marks)

^[1]- Complications: permanent frontal hair loss with major cosmetic and psychosocial impact; loss of eyebrows; ocular irritation if eyelashes are involved; and failure to recognise the condition leads to progressive, irreversible destruction.

• Good prognosis: early diagnosis, limited disease, prompt anti-inflammatory therapy, good adherence and avoidance of traction/sun exposure.
• Poor prognosis: late presentation, extensive scarring, rapid progression despite therapy, and continued exposure to triggers.