Tips on Evaluation and Diagnosis of Scarring Alopecias. Melissa Peck Piliang, MD Dermatology and Anatomic Pathology Cleveland Clinic

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Tips on Evaluation and Diagnosis of Scarring Alopecias Melissa Peck Piliang, MD Dermatology and Anatomic Pathology Cleveland Clinic

Disclosures I do not have any relevant relationships with industry Investigator: Samumed, Kythera, Incyte, Concert, Allergan Advisory Board/Consultant: Samumed, Castle Biosciences, Proctor and Gamble

Scarring Alopecias Evaluation Labs Biopsy Additional tests Present diagnostic challenge: May start subtly Well described clinical patterns variations often missed Age/gender/ethnic variations Biopsy location key for accurate diagnosis Mimicker other diagnoses Mask other diagnoses

Cicatricial Alopecia - Overlap FAPD + Pseudopelade of Brocq LPP FFA CCSA Pseudopelade FDS AK CCLE/ DLE + Folliculitis Decalvans Dissecting Cellulitis + Predominantly African Americans FFA = Frontal Fibrosing Alopecia Significant Neutrophilic Inflammation CCSA = Central Centrifugal Scarring Alopecia Significant Inflammatory Interface Alteration and Scarring Alopecic Disorders FDS = Follicular Degeneration Syndrome AK = Acne Keloidalis Sperling,Arch Dermatol, 2000. CCLE/DLE = Chronic Cutaneous Lupus Erythematosus/ Discoid Lupus Erythematosus Modified Sperling, Arch Dermatol 2000

Evaluation

History Thorough Symptoms Pain, pruritus, itch Scale Pustules, oozing Course Rapid onset Slow and steady Prior treatments

Bacterial and Fungal Culture Pustules Scale Pain Drainage Folliculitis Decalvans with staph colonization Tinea Capitis

Laboratory Evaluation General Health CBC CMP Nutritional FERRITIN ZINC VITAMIN D Hormonal TSH Others (as indicated) Autoimmune - lupus ANA AA/LPP/FFA MICROSOMAL AB Androgen Excess DHEAS TESTOSTERONE (FREE & TOTAL) SHBG HgA1C

Dermoscopy White patches and lack of follicular orifices Telangectasias perifollicular Lichen Planopilaris JAAD Dec 2015

Wood s Light Wood s light Courtesy Dr. Wilma Bergfeld White ban frontal hair line

Scalp Biopsy Two punch specimen Vertical Horizontal One punch specimen for DIF P. Foliaceous

Scalp Biopsy Lichen Planopilaris Frontal Fibrosing Alopecia

Scalp Biopsy

CCCA

Folliculitis decalvans (and other inflammatory alopecias)

Frontal Fibrosing Alopecia: Clues

Frontal Fibrosing Alopecia Middle-aged, post-menopausal women Rarely men, but does happen Rarely young women Preferential involvement of vellus and intermediate hair follicles Eyebrows are affected in 50% to 75% Less frequently, eyelashes and hairs in the axilla Body vellus involvement -> Arms, axilla, pubic, legs Manifested almost always as non-inflammatory diffuse hair loss

Difficult Clinical Diagnosis Confused with AGA, Alopecia Areata Subtle scarring Inflammation may be sparse Symptoms mild or absent Loss of eyebrows and/eyelashes = alopecia areata Often younger women or men

Clues for FFA?

Atrophy

Hypopigmentation

Subtle Hypopigmentation Wood s Light

Decrease melanocytes in FFA

Prominent Veins

Kossard S. Arch Derm 1994

Always Lift the Bangs

Young Women

Hypopigmentation

Unusual Distribution

Always Look Behind the Ears

Facial Papules

From: Facial Papules in Frontal Fibrosing Alopecia: Evidence of Vellus Follicle Involvement Arch Dermatol. 2011;147(12):1424-1427. doi:10.1001/archdermatol.2011.321

JAAD Dec 2015

JAAD Dec 2015 Lichen planus pigmentosis

FFA and LPP Happen in African Americans.

J Clin Aesthet Derm 2016; 9(4):45-51

J Clin Aesthet Derm 2016; 9(4):45-51

Rapid onset loss of eyebrows and scalp hair Hypopigmentation

Perifolicular Erythema

Fringe sign

Clues in African Americans It is not insidious Symptoms Rapid loss Perifollicular erythema Subtle atrophy

Scarring Alopecia Happens in Men.

Images courtesy of Dr. Wilma Bergfeld

Images courtesy of Dr. Wilma Bergfeld Facial Papules Dyspigmentation

Mimics A Reminder to Biopsy

Beware of Lichenoid Keratoses Lupus Erythematosus

Superficial and deep infiltrate Perivascular and periadnexal Follicular plugging

Interface dermatitis -Follicular -Interfollicular

Discoid Lupus Erythematosus Mimics AK or SCC clinical and histology Beware or superficial biopsy, recurrent AK/SCC Biopsy technique critical Punch!

Middle aged man Scalp dermatitis and hair loss VERY itchy

Treatment Methotrexate Azathioprine Prednisone Topical steroids Antihistamines

Crusted (Norwegian) Scabies All immunosuppressant medications stopped Treatment: Permethrin -> x2, 1 week apart Ivermectin -> x2, 2 weeks apart

Outcome Pruritus rapidly improve LP Pigmentosa faded Body and facial itch resolved Scalp scale and itch persisted, but mild Alopecia and perifollicular erythema persisted Repeat scalp biopsy -> LPP without scabies

Clinical Infectious Diseases. 54(6):882;2012

When symptoms are out of proportion to findings Gets worse with treatment -> Repeat biopsy!

48 yo with pre diabetes - Hair loss - Scalp pustules - Folliculitis - No pruritus Frontal Hairline Vertex

Tinea Capitis Biopsy and cultures Treatment: griseofulvin Resolved

Summary Clinical Clues Hypopigmentation Prominent veins Loss of eyebrows/eyelashes Density gradient Unusual presentations - Beware Men Young women Parietal scalp/ophiasis -> may spare frontal hair line Mimics Lupus erythematosus Infections

Thank You! pilianm@ccf.org

Always Think About Contact Dermatitis

Biopsy showed: - Spongiotic dermatitis with eosinophils - Impetiginization - Telogen effluvium Culture grew MSSA, negative fungus

Clues: - Cyclical recurrence coincided with hair coloring - Weeping - Extreme pruritus

Central Scalp Hair Loss CCCA or AGA?

CCCA

CCCA Biopsy location important