Phoebe Rich MD Adjunct Professor OHSU Portland, Oregon

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Nail Tips for Diagnosis and Management of Nail Disorders Winter Clinical Dermatology Conference 2017 Hawaii Phoebe Rich MD Adjunct Professor OHSU Portland, Oregon Objectives diagnostic clues for benign and malignant melanonychia The spectrum of clinical presentation in Myxoid cysts No Conflicts of Interest with this Presentation I will not discuss any pharmaceutical products in this presentation. Patients have given photo consent to have their photos used by me in educational talks but not published or reproduced Nail Melanomas 1-3 % of cutaneous melanomas Worse prognosis of 16-87% for 5 year survival Mean Breslow thickness is 4.8mm ref 6 Mean age is 55 Tang, et a; Surgical Science, 2012, 3, 78-83 Subungual Melanoma 1-3 % of cutaneous melanomas are perungual/subungual Worse prognosis 87% 5 year survival Mean Breslow thickness is 4.8mm Unlike cutaneous melanoma in which UV light is a well know risk factor, little to no UV light penetrates nail plate 23-44 % of subungual melanomas have a history of significant injury to that digit, although direct relationship is controversial Tang, et a; Surgical Science, 2012, 3, 78-83 1

Biopsy? Yes or No? What are the clues? Levit EK, Kagen MH, Scher RK, et al. The ABC rule for clinical detection of subungual melanoma. J Am Acad Dermatol 2000;42:269 74. 4 STEP WORKUP Step 1: is the pigment melanin from nail melanocytes? No (fungal, hemorrhage, stain, drugs) Yes Step 2: how many nails involved? Multiple (observation) Only one nail -- Step 3: Can it be explained by other reasons? Yes- psoriasis, trauma, lichen planus, SCC, Onychomatricoma? No --Step 4: Consider age of onset? Child watch unless entire nail is black Adult --- biopsy Piricini, BM. Dermatologic Clinics (2015)Volume: 33, Issue: 2, Publisher: W.B. Saunders, Pages: 185-195 2

Melanonychia: Brown, black, or gray pigmentation of the nail from any cause Non-melanocytic pigment in nail Fungus hemorrhage Stains Melanin from nail matrix melanocytes Sawada, M., Yokota, K., Matsumoto, T., Shibata, S., Yasue, S., Sakakibara, A., Akiyama, M. (2014). Proposed classification of longitudinal melanonychia based on clinical and dermoscopic criteria. Int J Dermatol, 53(5), 581 585. Biopsy? Yes or No? What are the clues? Non-melanocytic melanonychia: Where is the pigment? What is the pigment? Surface of nail plate ( can you scrape it off?) Within the nail plate? Does it clip? Under nail plate ( nail bed ) Melanin vs non-melanin? 3

What is the clue? What is the pigment? Where is the pigment? Is the pigment melanin or blood? 1. Clip back nail and examine the nail bed visually and examine the nail clipping microscopically 2. Dermoscopy of nail plate 4

Melanoma or blood? But Doc, I don t remember any recent trauma to my nail? Where in the nail unit did the trauma occur? How long does it take to grow past the proximal nail fold? Ask about trauma in the past 30-90 days 30-60 days for growth Melanoma or blood? 2-4-6 months 5

Melanoma or blood? Hemorrhage or melanoma? 90 nail lesions Homogenous 92% Globules 42% Streaks 34% Blood in nail as a cause of pigmentation Blood in the nail results in dark pigmentation in nail clinically similar in appearance to melanoma. Dermoscopy can help 6

What is the clue? Melanonychia: Brown, black, or gray pigmentation of the nail from any cause Non-melanocytic pigment in nail Fungus hemorrhage Stains Melanin from nail matrix melanocytes Melanin from nail matrix melanocytes Activation of dormant melanocytes ( produce melanin) Benign : racial, drug, frictional, hormones, pregnancy Proliferation of melanocytes to produce melanin, and divide multiply Nevus, Lentigo, melanoma Di Chiacchio, N., Ruben, B. S., & Loureiro, W. R. (2013). Longitudinal melanonychias. Clinics in Dermatology, 31(5), 594 601. http://doi.org/10.1016/j.clindermatol.2013.06.007 Dermoscopy of Pigmented Bands in nails Can provide helpful clues about whether nail biopsy is necessary Not a substitute for nail matrix biopsy histopathology is the gold standard. Luc Thomas, Stéphane Dalle Dermoscopy provides useful information for the management of melanonychia striata. Dermatologic Therapy 2007 7

Dermoscopy of pigmented bands in nails Two Steps What is the background color of the band ( brown, black or gray) What are the characteristics of the lines within the band? ( Uniform or variable size, width color?) Background: Gray +/- Brown/black? Melanocyte activation-- gray melanocyte proliferation-- brown /black,+/-gray Background color of pigmented bands Melanocyte activation ( racial, hormonal, drug, etc) benign Gray color only = Good usually Melanocyte proliferation ( nevus, lentigo, melanoma) Brown, Black +/-gray = Bad possibly Step 2 : Arrangement of lines within the pigmented bands Step 1: background Gray or Brown/Black? Step 2: lines within the band : regular or irregular? Longitudinal lines with regular thickness, spacing and color and parallelism suggest benign lesion (nevus or lentigo) Longitudinal lines of irregular thickness, spacing and color are associated with melanoma. 8

Dermoscopy of pigmented bands in nails Can provide helpful clues about whether biopsy is necessary Not a substitute for biopsy histopathology is the gold standard. Frictional Drug Gray Melanoma brown background irregular lines Melanoma brown background with irregular lines Melanoma? 9

Other sites of pigment in the Nail Unit Distal nail plate end-on exam shows where pigment is in the nail plate Proximal matrix superior ( dorsal ) nail plate Distal matrix inferior ( ventral ) nail plate Nail folds and hyponychium pigmentation Hutchinson s Melanoma Dermoscopy helpful but don t forget history and ABCDEs of nail melanoma Dermoscopy of pigmented bands in nails Can provide helpful clues about whether biopsy is necessary Not a substitute for nail matrix biopsy histopathology is the gold standard. 10

Punch not obsolete. Try to remove the origin of band. 11

Summary Melanonychia Melanin from matrix melanocytes 1. Activation of melanocytes - no proliferation Racial, drug, frictional, systemic, other local nail disease ie, Bowens, LP (Adults= Pediatric) 2 Benign proliferation Lentigo, nevus (Children> Adults) 3 Malignant proliferation Melanoma, MIS (Adults> Children) Nail Melanomas 1-3 % of cutaneous melanomas Worse prognosis of 16-87 5 year survival Mean Breslow thickness is 4.8mm ref 6 Mean age is 55 Tang, et a; Surgical Science, 2012, 3, 78-83 Must diagnose Nail Melanoma Early 1-3 % of cutaneous melanomas are periungual/subungual Worse prognosis 87% 5 year survival Mean Breslow thickness is 4.8mm at diagnosis Tang, et a; Surgical Science, 2012, 3, 78-83 Nail Questions? PhoebeRich@aol.com 12

Case 1 asymptomatic longitudinal groove in nail. What is causing it? Pink bulge over the lunula. What is causing it? Myxoid cyst UNDER the lunula ( matrix) 13

Myxoid cyst. What determines the clinical appearance? Above matrix -Groove Location, Location, Location! Below matrix pink Bulge Within close proximity to the nail matrix 14

Digital Myxoid Pseudocyt Make the diagnosis with certainty Puncture, image or biopsy Harmless so no treatment is necessary unless patientt has pain or limitations DMC Digital myxoid pseudocyst Benign ganglion cysts on digits Usually around DIP or in proximal nail fold More common on fingers than toes May be associated with arthritis of the DIP but not mandatory Usually not symptomatic but may sometimes spontaneously drain Lin YC 1, Wu YH, Scher RKNail changes and association of osteoarthritis in digital myxoid cyst. Dermatol Surg. 2008 Mar;34. Dermoscopy of Myxoid pseudocysts 2 varieties 1. myxomatous type. Focal collection of mucin in the dermis without a lining 2. A ganglion cyst type with patent with a pedicle (channel) to the joint ( DIP) DMC Yes? No? 15

DMC Yes? No? DMC Yes? No? DMC Yes? No? DMC Yes? No? DMC Yes? No? Giant DMC cell Yes? tumor of No? the tendon sheath 16

DMC Yes? No? DMC Yes? No? Above matrix -Groove Within close proximity to the nail matrix Below matrix Bulge Myxoid cysts Prove the diagnosis Clincial assessment or pierce with needle to see the myxoid material Reassure that it is not harmful Treat if patient's activity is limited or there is pain. If conservative tx not helpful, refer Treatment Treat if myxoid cyst is limiting function Options Drain, pressure, splint Liquid nitrogen Inject with kenalog 5 mg/cc +/- polidocanol sclerotherapy 3% 0.2-.5 ml Dye in joint and ligate de Berker D 1, Lawrence C. Arch Dermatol. 2001 May;137(5):607-10 Full excision of lesion with scaring of channel Nail Questions? PhoebeRich@aol.com 17