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Inflammatory Diseases of the Nail

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Inflammatory Diseases of the Nail Lever Chapter 19: Inflammatory diseases of the nail Dr. Jessica Spies, dermatopathologist CTA Pathology Portland, Oregon Inflammatory processes of nail ● Large number of skin diseases can also affect the nails ● Most of clinical changes are not specific to the disease ● Nail unit bx rarely the way to diagnosis but indicated in rare instances (e.g. nail unit lichen planus) ● Today’s lecture will NOT be exhaustive list of all diseases that affect the nails ● It will also NOT be an exhaustive list of all the myriad clinical findings in the diseases I do mention ● Rather, I will follow the Lever chapter and what it’s editors thought it pertinent to cover ● You will likely have very few nail pathology questions on your boards and those that will be there should be classic examples in nail pathology Nail pathology--a few caveats ● Nail unit sampling technique is an important component of nail disease diagnosis ● The hard keratin of the nail plate complicates the biopsy procedure and the specimen submission and processing of the biopsy ○ Biopsy often misembedded/partially destroyed in histology labs unused to processing nail specimens ○ At CTA, we are lucky enough to work closely with Dr. Phoebe Rich, a dermatologist who specializes in nail disease so we get a large number of nail bxs and have specially trained technicians in our lab to handle them ● These limitations can affect the quality of the sections available for interpretation by the pathologist The nail apparatus Nail unit histology Inflammatory processes of nail ● Inflammation mainly affects matrix, nail bed, hyponychium and nail fold ● Nail plate changes = mostly secondary ○ matrix diseases can lead to irreversible damage of nail plate ○ nail bed and hyponychium processes that do not affect plate formation can still affect nail plate shape and/or adhesiveness ■ Nail bed injury causes switch from onycholemmal keratinization to epidermoid keratinization (“metaplasia”) ■ Products of epidermoid keratinization build up between bed and plate, causing plate shape change and/or shedding (e.g. psoriasis and onychomycosis) ● Nail fold involvement ○ Ventral surface of proximal nail fold inflammation can lead to cuticle inflammation and changes in dorsal nail plate Eczematous dermatitis ● Atopic dermatitis most common ● Contact dermatitis can affect all of nail unit areas ● Nail plate changes usually from matrix or proximal nail fold inflammation ● Onycholysis may be due to involvement of distal hyponychium which spreads proximally to nail bed ● Secondary paronychial infection can occur ● Biopsies should be focused on areas of involvement ● Histology (don’t need nail plate/bed bx, just adjacent involved skin): ○ Spongiosis, epidermal acanthosis, negative PAS for fungus Eczema: spongiotic dermatitis Dyshidrotic eczema spongiosis Eczematous dermatitis Psoriasis of the nail unit ● Occurs in up to 50% of psx patients, 80% with psoriatic arthritis ● May involve any part of nail unit ● Best to biopsy through nail plate and nail bed if you do biopsy it ● Must rule out fungal infection with PAS stain (which may have same histologic features) ○ Proximal nail matrix involvement: pitting, roughening ■ Histology: parakeratosis on outgoing plate surface, sheds and leaves “pits” ○ Mid and distal matrix involvement: leukonychia ■ Histology: parakeratosis in body of plate Psoriasis of nail cont’d ○ Nail bed involvement ■ “Onycholysis” ● Separation of nail bed and nail plate; white-yellow discoloration ■ “oil drop” or “salmon patch” sign ● Yellow red discoloration proximal to onycholysis ■ Histology: collections of neutrophils in the stratum corneum ● LSS14B in Philips Psoriasis of nail: pits, oil drop sign (salmon patch) and onycholysis Lichen planus of the nail ● Incidence 1%-10% in patient with disseminated LP ● May develop in absence of cutaneous LP ● fingernails>toenails ● Proximal nail matrix involved: ○ Longitudinal grooves and ridges “onychorrhexis” ○ Histology (just like cutaneous LP): ■ Hyperkeratosis, hypergranulosis, vacuolar degeneration, necrotic keratinocytes at DEJ ■ Band like infiltrate of lymphocytes and histiocytes, occ. Melanophages ○ +/-hyperpigmentation ○ May be reversible if treated early before scar formation ● Diffuse nail matrix involvement: ○ Onychoschezia (lamellar changes, fragile, brittle) ○ Late stage pterygium (proximal nail fold and bed fused together by scar) Lichen planus of nail ● Nail bed only involved: ○ Proximal onycholysis with otherwise normal nail plate ■ Different from distal onycholysis of psoriasis ● Papular lesions of LP in nail bed ○ Focal atrophy leads to “spooned nails” i.e. koilonychia ● Diffuse nail bed involvement may lead to shedding whole nail plate LP of nail: Longitudinal grooves and ridges (proximal matrix involvement) “onychorrhexis” LP of nail, late stage Pterygium (scar) LP of nail histology is similar to cutaneous LP (may be more muted) Lichenoid reaction pattern Jagged/sawtooth rete, colloid/civatte bodies Connective tissue diseases of nail ● Nail unit affected by most CTDz ● Microvasculature primarily affected ○ Dilated capillary loops ○ Hemorrhage ○ Avascularity ● PAS-D positive material in keratin of cuticle (per Lever) ● Identical patterns may be found in Raynaud phenomenon--not known if nail unit bx distinguishes Lupus of nail bed ● Discoid lupus can occur in nail but never localized to it, so nail bx rarely needed ○ Nail dystrophy and longitudinal ridging ● Hypertrophic LE can cause hyperkeratosis in nail bed ○ Longitudinal ridging ● Systemic LE--wide range of nail abnormalities not specific to disease ● Similar histology as cutaneous lupus ○ Interface dermatitis ○ Squamotization of basalis ○ Lymphocytic infiltrate, occasional melanophages ○ LSS14-I in Philips Bullous disease of nail: histology similar to cutaneous disease ● Darier-White disease ○ Most often occur in association with other clinical findings ○ Histology: intraepidermal acantholysis with dyskeratosis ○ Nail matrix involvement: white streak in distal lunula ○ Nail bed and hyponychium: red and white longitudinal streaks ● Pemphigus vulgaris (and foliaceous) ○ Uncommonly involves nails, usually only in severe disease ○ Proximal and lateral nail fold involvement leads to chronic paronychia ○ Histology: suprabasilar acantholysis, + DIF with intercellular IgG and C3 deposition ○ Matrix involvement: onychomadesis (proximal separation of nail plate) ● Other bullous diseases reported to involve nail unit: pemphigoid, EM/TEN, EBA Darier’s disease: red and white longitudinal streaks, V shaped notching Darier’s disease: intraepidermal acantholysis with dyskeratosis (corps rond, grains) Pemphigus vulgaris: paronychia and onychomadesis (prox nail plate separation) Pemphigus: intraepidermal acantholysis with DIF: intercellular IgG and C3 tombstones Nail infections: onychomycosis ● Dermatophytes most common with 2 species causing >60% of infections ○ Trichophyton rubrum ○ Trichophyton mentagrophytes ● Molds ○ Acremonium sp. ○ Fusarium sp. ○ Scopulariopsis sp. ● Yeasts ○ Mainly Candida species, esp Candida parapsilosis ● Toenail infections ○ 90% either trichophyton sp., microsporum sp., epidermophyton sp. ● Fingernail infections ○ Up to ⅓ due to yeast Nail infections-- 4 main types of onychomycosis ● Distal subungual (most common) ■ Usually T. Rubrum ● First Involves hyponychium and lateral folds → yellowing, onycholysis, eventual subungual hyperkeratosis ● Proximal white subungual ■ Rare in general population; common in HIV pts ■ Also T. rubrum ● Superficial white ■ T. mentagrophytes ■ In HIV, T. rubrum ● Candidal : ○ Common cause chronic paronychia ○ May involve plate and bed in chronic mucocandidiasis and HIV pts, pediatric HIV pts DISTAL SUBUNGUAL ONYCHOMYCOSIS Proximal white subungual onychomycosis Superficial white onychomycosis Candidal onychomycosis and paronychia Onychomycosis diagnosis ● Biopsy with PAS staining is most sensitive (99%), highest negative predictive value ○ Make sure to sample subungual debris--by far the highest yield!! ○ Will highlight dermatophyte, yeast and mold but cannot distinguish between these organisms ● KOH with chlorazole black on scrapings in clinic most cost effective test (94% sensitivity) ● Culture much less sensitive than PAS or KOH but will ID organism, which PAS/KOH cannot!! ● Newest technology to ID organism: PCR ○ Currently the most accurate method for identifying infectious agent in nails, far superior to culture and highly concordant with PAS stain ○ we now have at CTA Lab, can be done as reflex test on PAS positive nails ● Histology: LSS14D in Philips (dermatophyte) Onychomycosis due to mold ● Healthy or immunocompromised pts ● Can coinfect with dermatophytes (skin too!) ● Often + paronychia ● Cannot be distinguished on PAS and KOH from dermatophytes ● May be thought to be contaminant and left out of culture report ** ● Our PCR will report all but Aspergillus (too ubiquitous) ● Difficult to treat, think about in tx resistant onychomycosis PCR for onychomycosis Cue to PDF Bacterial infection of nails ● Nail folds with chronic paronychia ○ Staph aureus ○ Pseudomonas aeruginosa ○ Coinfection with Candida ● “Green nails” ○ Pseudomonas colonizing onycholytic/damaged nail plates ○ Pyocyanin pigment made by bacteria ○ Likes wet environments (same etiology in swimmer’s ear, hot tub folliculitis) ■ Under artificial nails (gaps between natural and artificial nail) ■ Prolonged exposure to water or soaps ■ Periungual trauma Pseudomonas nails.
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