Presentation on theme: "Nail Disorders: Clues to Systemic Disease"— Presentation transcript:
1Nail Disorders: Clues to Systemic Disease Lianne Beck, MDAssistant ProfessorEmory Family MedicineNail Disorders: Clues to Systemic Disease
2Nail AnatomyThe nail plate is the hard keratin cover of the dorsal portion of the distal phalanx. The nail plate is generated by the nail matrix at the proximal portion of the nail bed (Figure 1). As the nail grows, the distal part of the matrix produces the deeper layers of the nail plate, while the proximal portion makes the superficial layers.This production is important, because a disruption of function in the proximal matrix (as may occur in patients with psoriasis) results in more superficial nail problems (e.g., pitting). A disruption of the distal matrix may cause problems with the deeper layers, resulting in ridging or splitting. A transient problem causing growth disturbance may lead to the formation of transverse lines across the nail plate, as in Mees’, Muehrcke’s, and Beau’s linesThe nail is bound proximally by the eponychium (the skin just proximal to the cuticle), laterally by the nail folds, and distally by the distal nail fold (defined by the separation created by the anterior ligament between the distal nail bed and the nail plate;3 different layers:The nail plate (the nail). This is the keratinized structure, which grows throughout life;The nail bed (ventral matrix, sterile matrix). This is the vascular bed that is responsible for nail growth and support. It lies protected between the lunula (the "half moon" seen through the nail) and the hyponychium (the posterior part of the nail bed epithelium); andThe eponychium (cuticle). The epidermal layer between the proximal nail fold and the dorsal aspect of the nail plate.
3Nail Anatomy Fingernails grow 2 -3 mm a month or 0.1 – 0.15 mm a day Toenails grow 1 mm a monthGrowth rate (about 6 months from cuticle to free edge). Time of events can be estimated from location
5Nail Shape and Surface Beau's lines Transverse depression across the nail plateOccurs when growth at the nail root (matrix) is interrupted by trauma OR any severe acute illness e.g. heart attack, measles, pneumonia, or fever.These lines emerge from under the nail folds weeks later, and allow us to estimate when the patient was sick.Transverse depression across the nail plate.• Etiology: Temporary cessation of nailgrowth in the matrix.•Cause:Trauma to the proximal nail fold.Nonspecific finding in systemic disease
6Thin Brittle NailsRepetitive trauma particularly with long nails, repetitive wetting and drying, detergents, harsh solventMetabolic bone disease (nail thinness is correlated with osteopenia)Thyroid disorder, anemiaSystemic amyloidosis (indicated by yellow waxy flaking)Severe malnutritionMore common in womenNails that chip, peel, crack, or break easily - suggest a nutritional deficiency, lacking hydrochloric acid, protein or minerals.Brittle Nails•• Causes: Repetitive trauma particularly with longnails, repetitive wetting and drying, detergents,harsh solvents e.g. nail polish remover, andartificial nails. Check for anemia & thyroidproblems• Not caused by low calcium• Diffusion constant of water of the nail is 100x thatof the skin.• Clinical:Longitudinal & horizontal cracking of thenail plate & splitting of the free distal end(Hang Nail)• Tx: Avoid the causes. Moisturize the nailswith petrolatum 3 to 4x qd & after washing.Keep nails cut short. Biotin 2.5 to 5 mg qd
8Onychorrhexis Presence of longitudinal striations or ridges A sign of advanced age but it can also occur with the following:Rheumatoid arthritisPeripheral vascular diseaseLichen planusDarier's disease (striations are red/white).Central ridges can be caused by iron , folic acid or protein deficiency.
9Central Nail Canal (Median Nail Dystrophy) Associated with severe arterial disease ("Heller's fir tree deformity" -- a central canal with a fir tree appearance -- may occur with peripheral artery diseaseSevere malnutritionRepetitive trauma
10Nail Beading The beads seem to drip down the nail like wax. Associated with endocrine conditions, including the following:Diabetes mellitusThyroid disordersAddison's diseaseVitamin B deficiency
12ClubbingThickening of the soft tissue beneath the proximal nail plate resulting in sponginess of the proximal plate and thickening in that area of the digit.Occurs in patients with neoplastic diseases, particularly those of the lung and pleura.Associated with other pulmonary diseases, including bronchiectasis, lung abscess, empyema, pulmonary fibrosis, and cystic fibrosis.Others: AVM or fistulas, celiac disease, cirrhosis, and inflammatory bowel disease, congenital heart disease and endocarditisChanges in the configuration of the capillaries in the proximal nail bed are responsible for some of the alterations that occur in patients with connective tissue disorders, while abnormalities in the periosteal vessels contribute to clubbing.2Clubbing may result from megakaryocytes and platelet clumps that have escaped filtration in the pulmonary bed and have entered the systemic circulation. Platelets then may release platelet-derived growth factor at the nail bed, causing periosteal changesThe finding of clubbing without an obvious associated disease should prompt a search for bronchogenic carcinoma or another occult reason for the finding.
13Clubbing• The angle of the base of the nail is greater than thenormal 160°.The proximal nail fold has a spongy feel.• Classification: Idiopathic, Congenital, & Acquired• CauseAcquired bilateral: Respiratory disease 80%Cardiovascular extrathoracic 10%Acquired unilateral: Lymphadenitis or a Vascularlesion in the extremity e.g. av fistula• When a single nail is involved consider trauma ora congenital cause
14Nails affected by psoriasis can have small pinpoint pits or large yellowish separations of the nail plate called "oil spots."Pitting
15PittingPunctate depressions in the nail plate caused by defective layering of the superficial nail plate by the proximal nail matrix.Usually associated with psoriasis, affecting 10 to 50 % of patientsAlso caused by systemic diseases, including Reiter’s syndrome and other connective tissue disorders, sarcoidosis, pemphigus, alopecia areata, and incontinentia pigmenti.Any localized dermatitis (atopic or chemical dermatitis) that disrupts orderly growth in proximal nail fold also can cause pitting.
16Oil Spot Sign or Salmon Patch 80% of psoriatic arthritis patients have nailinvolvement.• ClinicalRandom large deep pits on the surface of the nail.Red brown discoloration of the nail bed (oil spotsign, salmon patch)Leukonychia, splinter hemorrhages, subungual hyperkeratosis, and/or onycholysis. May have complete crumbling of nail plate.Nail Psoriasis• Ddx: Tinea unguiumPatients with negative cultures & no skinfindings for psoriasis refer.• Tx: Refer. Topical class 1 steroids,calcipotriol ointment, fluorouracil solution,topical retinoid ( Tazarotene gel) andintralesional steroids, very painful.PittingOil Spot Sign or Salmon Patch
18KoilonychiaTransverse and longitudinal concavity of the nail, resulting in a “spoon-shaped” nail.Normal nail variant in infants, but corrects itself within the first few years of life.Causes by iron deficiency anemia, hemochromatosis, Raynaud’s disease, SLE, trauma, occupational exposure of the hands to petroleum-based solvents, nail-patella syndromeNail-Patella Syndrome: autosomal-dominant condition that includes hypoplastic, easily dislocated patellas, renal and skeletal abnormalities, and glaucoma
20Onycholysis Lifting of the nail plate from the nail bed. Causes: TraumaPsoriasisDrug reactions (tetracycline)Bacterial/fungal/viral infectionContact dermatitis from using nail hardenersThyroid disease (“Plummer’s nails”)Iron deficiency anemiaSyphilisEczemaPorphyria cutanea tardaAmyloidosis, connective tissue disorders
22Nail hypertrophy Causes Onychomycosis Chronic eczema Peripheral vascular diseaseYellow nail syndromePsoriasisNot cutting the nails, traumaSlow nail growth produces thickness
23Onychomycosis • Treatment Terbinafine ( Lamisil) 250mg po qd 6 wks for fingernails and 3mos for toenails. If one or two nails are thick and• Treatmentsymptomatic may surgically or chemically avulse ( 40% urea) Ciclopirox lacquer (Penlac) Topical medthat can be used for patients who cannot take oral meds. Mycologic cure rate 29% to 36%Distal Lateral Subungual Infection• Terbinafine well tolerated. Occasional GI upsets.• Rare hepatotoxicity so cannot be used in ptswith history of liver disease. Check baseline LFTs and repeat in 4 to 6weeks or if clinically indicated.• Drug interactions not as common as azoles.• Cost: Walmart $4 for 30 pills, $10 for 90!Drug Interactions With TerbinafineContraindicated with:Patients hypersensitive to terbinafine or to any other ingredients of the formulationInhibits the CYP2D6 isoenzyme. May affect metabolism of Tricyclics, Serotonin reuptake inhibitors, beta-blockers & monoamine oxidase inhibitors May increase or decrease prothrombin times in patientsconcomitantly taking terbinafine and warfarin Decrease plasma concentration of terbinafine-Terbinafine clearance is increased 100% by rifampinIncrease plasma concentration of terbinafine-Terbinafine clearance is decreased 33% by cimetidineDrug plasma concentration increased by terbinafine-Decreases caffeine clearance by 19%-Decreases theophylline clearance by 14%Drug plasma concentration decreased by terbinafine-Increases clearance of cyclosporine by 15% 40% ureaChemical Avulsion Regrowing New NailOnychomycosis
24Onychomycosis Trichophyton Rubrum most common dermatophyte Toenails more commonly affected than fingernails and often associated with tinea pedisCausesAge, M>F, immunosuppression, HIV, DM, chronic trauma, PVD, hyperhydrosisUncommon in children
25Reasons for Relapse 23% relapse rate w/ terbinafine Old age and slow nail growthImmunosuppressionReinfection from surrounding skinPreventing RelapseAvoid barefeet in public placesKeep feet dryApply antifungal cream to feet weeklyApply antifungal powder or spray to shoes weeklyDiscard old shoes
26Nail atrophyThe nail becomes thin, rudimentary and smaller size congenital or acquired.Causes:Lichen planusEpidermolysis bullosaDarier‘s diseaseVascular disturbancesLeprosyDarier's is characterized by dark crusty patches on the skin, sometimes containing pus. The crusty patches are also known as keratotic papules, keratosis follicularis or dyskeratosis follicularis.
27Pigmentation Abnormalities CategoriesDerm conditions: Psoriasis oil spotSystemic drugs or ingestants: AZT, Minocin, Chemotherapy drugsSystemic Disease: Mees Lines (Leukonychia)Local agents: Cosmetics, physical agents
28Diagnostic PearlsIf the discoloration follows the shape of the lunula and effects multiple nails think of a systemic cause.If the discoloration corresponds to the shape of the proximal nail fold and effects only a few nails think of an external cause.If scraping the nail surface or using acetone removes the color then the cause is a topical substance.
29Abnormalities of the Lunula If the lunula is absent, consider anemia or malnutritionA pyramidal lunula might indicate excessive manicure or traumaA pale blue lunula suggests diabetes mellitusIf the lunula has red discoloration, consider the following causes among others: cardiovascular disease; collagen vascular disease; and hematologic malignancy.
31ArgyriaArgyria occurs in people who ingest or inhale silver in large quantities over a long period (several months to many years).Stan Jones of Montana, a Libertarian candidate for the United States Senate in 2002 and Jones acquired argyria through consumption of a home-made silver product that he made due to fears that the Year 2000 problem would make antibiotics unavailable
36Mee's linesTransverse type of true leukonychia caused by systemic disease.Clinical: Single or multiple transverse lines that involve multiple nails.The pigment is in the nail plate.Causes: Arsenic poisoning, Hodgkin’s disease, CHF, leprosy, malaria, chemotherapy, carbon monoxide poisoning, other systemic insults
37Leukonychia Striae caused by trauma to the matrix from aggressive manicuring, involves only a few nails & does not extend across the entire nail plate.
39Muehrcke's LinesConfined to the nail bed. Will disappear when distal digit is squeezed.Clinical: Double white transverse lines affecting numerous nails.Causes: Chemotherapy and Hypoalbuminemia secondary to nephrotic syndrome, liver disease, or glomerulonephritis.
41Terry's NailsClinical: Proximal white nail with narrow distal pink or brown band (0.5 to 3mm)The nail looks opaque and white, but the nail tip has a dark pink to brown band.Causes: cirrhosis, CHF, DM, cancer, hyperthyroidism, malnutrition, ageing
43Half and Half NailsClinical: Proximal half of nail plate is white & distal half is red brown.Cause: Present in 9 to 15 % of chronic renal failure patients. (Lindsay's nails) — Look for an arc of brownish discoloration.
45Splinter HemorrhagesLongitudinal hemorrhagic streaks involving the nail bed.Causes: Trauma (most common), Derm disease (psoriasis), Idiopathic, and Systemic disease (subacute bacterial endocarditis )Diagnostic PearlsIf multiple nails are involved simultaneously and they occur near the lunula think of systemic disease.If one or a few nails are involved and they occur near the end of the nail plate think of traumaLooks like a splinter underneath the nail, virtually 100% diagnostic of Sub-acute Bacterial Endocarditis (SBE).Occasionally caused by Trichinosis, a parasitic infection caused by eating raw or undercooked Pork.
48Pink or Red Nails Causes: Polycythemia (dark) Systemic lupus erythematosusCarbon monoxide (cherry red)AngiomaMalnutrition
49Brown-Gray Nails Causes: Cardiovascular disease Diabetes mellitus Vitamin B12 deficiencyBreast cancerMalignant melanomaLichen planusSyphilisTopical agents, including hair dyes, solvents for false nails, varnish, and formaldehyde (among many others)
50Yellow Nails Diabetes mellitus Amyloidosis Median/ulnar nerve injury Thermal injuryJaundice
51Yellow Nail SyndromeArrest in nail growth with yellow or green nails, absence of cuticle, thick nail plate. Usually all nails affectedCan indicate internal disorders long before other symptoms appear.Associated with pulmonary disease (COPD, TB, asthma, chronic bronchitis), chronic lymphedema, and thyroid disease, diabetes, liver disorders.Consider yellow nail syndrome if a patient has lymphedema and bronchiectasis
52Green or Black Nails Causes: Topical preparations, including chlorophyll derivations, methyl green, and silver nitrate (among others)Chronic Pseudomonas spp infectionTrauma
54Melanonychia Longitudinal Pigmented Bands (LPB) Cause: Pigmented band appearing in the distal matrix and extending to the tip of the nail.Clinical: Tan, brown or black longitudinal streaks within the nail plate. May be single or multiple bands.Causes:Melanocytic activationNo increase in melanocyte numberResponsible for 73% of single LPB in adults.Melanocytic hyperplasiaIncrease in melanocyte numberResponsible for 77% of single LPB in children
55Melanonychia Common in darkly pigmented persons African Americans: 77% by age 20, 100% by age 50Asian: 10-20%Hispanic, Indian commonUnusual in CaucasiansThumb & index finger most commonly affected
56Causes of Longitudinal Melanonychia Melanocyte activationRacial, pregnancyTrauma, post inflammatory (lichen plannus, pustular psoriasis, onychomycosis, chronic radiodermatitis)Drugs (doxyrubicin, 5-FU, AZT, psoralens)Laugler-Hunziker/Putz Jeger SyndromesAddison’s DiseaseHIV
59Subungual Melanoma Rare in caucasians 1 to 4% of all melanomas African Americans 25%Asians 17 to 23%Peak age 50 to 80 but can occur at any ageRare in childrenLPB is first sign in 38 to 76 % of nail melanoma45-60% on hand40%-55% on footThumb, index finger, great toe most common sites
60Be Suspicious of Melanoma if LPB: Develops in a single digit in adult life especially in 6th decade or laterDevelops abruptly in previously normal nailBecomes suddenly darker or widerPreceding history of digital trauma
61Subungual MelanomaOccurs as a single band in the thumb, index finger or great toe in a dark skinned patientDemonstrates blurred lateral marginOccurs in a patient with a personal history of melanomaOccurs in a patient with a family history of melanomaPresence of partial or complete nail dystrophy
62Modified ABCDEF Rule Age: Peak incidence 5th to 7th decade Band: Brown – black band, Breadth > 3mm, Border irregular/ blurredChange: Rapid change in growth and/or color or lack of change: Failure of nail dystrophy to improve with adequate treatmentDigit involved: Thumb > great toe > index finger, Single > multiple digits, Dominant handExtension: Of pigment to proximal or lateral nail foldFamily or personal history: Previous melanoma or dysplastic nevus syndrome
65Presenting Signs of Subungual Melanoma Mass below nail with partial or complete nail destruction - 50%Periungual infection/ulceration of nail bed/granulation tissue - 33%Discoloration of nail area - 33%Amelanotic - 25%Some begin as LPB: 38% -76%Hutchinson's sign less frequentFrequent delay in diagnosis: Mistaken for traumatic, infected, or inflamed lesion
66Subungual Melanoma Most patients present with late disease 5 year survival %25% nodal spread
67Amelanotic Melanoma 25% of nail melanomas Misdiagnoses Pyogenic granulomaChronic granulation tissueParonychia
70Subungual Hematoma Acute lesions are red and painful Older lesions are purple or black and nontenderDDx: Melanoma is a concern especially in an older patientA pigmented lesion will persist as the nail growsHemorrhage will be replaced proximally by a normal nail plate as the nail growsMelanomas can produce hemorrhageBiopsy if unsure or concernedTx: If painful, decompress an acute lesion
72Mucous or Myxoid CystSmooth flesh colored semi-translucent nodule between the DIP joint & proximal nail foldContains a viscous clear fluidExerts pressure on the matrix resulting in a longitudinal grooveUsually associated with osteoarthritis & may connect with the joint spaceTX: A cyst that does not connect with the joint space, I&D and intralesional steroids or cryotherapyA cyst that connects with joint space may require surgical removal
74Pyogenic GranulomaClinical: Over growth of vascular tissue involving the paronychia. Bleeds easily.Causes: Trauma, infection, ingrown nailTx: Biopsy to rule out amelanotic melanomaTreat underlying cause
75Bowens Disease Squamous Cell Carcinoma in Situ Common in males on the fingernails.Causes: UV light, HPV, chronic infection, trauma, X ray exposure.Clinical: Long standing scaly or wart like growth involving the lateral nail fold. May see onycholysis & nail plate deformity.Biopsy all recalcitrant warts in older patients.Tx: Mohs micrographic surgery.
77Autoimmune and Arthritic Diseases All have nail findings that for the most part are nonspecific.Periungual erythema and telangiectasias are specific & can be an early finding that may help make the diagnosis in difficult cases.
79EczemaCauses: Atopic dermatitis, dyshidrotic eczema, irritant or contact dermatitis e.g. acrylates in sculptured nails.Clinical: Paronychium -Acute or chronic paronychia, Beau's lines, and pits. Hyponychium -Subungual hyperkeratosis onycolysis & finger tip scaling.Tx: Remove cause & medium strength topical steroids.
81Acute ParonychiaInflammation of the nail folds, which appear red, swollen and tender.Non bacterial causes: contact irritant dermatitis, dyshidrotic eczema, & drugs, fungalAcute bacterial paronychia most common infection of the hand. (Staph, strep & pseudomonas)Caused by aggressive manicuring or nail bitingTx: I&D when necessary and culture when possibleAntistaphylococcal antibiotic for 7 to 10 days.For unresponsive cases r/oosteomyelitis and Bx to r/o malignancy.
83Simple Chronic Paronychia Inflammation involving the nail folds lasting > 6 wks.Causes: Aggressive manicuring, chronic exposure to water, contact irritants, & in children finger suckingBakers, bartenders, waitresses, food handlers, maids, mothers with childrenCandida & bacteria are secondarily presentChronic swelling of the nail folds & loss of the cuticleTx: Eliminate cause & minimize water contact. Mid or high potency topical steroids & ciclopirox ( Loprox suspension).
88ReferencesFawcett L, Linford S. Nail Abnormalities: Clues to Systemic Disease. American Family Physician. March 15, 2004.Williams M. Examining the Fingernails When Evaluating Presenting Symptoms in Elderly Patients. Medscape. 11/23/2009.