Refer to: Shearn MA: Nails and systemic disease (Medical Infor-mation). West J Med 129:358-363. Oct 1978

Medical Information

Nails and Systemic DiseaseMARTIN A. SHEARN, MDOakland, California

Important diagnostic clues may be providedby changes in the nails. Because nail findingsare easily observable and yield valuable in-formation, attention to these features is oftenrewarding. Some of the nail changes that maybe observed in systemic disorders are re-viewed.

MANY SYSTEMIC DISORDERS leave their mark onthe nails and, therefore, provide physicians withvaluable clues to diagnosis. In spite of this knowl-edge, there has been relatively little attentionpaid to the nails, which is surprising in view ofthe fact that they are among the most accessibleparts of the body and can be viewed with thenaked eye, requiring no sophisticated equipment.Although similar findings occur on the fingernailsand the toenails, the traumatic effects of shoesand more frequent mycotic infections make toe-nails less valuable for diagnosis.Some of the more distinctive changes in the

fingernails that have been observed in systemicdisease are herein reviewed. Local disorders re-

sulting from trauma, tumor and infection arewell described in dermatologic literature and arenot included here.

Nail Growth and AgingNails grow from the nail matrix, which is lo-

cated approximately 3 to 4 mm proximal to thecuticle. The matrix and the nail bed are suppliedwith blood through the paired digital arteries,

Dr. Shearn is Director of Medical Education, Kaiser-Perma-nente Medical Center, Oakland, and Clinical Professor of Medicine,University of California, San Francisco.Submitted June 2, 1978.Reprint requests to: Martin A. Shearn, MD, Kaiser-Permanente

Medical Center, 280 W. MacArthur Blvd, Oakland, CA 94611.

which provide a rich capillary loop system withmany arteriovenous anastomoses. Nails grow ap-proximately 0.1 mm per day, with more rapidgrowth in adolescence and a slower rate withaging. The dominant thumbnail tends to growfaster than the nondominant nail, and disease mayslow nail growth. Pregnancy and hyperthyroidismare associated with increased rates of growth;starvation and lactation, with slower growth.'The most detailed study of nail growth was re-

ported by Bean.2-4 In a remarkable effort span-ning three decades, he observed the gradual slow-ing of his nail growth. In his early 30's the thumb-nail grew from the cuticle to the free edge in 117days; in his 50's the nail grew the same distancein 137 days. At the completion of his study tenyears later, 144 days were required for the samenail growth. Seasonal changes in nail growthrate were not observed.

With aging, nails become ridged, more opaque,greyish in color and less flexible (Figure 1).

Longitudinal ridging appears in the early mid-dle years, increasing with age, and may beaggravated by trauma, rheumatoid arthritis andperipheral vascular disease, among other condi-tions.

HandednessThe thumbnail may provide a clue to dominant

handedness (Figure 2), a feature that might beof value in predicting the outlook of a comatosepatient with a neurologic deficit. The dominantthumbnail has a wider base and the angles formed

Figure 1.--Longitudinal ridging of nail in normal aging.

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by the base and the lateral aspects of the nail aremore obtuse. When a trained observer judgedhandedness in a blind fashion (when fingernailswere draped, and crossed or uncrossed accordingto a coin toss), handedness was identified cor-rectly in the great majority of the subjects.5

LunulaeThe lunula or half-moon is present in most

normal people. Lunulae are almost always foundin the thumbnails, even when absent in the othernails. However, approximately 5 percent of pre-sumably normal persons, usually older ones, donot have visible lunulae. A number of illnessesalso may cause the loss of the lunulae: theseinclude trauma to the terminal digit, multiplemyeloma, scleroderma (Figure 3), bone tumorsand hyperparathyroidism.

Figure 2.-Thumbs of left-handed person uncrossed.Note wider base and more obtuse angles at the cornersof the dominant thumbnail.

DystrophyDystrophy of the nails results from a variety

of conditions-hereditary, congenital and ac-quired. Dystrophic nails are seen in persons whoconstantly traumatize their nails by biting orpicking. Dystrophy may result from faulty mani-curing. Such nails may present with irregular,transverse lines. Dystrophy of nails may also besecondary to dermatologic conditions which affectthe terminal digits.

Punctate leukonychia, or small white spots onthe nails resulting from incomplete keratiniza-tion of the nail plate, is found at some time invirtually all people and has no clinical impor-tance.The partial or complete absence of the nails

that occurs in the nail-patella syndrome serves toalert one to the renal failure associated with thiscondition.

Mees Nail; Beau NailMees nail (Figure 4) is characterized by a

single, transverse, narrow whitish line that runsthe width of the nail plate and is seen on mul-tiple nails. The condition was originally describedin arsenic poisoning but appears to be a legacyof remote, serious medical disorders, such assepticemia, dissection of the aorta, acute renalfailure, sickle cell anemia in crisis and poisoningwith heavy metals. The approximate number ofdays since the serious event occurred can be deter-mined by measuring from the cuticle to the trans-verse line, adding 3 to 4 mm for that portion ofthe nail which is hidden between the matrix andthe cuticle, then dividing by the estimated averagedaily nail growth of approximately 0.1 mm.

Figure 3.-A, Thumbnail of patient with scieroderma shows dystrophy and loss of lunula. B. Radiograph of handsof same patient shows bony abnormalities involving distal phalanges.

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Beau nail, which displays a fault line appear-ance, is probably a more serious form of Meesnail that results from temporary arrest of growthat the nail matrix. In Beau nail, transverse deepdepressions in the nail are seen, rather than thetransverse white lines of Mees nail. The Beauline may indicate the same prior, serious medicalevents; the measurement is identical to that forMees nail. Beau nail, like Mees nail, is seen atthe cuticle edge approximately one month afterthe serious illness has occurred, as the averagenail grows approximately 3 mm per month, thedistance represented by the hidden area of nailgrowth.

Nail PittingIn psoriasis, the nails are affected in approxi-

mately 25 percent to 50 percent of patients; inabout 15 percent of these, permanent pitting oc-curs (Figure 5). In a patient with psoriasis whosecutaneous lesions have cleared, the presence ofpsoriatic arthritis is seldom considered, since suchpatients frequently forget to report a skin diseaseof the past, perceiving no connection of arthritisto that remote illness. However, the finding ofpitted nails in an arthritic patient is of help inidentifying psoriasis as a possible underlying causeof the articular disorder.

Rarely, nail pitting may be congenital, but thehistory of such nails since birth should avoid con-fusion with psoriatic nails.

Nails may occasionally be pitted as a result of

Figure 4.-Mees nail. Note transverse white line onmiddle of nail plate.

neurotrophic conditions such as nerve damage. Insuch instances, the pits often clear with recovery.The Reiter syndrome occasionally leads to

pitting, but frequently the nails are surprisinglynormal when the cutaneous lesions (keratodermiablennorrhagica) abate.

ClubbingClubbing of the nails has been of interest to

physicians since its initial description by Hippoc-rates in empyema. It is known to occur in avariety of disparate conditions, only some of whichhave a common denominator. Many patients withclubbing such as those with cyanotic congenitalheart disease have decreased arterial oxygensaturation; many others have some disturbance ofpulmonary circulation. However, clubbing occursin diseases as different as Crohn disease, ulcera-tive colitis, biliary cirrhosis, primary hypertrophicosteoarthropathy, cystic fibrosis and cancer of theesophagus. As with all diseases, the geographicdistribution of illness should provide information,since the prevalence of a disease so often dependsupon geography. For example, nail clubbing innortheastern Brazil is most often a manifestationof schistosomiasis of the lung which results inarteriovenous shunts decreasing arterial oxygensaturation.

Familial clubbing is unassociated with systemicdisease and should offer no problem in differentialdiagnosis.An objective assessment of the presence of

clubbing can be obtained by measuring the ratioof the depth of the finger at the cuticle and alsoat the distal interphalangeal joint. The ratio is

Figure 5.-Pitting of nails in patient with psoriasis.

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greater than one in digital clubbing and less thanone in normal subjects.0 Clubbed nails have beenobserved to manifest more rapid growth thannormal nails.1

Renal DiseaseCertain findings in the nail give valuable clues

about the presence of renal disease. The nail-patella syndrome has already been mentioned.What has been termed the half-and-half nail(Figure 6), in which the proximal portion is whiteand the distal portion has a deep reddish color,was first noted by Bean and Clifton,' and sub-sequently studied by Lindsay.7 Of the 25 patientswhom Lindsay identified with the half-and-halfnail, 24 had azotemia; the remaining patient hadcirrhosis.

In nephrotic syndrome, the fingernails may alsoundergo a distinctive change, consisting of singleor multiple transverse whitish bands seen in thenail bed. These bands described by Muercke8 werefouind in patients whose serum albumin was lessthan 2.2 grams per dl. The bands were mostprominent in patients with severe and prolongedhypoalbuminemia from any cause, although mostof the patients were nephrotic. The banding re-gressed and disappeared when the serum albuminwas raised toward normal.

Multiple splinter hemorrhages have been notedin patients undergoing hemodialysis. In one series,

x -~~~~~~~~~~~~~~~~~~~~~l-

Figure 6.-Half-and-half nail (Lindsay nail) of renalfailure.

20 percent of patients on dialysis manifested thisfinding.9

Liver DiseaseIn chronic liver disorders, certain characteristic

changes also appear in the nails. In cirrhosis ofthe liver, the nail may appear completely pale.The white nail has a high specificity for liver dis-ease; less than 7 percent of patients withouthepatic disease manifest this finding.'The nail in liver disease may take on the ap-

pearance of a wide, pale, proximal portion and anarrow, deep-red distal portion (Figure 7). Thisfinding, termed the three-quarter, one-quarternail (Terry nail) is frequently seen in chronichepatic cirrhosis but is not specific for liver dis-ease; it may be observed in normal subjects andin patients with peripheral vascular disease, Ray-naud phenomenon, systemic lupus erythematosusand also in other connective tissue disorders. Thenails may give a clue to the presence of twochronic liver disorders that are potentially re-versible: Wilson disease and hemochromatosis.Bluish lunulae have been observed in Wilsondisease; in hemochromatosis, a blackish pigment,presumably melanin, appears on the nails as itdoes on the skin.

Pigmentation of the NailsIn addition to hemochromatosis, nails may

undergo a variety of color changes from variousdrugs as well as from conditions that causemelanin or other pigments to be deposited. In

Figure 7.-Three-quarter, one-quarter nail of chronicliver disease shows distal band of deep color andlarger, pale proximal area.

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small cell carcinoma of the lung, adrenocortico-trophic hormone and melanin-stimulating hor-mone may be produced and may result in pig-mentation of the nails as well as associated skin.Nail pigmentation may also be observed in Addi-son disease. With respect to drugs, tetracyclinemay produce a yellowish nail and chloroquine, ablue-black nail; adreamycin and cyclophospha-mide10 both have been reported to cause blackishnails. Argyria, which is a well-known cause ofpigmentation of the gums, also results in a slate-grey color to the nails. Other causes of nail colorchange include industrial and cosmetic dyes,resorcin, pseudomonas infection, phenophthalein,and nicotine.

Blood Diseases

Chronic anemia produces a condition knownas koilonychia, in which the nail becomes spoon-shaped. This condition is quite uncommon in de-veloped countries but is seen with relative fre-quency in areas where chronic anemia, secondaryto parasitic disease, is widespread. Hemorrhagesunder the nail plate are found in leukemic pa-tients and in those with bleeding diatheses.

Miscellaneous Conditions

The yellow nail, which presents as a thick,opaque slow-growing nail, is seen in lymphaticblockage. Onycholysis, in which the distal portionof the nail is pulled away from the bed, is a signof hyperthyroidism (Plummer nails) and also is

Figure 8.-Splinter hemorrhages in patient with vascu-litis.

found in hypothyroidism, in traumatic conditions,fungus disease, impaired circulation and psoriasis.

Connective Tissue DisordersThe diagnostic value of splinter hemorrhages

(Figure 8) was mitigated following a report inwhich about 10 percent of hospital inpatients hadthis finding;'1 however, patients in hospital fre-quently harbor infections, vasculitis, renal failureand other diseases in which immune complexesare found. Immune complexes are thought toparticipate in the production of the subungualhemorrhages. An occasional splinter hemorrhageis of no diagnostic value and is often the resultof trauma; however, multiple splinter hemor-rhages, especially when trauma can be eliminated,increase their diagnostic value. Splinter hemor-rhages in profuse numbers are found frequentlyin systemic lupus erythematosus, renal disease,rheumatoid arthritis, mixed connective tissue dis-ease, vasculitic syndromes, bacterial endocarditisand trichinosis. They have also been observed ineosinophilic polymyositis.12 These lesions, whichinvolve the spirally wound capillaries, are deepin the hyponychia and give a positive benzidinetest.13

Dermatomyositis causes an erythema of thedistal nail folds (Figure 9). This condition is asso-ciated with hyperkeratotic thickening and rough-ening of the cuticles.14

Figure 9.-Erythema of nail folds in patient with derma-tomyositis.

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The capillaries in the skin proximal to the,cuticle can be viewed by the naked eye but areseen more clearly with a magnifying lens and adrop of oil. Giant capillary loops adjacent toareas with no capillaries are common to derma-tomyositis and scleroderma and are rarely ob-served in other connective tissue disorders. Thefinding of giant capillary loops in a patient withRaynaud phenomenon is evidence that the condi-tion is not innocent and that there may be anassociated collagen disease.15 These large capillaryloops have also been observed in patients whodevelop a scleroderma-like disorder when exposedto polyvinyl chloride.1; In systemic lupus erythe-matosus and rheumatoid arthritis, one may seean increased size of nail fold capillaries whichhave the appearance of a picket fence, but giantloops are rarely seen. In various disorders asso-ciated with arteritis, small infarcts about the nailfolds occur. In such instances, an arteriogram ofthe hand will often show changes that are typicalof vasculitis.

REFERENCES1. Bean WB: A discourse on nail growth and unusual finger-

nails. Trans Am Clin Climatol Assoc 74:152-167, 19622. Bean WB: Nail growth: A twenty year study. Arch Intern

Med 111:476-482, Apr 19633. Bean WB: Nail growth: Twenty-five years' observation. Arch

Intern Med 122:359-361, Oct 19684. Bean WB: Nail growth: 30 years of observation. Arch Intern

Med 134:497-502, Sep 19745. Pittsley RA, Shearn MA: Nail down handedness. JAMA 236:

819, Aug 16, 19766. Sly RM, Ghazanshahi S, Buranakul B, et al: Objective assess-

ment for digital clubbing in Caucasion, Negro, and Oriental sub-jects. Chest 64:687-689, Dec 1973

7. Lindsay PG: The half-and-half nail. Arch Intern Med 119:583-587, Jun 1967

8. Muehrcke RC: The finger-nails in chronic hypoalbuminaemia.Br Med J 1:1327-1328, Jun 9, 1956

9. Blum M, Aviram A: Splinter hemorrhages in patients re-ceiving regular hemodialysis. JAMA 239:47, Jan 2, 1978

10. Nixon DW: Alterations in nail pigment with cancer chemo-therapy. Arch Intern Med 136:1117-1118, Oct 1976

11. Kilpatrick ZM, Greenberg PA, Sanford JP: Splinter hemor-rhages: Their clinical significance. Arch Intern Med 115:730-735,Jun 1965

12. Layzer RB, Shearn MA, Satya-Murti S: Eosinophilic poly-myositis. Ann Neurol 1:65-71, Jan 1977

13. Martin BF, Platts MM: A histological study of the nailregion in normal human subjects and in those showing splinterhaemorrhages of the nail. J Anat 93:323-330, Jul 1959

14. Samitz MH: Cuticular changes in dermatomyositis: A clinicalsign. Arch Dermatol 110:866-867, Dec 1974

15. Maricq HR, Spencer-Green G, LeRoy EC: Skin capillaryabnormalities as indicators of organ involvement in scleroderma(systemic sclerosis), Raynaud's syndrome and dermatomyositis.Am J Med 61:862-870, Dec 1976

16. Maricq HR, Johnson MN, Whetstone CL, et al: Capillaryabnormalities in polyvinyl chloride production workers: Examina-tion by in vivo microscopy. JAMA 236:1368-1371, Sep 20, 1976

Advantage of 'Pencil-Point' Needlesin Reducing Postspinal Headaches

WE ARE LIMITED, or should be limited as much as possible, to 25-gauge spinalneedles (when doing subarachnoid blocks) in order to avoid postpuncture head-aches. It is a more difficult technique than it would be on a medical ward wherelarge size needles such as an 18-gauge or a 20-gauge needle are frequently used.If one wishes, instead of using a 25-gauge spinal needle, without increasing therisks of postpuncture headaches, one can use pencil-point type needles such asthe Whitacre needle-which is commercially available. The advantage of theWhitacre needle is that it has the rigidity of a 22-gauge needle, but separates thefibers rather than cuts them and hence this diminishes the incidence of postpunctureheadaches to that of the level of a 25-gauge spinal needle.

-WILLIAM GOTTSCHALK, MD, ChicagoExtracted from Audio-Digest Obstetrics and Gynecology, Volume25, Number 9, in the Audio-Digest Foundation's subscriptionseries of tape-recorded programs. For subscription information:1577 E. Chevy Chase Drive, Glendale, CA 91206.

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