THE JOURNAL OF FAMILY PRACTICE Stamatis Gregoriou, MD, Nail disorders and systemic George Argyriou, MD, George Larios, MD, and disease: What the nails tell us Dimitris Rigopoulos, MD, PhD University of Athens Here’s what you’ll see and what to suspect with these Medical School, Dermatology 11 nail disorders Department, Nail Clinic, Athens [email protected] ® Dowden Health Media CopyrightFor personal use only IN THIS ARTICLE ❚ Onycholysis ❚ Clubbing ❚ Can you name these 2 nail conditions? ❚ Koilonychia ❚ What underlying diseases do you suspect ❚ Onychomadesis are behind these conditions? ❚ Beau’s lines ❚ Pitted nails If you said onycholysis (left) and red lunula (right), you are correct. As for ❚ Muehrcke’s nails the underlying diseases: The patient with onycholysis has hyperthyroidism ❚ and the patient with red lunula has chronic obstructive pulmonary disease Terry’s nails (COPD). Onycholysis and red lunula are among the more common changes ❚ Half-and-half nails to the morphology (shape) and color of the nail—the 2 ways by which nail ❚ Red lunula changes are classifi ed. ❚ Splinter hemorrhages ail abnormalities can be a reveal- This review of common—and not so ing sign of underlying disease, common—nail disorders shows which Nand because the nails are readily changes to the nail are more likely to examined, a convenient diagnostic tool, occur with which underlying internal as well. diseases. CONTINUED www.jfponline.com VOL 57, NO 8 / AUGUST 2008 509 For mass reproduction, content licensing and permissions contact Dowden Health Media. 509_JFP0808 509 7/18/08 12:11:50 PM THE JOURNAL OF FAMILY PRACTICE Proximal Nail anatomy nail fold Nail changes are classifi ed according Eponychium to whether they occur in the morphology Nail bed (shape) or color of the nail. Onycholysis, clubbing, and koilonychia are some Hyponychium of the most common changes in the morphology of the nail. Red lunula is Nail matrix one of the most common changes in the color of the nail. Distal edge of nail plate Nail plate Lateral nail groove Lunula Lateral nail fold Eponychium Distal phalanx Epidermis ILLUSTRATION © ROB FLEWELL Onycholysis-associated ❚ Onycholysis 1,2 systemic diseases What you’ll see: Distal separation of • Amyloid and multiple the nail plate from the underlying nail myeloma bed. Nails with onycholysis are usually • Anemia smooth, fi rm, and without nail bed in- • Bronchiectasis fl ammation. It is not a disease of the nail • Carcinoma (lung) matrix, though nail discoloration may • Erythropoietic porphyria appear underneath the nail as a result of • Histiocytosis X • Ischemia (peripheral) secondary infection. • Leprosy What to suspect: Onycholysis is associ- • Lupus erythematosus ated with many systemic conditions, • Neuritis including thyroid disease—especially • Pellagra hyperthyroidism. (See list at left.) The • Pemphigus vulgaris nail changes seen with hyperthyroidism • Pleural effusion usually consist of onycholysis beginning • Porphyria cutanea tarda in the fourth or fi fth nail, the so-called • Psoriatic arthritis Plummer’s nails.1 Nakatsui and Lin2 have • Reiter’s syndrome suggested that patients with unexplained • Scleroderma • Syphilis (secondary onycholysis be screened for asymptom- and tertiary) atic thyroid disease. • Thyroid disease 510 VOL 57, NO 8 / AUGUST 2008 THE JOURNAL OF FAMILY PRACTICE 510_JFP0808 510 7/18/08 12:22:24 PM Nail disorders ▼ ❚ Clubbing What you’ll see: Increased transverse and longitudinal nail curvature with fi bro- vascular hyperplasia of the soft tissue proximal to the cuticle. With clubbing, the Lovibond’s angle, formed between the dorsal surface of the distal phalanx and the nail plate, is greater than 180 de- grees. Schamroth’s sign—the disappear- ance of the normal window between the back surfaces of opposite terminal pha- langes—may also be present.3 What to suspect: Clubbing may be heredi- tary, idiopathic, or acquired in association with a variety of disorders. It may also be unilateral or bilateral. Unilateral clubbing has been associated with hemiplegia and of nail disorders in HIV-infected patients vascular lesions, while bilateral clubbing and found that clubbing affects 5.8% of has been linked to neoplastic, pulmonary, these patients. Moreover, Cribier’s data cardiac, gastrointestinal, infectious, endo- reinforced the notion that clubbing could crine, vascular, and multisystem diseases. be an early sign of AIDS in pediatric pa- Cribier et al4 studied the frequency tients, and thus play a role in diagnosis. ❚ Koilonychia What you’ll see: Concave thin nails with everted edges shaped like a spoon and capable of retaining a drop of water. It is more common in fi ngernails, but is oc- FAST TRACK casionally seen in toenails. Koilonychia is What to suspect: This nail sign may result from trauma, constant exposure of hands sometimes to petroleum-based solvents, or nail-patella a normal variant syndrome. Koilonychia is most commonly in infants; associated with iron defi ciency anemia it usually and occasionally occurs in patients with hemochromatosis. Other frequent system- koilonychia is sometimes a normal variant disappears in ic causes of koilonychia include coronary in infants; it usually disappears in the fi rst the fi rst few disease and hypothyroidism.5 In addition, few years of life. years of life ❚ Onychomadesis What you’ll see: Proximal separation of the nail plate from the nail bed. This typically results in shedding of the nail. What to suspect: Trauma is the usual cause. Less common causes include poor nutritional status, febrile illness, or drug sensitivity. Wester et al6 observed the develop- ment of onychomadesis in a critically www.jfponline.com VOL 57, NO 8 / AUGUST 2008 511 511_JFP0808 511 7/17/08 2:54:18 PM THE JOURNAL OF FAMILY PRACTICE ill patient with a large pulmonary ab- It has also been associated with Kawa- scess. Onychomadesis is often a clinical saki disease8 and hand, foot, and mouth manifestation of pemphigus vulgaris.7 disease.9 ❚ Beau’s lines What you’ll see: Transverse depressions in the nail plate that occur as a result of a temporary cessation in nail growth. What to suspect: The causes are similar to those of onychomadesis and include trauma, poor nutritional status, febrile illness, and drug sensitivity. ❚ Pitted nails What you’ll see: Pinpoint (or larger) de- pressions in an otherwise normal nail. What to suspect: Pitting is usually associat- ed with psoriasis and affects 10% to 15% of patients with the disorder.10 Pitting has also been reported in patients with Reiter’s syndrome (and other connective tissue dis- orders), sarcoidosis, pemphigus, alopecia areata, and incontinentia pigmenti.5 FAST TRACK Terry’s nails have ❚ Muehrcke’s nails been reported What you’ll see: Transverse white bands in hemodialysis parallel to the lunula. These bands usu- patients and renal ally occur in pairs and extend all the transplant patients way across the nail. This nail disorder is uncommon, and is 1 of 3 forms of leukonychia caused by abnormalities in nail bed vascularization. (The other 2 forms—Terry’s nails and half-and-half nails—are described on page 513.) What to suspect: Muehrcke’s nails ap- pear in patients with hypoalbuminemia and can improve if serum albumin lev- els return to normal. They may also oc- cur in patients with:11,12 • nephrotic syndrome, chemotherapy. • glomerulonephritis, Muehrcke’s lines have also been de- • liver disease, scribed in a patient with Peutz-Jeghers • malnutrition, and syndrome,13 as well as in a heart transplant • those who have undergone recipient.14 512 VOL 57, NO 8 / AUGUST 2008 THE JOURNAL OF FAMILY PRACTICE 512_JFP0808 512 7/17/08 2:54:22 PM Nail disorders ▼ ❚ Terry’s nails What you’ll see: Most of the nail plate is white, with a narrow pink distal band. All nails tend to be uniformly affected, with an appearance of ground glass.15 Terry’s nails have been found in 80% of patients with cirrhosis of the liver.15 What to suspect: One study found Terry’s nails in 25% of 512 consecutive hospi- tal inpatients, with researchers linking the disorder with cirrhosis, chronic CHF, and adult-onset diabetes mellitus.16 On rare occasions, Terry’s nails have been re- ported in hemodialysis patients and renal transplant recipients.17 Terry’s nails have also been observed in HIV patients.4 ❚ Half-and-half nails (Lindsay’s nails) What you’ll see: The proximal portion on the nail bed is white because of edema of the nail bed and capillary network; the distal portion is pink or reddish brown. The nail plate is unaffected. What to suspect: This nail disorder has occurred in patients with renal disease as- sociated with azotemia.18 Half-and-half nails have also been detected in hemo- dialysis patients, renal transplant recipi- FAST TRACK ents,17 and in HIV patients.4 Azure lunula has occurred in argyria ❚ Red lunula and in patients What you’ll see: The lunula is red. In ad- taking medications dition to the red lunula pictured here, like 5-fl uorouracil there is also the absence of lunula and azure lunula. and azidothymidine What to suspect: Red lunula has been associated with alopecia areata, and collagen vascular disease. It has also oc- curred in patients on oral prednisone for rheumatoid arthritis. Red lunulae are seen in cardiac failure, COPD, cir- rhosis, chronic urticaria, psoriasis, and carbon monoxide poisoning.19 Absence of lunula was the most common nail disorder in a group of he- modialysis patients (31.9%) and has also been reported in renal transplant recipi- occurred in argyria and in patients tak- ents (17.1%).17 Azure lunula occurs in ing medications like 5-fl uorouracil and patients with Wilson disease. It has also azidothymidine.20 CONTINUED www.jfponline.com VOL 57, NO 8 / AUGUST 2008 513 513_JFP0808 513 7/17/08 2:54:27 PM THE JOURNAL OF FAMILY PRACTICE ❚ Splinter hemorrhages What you’ll see: Extravasations of blood from the longitudinally oriented ves- sels of the nail bed.