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NAIL PATHOLOGIES
NAILS IN CHILDHOOD ----------
Nail plate is relatively thin and may show temporary koilonychias that is most prominent in thegreat toes
Normal surface markings of nails may be unduly prominent giving rise to herringbone nails
Solitary Beau’s lines are common in infancy
Premature infants may have nail plates shorter than the distal digital pulp giving rise to an ingrownappearance. In a more gross form there may present as congenital hypertrophy of the lateral nailfolds of the hallux. This disappears in some months
Punctate leuconychia, pitting and onychophagia are also seen
NAILS IN OLD AGE ---------
Grow slow
Pale, dull and opaque
The proximal half appears white with no discernable lunulae, the distal free edge is opaque but thecentral portion retains the normal pink hue. These have been termed “ Neapolitan nails” after thethree banded Neapolitan ice cream.
Both platonychia and koilonychias occur
Nails are often soft and fragile with splitting and fissuring. Longitudinal ridging is common
Pertinax bodies are often seen
NAILS IN PREGNANCY ----------
Accelerated growth
Brittleness or softening. Distal onycholysis and subungual hyperkeratosis may appear in the firsttrimester
Beau’s lines may develop after delivery.
The nail abnormalities usually reverse in 6 months to 1 year postpartum.
DEVELOPMENTAL DISORDERS OF THE NAIL UNITANONYCHIA(
absence of nails)---
Isolated
Nail patella syndrome
In association with hypoplastic or absent terminal phalanx
Hypohidrotic ectodermal dysplasia
With various craniofacial malformation
CONGENITAL MALALIGNMENT OF THE BIG TOE NAILS
---- There is usually lateral deviation of thelong axis of nail growth relative to the distal phalanx of one or both hallux. The affected nails are unableto grow to normal length, are discolored and thickened and the proximal pulp tissue in front of it becomesheaped up. The condition is AD with variable expression.
MACRONYCHIA AND MICRONYCHIA
---- Macronychia is seen in Von Recklinghausen’s disease,epidermal nevus syndrome and Maffucci syndrome. Micronychia is sometimes seen in Turner syndrome.
POLYONYCHIA
--- With polydactylt is found in Lawrence Moon Bidel syndrome
ECTOPIC NAILS
---------- found in palmer surface of fingers and on heels
RACKET NAILS
--------- It is a common developmental anomaly that is inherited as an autosomaldominant trait. Thumbs are more commonly involved. In affected individuals the epiphysis of the terminalphalanx of the thumb close prematurely at the age of 7-10 years. Therefore the distal phalanx of theaffected thumb is shorter and wider than the normal and the nail only conforms to the altered shape of the thumb. The affected nails are opaque and are short and wide with loss of normal curvature. Racketnails can also occur without any underlying bone anomaly. Acquired racket nails follow bone resorption asin different examples of acroosteolysis.
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PERIODIC NAIL SHEDDING
---- One or more nails are shed repeatedly. The developmental defect isinherited autosomally in a dominant fashion. Trauma from ill fitting shoes is a common cause of repeatedshedding of nails in sportsperson. The great toe nails are frequently affected.
DOLICONYCHIA
---- normally the quotient between the length and breadth of nail is 1 +/- 0.1. indoliconychia ot is greater and is about 1.9. seen in EHLER DANLOS, MARFAN’S or in association withEUNUCHOIDISM OR WITH HYPOPITUITARISM.
CIRCUMFERENTIAL NAILSNAIL PATELLA SYNDROME:
AUTOSOMAL DOMINANT.
•
The defect is in a protein involved in dorsoventral limb patterning during embyogenesis. There isabnormality of ectodermal and mesodermal structure.
•
Defects in nails are as follows:
Nails are grossly defective but the nail bed is always present
even if the nail iscompletely missing.
The size of the nails are 1/3
rd
to ½ the normal size
and never reach the free edge of the finger. The defect is greatest in the thumbnails and gradually becomes less towards thelittle finger.
The lunula may be triangular
.
•
Bone changes are:
Small patella
so that the knees are unstable.
Bony spines from the posterior aspect of iliac bones.
Hyperextension of joints.
•
Other abnormalities:
Skin laxity
Renal abnormalities
PACHYONYCHIA CONGENITA:
A genodermatoses, AUTOSOMAL DOMINANT.
•
2 variants: (1) PC-1 (Jadassohn- Lewandosky type) (2) PC-2 (Jackson Lawler type)
•
In PC-1, there is defect in gene coding for keratins 6a and 16 whereas in PC-2 , there is defect ingene coding for keratin 6b and 17.
•
In PC-1, nails are normal at birth but within months they undergo
yellow- browndiscolouration
,
lateral compression and uplifting of the nail plate(Hands> Feet
). In somecases there is also nail bed and hyponychial hyperkeratosis. Associated features are :
Palmoplanter hyperkeratosis and hyperhydrosis
Mouth and corneal dyskeratoses
: grayish white thickening of oral mucosa termedleukokeratoses. These changes are most marked over dorsum and lateral aspect of tongue.
Warty skin lesions at knee, elbow, ankle, popliteal fossa ,buttock.
Acral bulla
.
•
In PC-2,
There is
less severe nail changes and keratoses.
Natal teeth
Epidermiod cyst, hamartoma, sebocystomatosis
Dry lusterless kinky hair, straight eyebrows.
ECTODERMAL DYSPLASIA
----------- nail dystrophy. The nail grow slowly and are never able to reachthe fingertip. Affected nails are small and conical.
DYSKERATOSIS CONGENITA
-------- Nail abnormities are the first to appear. Nails are progressivelydystrophic and shed. There may be recurrent episodes of suppurative paronychia.
FOCAL DERMAL HYPOPLASIA
-------- the nails are thin and spoon shaped and there is no lunulae. Nailsare absent in 50% of instances.
TRICHOTHIODYSTROPHIES (BIDS. IBIDS, PIBIDS or Tay syndrome
) ------ onychauxis, subungualhyperkeratosis, onychoschizia, longitudinal grooving, koilonychias, yellow nail discoloration are some of the abnormalities.
KID syndrome
---------- thick white nails
Heritable PPK
----- nail thickening and increased curvature of nails
CHILD syndrome
---------- in hemidysplastic limbs nails are brittle.
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LAMELLAR ICHTHYOSIS
----- Thick and striated nails.
PROGERIA, PANGERIA AND ACROGERIA
----- Nails are small thin or dystrophic.
DOWN SYNDROME
---------- Macronychia and clubbing
TURNER SYNDROME
---------- Nails are narrow, hyperconvex and deep set. A recalcitrant chronicparonychia is ssen
ROTHMUND THOMSON SYNDROME
--------- Nails are small and dystrophic
INCONTINENTIA PIGMENTI
------ Nails are small and dystrophic. Tender subungual tumors and wartyperiungual lesions have been observed in adolescent children.
PEUTZ JEGHERS SYNDROME
---------- Brownish pigmentation
WILSON DISEASE
------------ Azure lunula
ACRODERMATITIS ENTEROPATHICA
---- Nail changes are secondary to paronychial inflammation.Longitudinal ridges, multiple Beau’s lines and dystrophy are common. Nail bed hyperplasia is also seen
TUBEROUS SCLEROSIS
---- Subungual or periungual fibromas (Koenan’s tumor) occur in 50% of patients with tuberous sclerosis. They are noticed after puberty and continue to develop with age. The nailplate frequently shows longitudinal ridging or grooving or may become partially or completely atrophicfrom pressure of the tumor on the matrix.
DARIER’S DISEASE
---- Nail changes are very common in Darier,s disease. They occur as:
•
Red and/or white longitudinal streaks in the nail often terminating in aV shaped nick. ------- Thestreak mar represent a zone of fragile or thinned nail which makes it prone to fragmentation at thetip .
•
Excess ridging and rough nail surface.
•
Thickening of nail plate(occasional)
•
Leuconychia
•
Subungal hyperkeratosis.
EPIDERMOLYSIS BULLOSA
---- Nail abnormalities have been reported in all forms of epidermolysisbullosa except the Weber Cockayne and the Koebner types and the Mendes da Costa variant but they aremost frequent and intense in the junctional and dystrophic varieties of epidermolysis bullosa. IT IS IN THEFORM OF PARONYCHIA, NAIL LOSS OR DYSTROPHY.****************************************************************************************
YELLOW NAIL SYNDROME
: The condition usually occurs in adults. The nails in yellow nail syndromeare:
•
Yellow due to
thickening
•
Increased
transverse and longitudinal
curvature
•
Nails grow at greatly reduced pace
: 0.1- 0.25mm/ week for fingernails compared with theslowest normal rate of 0.5mm/week.
•
All 20 nails may or may not be involved.
•
Histologically the nail
bed and matrix stroma are replaced by dense fibrous tissue
obstructing the lymph flow.
•
Other findings include:
Lymphoedema
mostly of legs; less often of hands and face. It is because of lymphaticchannel atresia.
Respiratory and sinus tract disease. 
•
The condition may
be associated with malignant neoplasm.
•
Treatment includes: IRTACONAZOLE/ FLUCONAZOLE WITH ORAL VITAMIN E.
•
Other causes of yellow nails:
Lichen planus
Hypothyroidism;
Diabetes mellitus
AIDS
TetracyclineSHELL NAIL SYNDROME:
It is probably a variant of yellow nail syndrome and described in associationwith bronchiectasis. There is excessive longitudinal curvature of the nail plate and a small shell like spaceis present between the thickened nail plate and the atrophic nail bed.
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Sallybabie

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reply05 / 24 / 2010
Claudia flor

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reply09 / 17 / 2009
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