Nails Flashcards
Eponychium
cuticle - thin strip of cornified epithelium that adheres to the nail at the edge of the proximal nail fold. Damage to the cuticle results in predisposition to inflammation of the nail fold –> disturb the underlying matrix and nail plate production
Lunula
white semi circle, junction between the matrix and the nail bed
Nail bed
- underlying support structure - extends from the distal margin of the lunula to the onychodermal band. Has rugose rete ridges to enhance adhesion to the nail plate
- The nail bed and nail plate lack a granular layer –> if there is a granular layer then that indicates pathology
Nail plate
packed onychocytes, continuous with the matrix
Vascular supply to the nail
- 2 dorsolateral and 2 ventrolateral arteries that don’t have an internal elastic lamina - very tortuous –> ++ anastamosis
- Cold environment –> constriction of vasculature, and AV anastamoses within glomus bodies maintain sufficient blood supply
- Glomus bodies: encapsulated oval structures containing modified smooth muscle cells that dilate upon exposure to the cold –> prominent in subungual tissue which is the most common site of glomus tumours
Nerve supply to the nail
- 2 dorsal and 2 ventral branches
- meissner and pacinian bodies (touch), type 1 fibres and temp all abundant in finger tips
Nail-patella syndrome:
- LMX1B mutation, autosomal dominant
- this encodes a transcription factor that regulates collagen synthesis
- involves the thumb, and other fingers to a lesser extent –> nails are absent or hypoplastic, dystrophy usually more marked on the radial side of the digit
- bone: absent or hypoplastic patellae, radial head dysplasia, iliac crest exostoses
- X-ray: iliac horns on pelvic x-ray
- 40% –> nephropathy –> 8% renal insufficiency
Clouston syndrome
form of ectodermal dysplasia due to gap junction gene mutation - GJB6 - encodes coonnexin –> thickened nails with onycholysis, hypotrichosis and palmoplantar keratoderma
Matrix function
- onychocytes migrate upward and distally along an oblique axis during maturation and differentiation
- proximal part of the matrix produces the dorsal nail plate. Abnormalities result in pitting, longitudinal ridging/splitting
- distal matrix produces the ventral nail plate, abnormalities result in true leukonychia
Nail unit melanocytes
- found in the nail matrix but not in the normal nail bed. foci of pigment in the nail bed that are separated from nail folds and matrix are not melanocytic in origin unless amelanotic melanoma or metastatic melanoa
- more abundant in the distal matrix
- main distribution in the nail plate is pigmented bands - longitudinal melanonychia
- multiple streaks seen in those with darker skin, trauma or inflammation
Nail immune system
- reduced expression of MHC class I and II antigens and decreased numbers of antigen presenting cells
- immune privilege prevents over-reactivity to environmental antigens and makes vulnerable to infections and their oncogenic effects
Pachyonychia congenita
AD disorder encoding keratin 6a, 6b, 16 or 17 - these are in the nail bed, HF, sebaceous gland and palmoplantar –> leads to thickening of the nail plate (subungual hyperkeratosis), painful palmoderma keratomer, follicular keratoses, oral leukokeratosis and cysts
Beau’s line
- transverse depressions of the nail plate surface from temporary interruption of the mitotic activity of the proximal nail matrix
- depth indicates extent of damage, width indicates duration
- most common causes: mechanical trauma, derm disease (eczema), if all fingernails –> systemic cause
Nail growth
- fingernails grow faster than toenails: 2-3 mm/month, 1 mm/month
- complete replacement of a nail requires 6-18 months (fingernails, toenails)
- as the nail plate emerges from the proximal nail fold and progresses distally, it strictly adheres to the nail bed, approaches the tip of the digit and detaches from the nunderlying tissue forming the hyponychium
Nail signs - principle
- Nail plate alterations –> due to matrix damage
- Nail plate detachment and/or uplifting arise from dysfunction of the hyponychium or nail bed
Onychomadesis
- detachment of the nail plate from the proximal nail fold - due to severe insult that produces a complete arrest of nail matrix activity
- similar causes to Beau’s line
- it can also be from: hand, foot & mouth, scarlet fever, Kawasaki, horizontal orange-brown chromonychia
Nail pitting
- punctate depressions
- foci of abnormal keratinization of the proximal nail matrix which results in clusters of parakeratotic cells within the dorsal plate
- over time, shedding of the parakeratotic cells leads to pits
- Cause: psoriasis, alopecia areata, eczema
Onychorrhexis
- longitudinal ridging and fissuring of the nail plate
- depth of the ridges can vary
- associated with nail thinning
- causes: lichen planus, impaired vascular supply, trauma, tumours that compress the nail matrix
- ddx: age related ridging of the nail plate
Trachyonychia/sand paper nails
- Excessive longitudinal ridging –> nail plate roughness
- Idiopathic, or can be from inflammatory skin disease - particularly alopecia areata, can be LP, eczema, psoriasis
- fairly asymptomatic
- Rx: systemic steroids and retinoids noted when treating alopecia areata or psoriasis, JAK inhibitor tofacitinib
True leukonychia
- looks white because of the presence of parakeratotic cells within its ventral aspect
- from nail matrix disturbance
- Three types:
- Punctate leukonychia - small opaque white spots that move distally with nail growth, and sometimes disappear before reaching the distal nail. Caused by trauma, see it in kids
- Striate leukonychia - one or more transverse opaque white, parallel lines that migrate distally with growth. often due to matrix trauma secondary to manicures, trauma from shoes, also typical of Mees lines - white transverse bands seen in arsenic and thallium poisoning
- Diffuse leukonychia - completely opaque. Rare, results from PLCD1 mutation or GJA1 (latter has keratoderma and hypotrichosis)
- Bart-Pumphrey syndrome: deafness, knuckle pads, palmoplantar keratoderma, leukonychia, mutation in GJB2
Koilonychia
- plate is thinned and flattened with upward eversion of its lateral and distal edges
- kids - 2nd - 4th toes is physiologic and resolves spontaneously
- adults - severe iron deficiency and systemic amyloidosis, manual laborers
Onycholysis
- distal nail plate is detached from the nail bed and appears white due to air in the subungual space
- if exogenous pigment present - yellow from fungi, green from pyocyanin
- Causes: environmental trauma, psoriasis, infections, drugs (tetracyclines), metabolic/systemic (hyperthyroidism), tumours (subungual exostoses and SCC)