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Andrew’s Chapter 33-Part II. David M. Bracciano D.O. An inflammatory reaction of the hair follicles Leads to cicatricial alopecia Small pustules surround the follicles Erythema, scaling, and smooth shiny depressed scars are apparent. Folliculitis Decalvans. Pseudopelade

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andrew s chapter 33 part ii
Andrew’s Chapter 33-Part II
  • David M. Bracciano D.O.
folliculitis decalvans
An inflammatory reaction of the hair follicles

Leads to cicatricial alopecia

Small pustules surround the follicles

Erythema, scaling, and smooth shiny depressed scars are apparent

Folliculitis Decalvans
folliculitis decalvans1
Pseudopelade

When the pustules have healed and scarring remains –pseudopelade occurs

Note intact follicles and single hairs growing

May occur on axillae and groin as well

Folliculitis Decalvans
folliculitis decalvans2
Etiology is unknown

Scarring alopecia in a middle-aged man, associated with a hyperkeratotic scale-crust with follicular hyperkeratosis and erythema

If gram stain and culture of the exudate reveals coagulase-positive staphylococci, this should be treated

Many times no organism is cultured

Folliculitis Decalvans
folliculitis decalvans4
Later lesions shows destruction of follicular epithelium, dense interstitial inflammation, and perifollicular fibrosis, with free hairs in the tissueFolliculitis Decalvans
folliculitis decalvans5
There is perifollicular and interstitial fibrosis accompanied by an interstitial infiltrate of lymphocytes and plasma cellsFolliculitis Decalvans
folliculitis decalvans6
Folliculitis Decalvans

The eventual loss of follicular epithelium leads to a granulomatous response triggered by free hair shafts

folliculitis decalvans7
Folliculitis Decalvans
  • TREATMENT:
  • Cephalosporins, dicloxacillin, and azithromycin and rifampin may be added to therapy for better long-term control
  • Oral zinc or vitamin C supplementation may enhance response
  • Chronic inflammation reactions may be helped with topical steroids and by intralesional triamcinolone
slide10
Thick, asbestos-like (amiantaceous), shiny scales attached to the lower part of the hair shaft, rather like tiles overlapping on a roof

Crusting may be localized or, less commonly generalized over the entire scalp

There are no structural changes in the hair, but in some patches where the crusting is thick, there may be purulent exudate under the crust and temporary alopecia may occur

Tinea Amiantacea

tinea amiantacea
Etiology is likely secondary to an infection occurring in seborrheic dermatitis or inverse psoriasis

Treatment should be shampoo daily or every other day with selenium sulfide susupension, or a tar shampoo , for a few weeks

Prior application of Baker’s P&S liquid is helpful to remove scale and crust

Derma-Smoothe and FS shampoo are also effective

Tinea Amiantacea
keratosis follicularis contagiosa
Also known as epidemic acne, epidemic follicular eruption, epidemic follicular keratosis, and Brooke’s disease

Unknown etiology

Occurs in children

Keratosis Follicularis Contagiosa
keratosis follicularis contagiosa1
Eruption is widespread and symmetrical, affecting chiefly the back of the neck, the shoulders, and the extensor surfaces of the extremities

Onset is acute, may affect large numbers of patients in a localized geographic area , and spontaneously involutes over a 3-to-6-week period

Keratosis Follicularis Contagiosa
keratosis follicularis contagiosa2
There is a horny thickening of these areas, especially pronounced about the follicles, where small black corneous may be discerned

Etiology has been hypothesized to be infectious- but not proven

Keratosis Follicularis Contagiosa
folliculitis nares perforans
Folliculitis Nares Perforans
  • Charcterized by small pustules near the tip of the inside of the nose
  • The lesion becomes crusted, and when the crust is removed it is found that the bulbous end of the affected vibrissa is embedded in the inspissated material
  • Staphylococccus aureus may at times be cultured from the pustules
  • Treatment is removal of the hair and topical ointment like mupirocin
perforating folliculitis
Charcterized by an asymptomatic eruption of erythematous follicular papules 2 to 8 mm diameter

Involving the extenxor ssurfaces of the upper arms, the buttockss, or the upper thighs

When small, whitish kerratotic plug is removed from the follicular papule, a small bleeding crater remains

Perforating Folliculitis
perforating folliculitis1
A dilated follicular unit contains a keratotic plug with an admixture of basophilic debris.

The follicular epithelium is perforated, and there are degenerated collagen fibers in the adjacent dermis.

Perforating Folliculitis
perforating folliculitis2
Resistant to treatment

Topical tretinoin is reported to be effective

One report of an HIV-infected man who responded well to thalidomide

Perforating Folliculitis
kyrle s disease
Originally termed hyperkeratosis follicularis et paarafollicularis in cutem penetrans

Rare disorder

Characterized by hyperkeraatosis, forming a horny cone that projects into the dermis, so that when it is removed a pitlike depression remains

Usually discrete papules, but may ccoalesce to form circinate plaques

Kyrle’s Disease
kyrle s disease1
There is a prediliction for the lower extremities, but the upper extremities, head, and neck may be involved

Koebner’s phenomenon may also be observed, in which elevated verrucous streaks or plaques are formed

The elevated verrucous streaks are only seen in the antecubital and popliteal spaces

Kyrle’s Disease
kyrle s disease2
Occurs almost exclusively in adults ages 20 to 63

No sex or racial differences seen

Has been associated with diabetes mellitus

Kyrle’s Disease
kyrle s disease3
Histologically:

Large keratotic and parakeratotic plugs penetrating through the epidermis into the dermis

These plugs cause an inflammatory an foreign-body giant cell reaction about the lower end of the plug in the dermis

Mild degenerative changes in the connective tissue with no increase in the elastic tissue also occur

Kyrle’s Disease
kyrle s disease4
Kyrle’s disease remains stable for years, with possible clearing when the associated illness is controlled

Ultraviolet treatment, methotrexate, topical corticosteroids, 5-fluorouracil, and keratolytics are usually ineffective

Topical retinoic acid 0.01% cream, isotretinoin, and etretinate have been effective in flattening the lesions

Kyrle’s Disease
reactive perforating collagenosis
Pinhead-sized, skin-colored papules that grow to 4 to 6 mm and develop a central area of umbilication in which keratinous material is lodged

The discrete papules may be numerous and involve sites of frequent trama

The lesions reach a maximum size in 4 weeks the slowly regress

Reactive Perforating Collagenosis
reactive perforating collagenosis2
Trichrome stain :

Blue-stained collagen fibers perforating the channel and extending to the surface

Reactive Perforating Collagenosis
reactive perforating collagenosis3
It is believed that this is caused by a peculiar reaction of the skin to superficial trauma

Koebnerization is often observed

Young children are most frequently affected

There is no specific treatment indicated, since the lesions involute spontaneously

Reactive Perforating Collagenosis
perforating disease of hemodialysis
It is reported that between 4% and 10% of dialysis patients develop perforating disorders

Lesions are characterized by dome-shaped papules on the legs, or less often on the trunk, neck, arms, or scalp, with variable itchiness

Early lesions may be pustular; late lesions resemble prurigo nodularis

Perforating Disease of Hemodialysis
perforating disease of hemodialysis1
Perforating Disease of Hemodialysis
  • The disease may remit promptly after a renal transplant and stopping dialysis
  • Topical tretinoin may be tried
traumatic anserine folliculosis
A curious gooseflesh-like follicular hyperkeratosis that may result from persistent pressure and lateral friction of one skin surface on another

Such friction is often caused by habitual pressure of elbows, chin or jaw, or neck, often while watching television

Two thirds of patients who develop this are atopic

Traumatic Anserine Folliculosis
erythromelanosis follicularis faciei et colli
A unique erythematous pigmentary disease involving the follicles

A reddish brown, sharply demarcated, symmetrical discoloration involving the preauricular and maxillary regions

Pigmentation may be blotchy

Follicular papules and erythema are present

Pityriasiform scaling and slight itching may occur

Keratosis pilaris is frequently found on the arms and shoulders

It preferentially affects Asian patients

Erythromelanosis Follicularis Faciei et Colli
disseminate and recurrent infundibulofolliculitis
A mildly pruritic eruption at times, and is chronic with recurrent exacerbations

Characterized by uniform papules, 1 or 2 mm in diameter, and involving all the follicles in the affected areas, which are usually the upper trunk and neck

Histologically, the infundibular portion of the follicles is chiefly affected, and the lesions are inflammatory rather than hyperkeratotic

Disseminate and Recurrent Infundibulofolliculitis
disseminate and recurrent infundibulofolliculitis1
Edema, lymphocytic and neutrophilic infiltration, and slight fibroblastic infiltration surround the affected follicles

Treatment with vitamin A has been reported to be effective

Isotretinoin or etretinate may also be useful

Disseminate and Recurrent Infundibulofolliculitis
lichen spinulosus
Disease frequently seen in children

Characterized by minute filiform horny spines protruding from follicular openings independent of any papule

The spines are discrete and grouped

Lichen Spinulosus
lichen spinulosus1
The lesions appear in crops and are symmetrically distributed over the trunk, limbs, and buttocks

There is a predilection for the

neck, buttocks, abdominal wall, popliteal fossa, extensor surfaces of the arms

A generalized distribution has been reported to occur with HIV infection

Lichen Spinulosus
lichen spinulosus3
Histology:

Shows simple inflammatory changes and follicular hyperkeratosis

Treatment:

The lesions respond to mild keratolytics like 3% resorcin or salicylic acid ointment, Keralyt gel, Lac-Hydrin lotion, and tretinoin

Lichen Spinulosus
hyperhidrosis
Palmoplantar Hyperhidrosis(Emotional Hyperhidrosis)

* Usually localized to the palms, soles or axillae

* May be worse during warm temperature *Can be AD inherited

*25% of patients with axillary hyperhidrosis have palmoplantar hyperhidrosis, but patients with palm and sole hyperhidrosis may have axillary hyperhidrosis

*Sweating may be intermittent or constant

Hyperhidrosis
hyperhidrosis1
Gustatory Hyperhidrosis

Experience excessive sweating of the forehead, upper lip, perioral region, or sternum a few minutes after eating spicy foods, tomato sauce, chocolate, coffee, tea, or hot soups

May also be caused by hyperactivity of the sympathetic nerves(Pancoast’s tumor) or postoperatively), sensory neuropathy (diabetes mellitus or subsequent to zoster), parotitis or parotid abscess, and surgery of the parotid gland

Hyperhidrosis
hyperhidrosis2
Other Localized Forms of Hyperhidrosis:

Localized sweating can occur over lesions of blue rubber bleb nevus, glomus tumors, hemangiomas and in POEMS syndrome, Gopalan’s syndrome, complex regional pain syndrome, and as a result of spinal cord tumors

Hyperhidrosis
hyperhidrosis3
Generalized Hyperhidrosis

May be induced by a hot, humid environment, a febrile illness, or vigorous exercise

Hormonal disturbances such as hyperthyroidism, acromegaly, diabetes mellitus, pregnancy, and menopause

Other causes include concussion, Parkinson’s disease, pheochromocytoma, hypogycemia, salicylism, and lymphoma, and metastatic tumors transecting the spinal cord

Hyperhidrosis
hyperhidrosis4
Treatment:

Treat underlying disorder

Topical aluminum chloride or aluminum chlorhydroxide

For the axillae, application of a 20% to 25% solution nightly to a very dry axillae

Avoid deodorant-use baking soda instead

For palmar hyperhidrosis apply 20% aluminum chloride tincture nightly, occluded with plastic gloves

Hyperhidrosis
anhidrosis absence of sweating
May be localized or generalized

Generalized anhidrosis occurs in anhidrotic ectodermal dysplasia, quinacrine anhidrosis, miliaria profunda, Sjogren’s syndrome, hereditary sensory neuropathy (type IV) with anhidrosis, some patients with diabetic neuropathy, and multiple myeloma

Anhidrosis with pruritis:

Present with itching when attempting to sweat

Absence of sweat when body temperature rises 0.5degrees C, fine papules appear at each eccrine orifice

Cooling completely resolves symptoms

Resolution may occur after several years

Anhidrosis= absence of sweating
anhidrosis
Ross syndrome= segmental anhidrosis associated with tonic pupils( Holmes-Adie syndrome)

Intolerance and segmental areas of anhidrosis, loss of deep tendon reflexes, and compensatory segmental hyperhidrosis of functionally intact areas

Anhidrosis localized to skin lesions over plaques of tuberculoid leprosy

Also over ares of segmental vitiligo, in the hypopigmented streaks of incontinentia pigmenti, and on the face of patients with follicular atrophoderma, basal cell carcinomas, and hypotrichosis

Anhidrosis
bromidrosis fetid sweat
Mainly in the axillae

Caused by bacterial decomposition of apocrine sweat, producing fatty acids with offensive odors

True bromidrosis is usually not recognized by the patient

Antibacterial soaps and many comercial deodorants are effective in controlling axillary malodor

Frequent bathing, changing of underclothes, shaving, and topical alumuminum chloride

Surgical removal of the glands is an option

Bromidrosis= fetid sweat
bromidrosis
Often patient s who complain of offensive axillary sweat actually have psychiatric disorders like delusion, paranoia, phobia, or a CNS lesion

Intranasal foreign body and chronic mycotic infection of the sinuses in differential

Fish odor syndrome-

Caused by excretion of trimethylamine( which smells like rotten fish) in the eccrine seat, urine, saliva, etc.

This chemical is produced from carnitine and choline in the diet

Caused by an autosomal dominant defect in the ability to metabolize trimethylamine

Bromidrosis
chromhidrosis
Rare disorder of the apocrine sweat glands

Frequently localized to the face or axillae

Most commonly sweat is yellow, less frequently blue, green, or black

Colored sweat appears in response to adrenergic stimuli, which cause myoepithelial contractions

Colored apocrine sweat fluoresces and is caused by lipofusion

Eccrine chromhidrosis is caused by the coloring of clear eccrine sweat by dyes, pigments, or metals on the skin surface

Examples of this: blue-green sweat from copper workers, the “red sweat” seen in flight attendants from the red dye in the labels of life-vests

Brownish staining from ochronosis

Small, round, brown or deep-green macules occur in the palms and soles in patients with excess bile secretion- liver disease

Chromhidrosis
fox fordyce disease
Fox-Fordyce Disease

Rare, occurs in women during adolescence or soon afterward

Conical, flesh-colored or grayish, intensely pruritic, discrete follicular papules in areas where the apocrine glands occur

fox fordyce disease2
Fox-Fordyce Disease

Apocrine gland sweating does not occur in areas of involvement

Hair density may be decreased

90% occur in women aged 13-35

Pregnancy leads to improvement

May occur postmenopausally or in males

fox fordyce disease3
Fox-Fordyce Disease

Treatment is difficult-

No form of therapy is uniformly effective

Estrogen therapy usually in the form of oral contraceptive pillsis effective

Topical tretinoin, topical and intralesional steroids, topical antibiotics, oral retinoids and UV phototherapy have been helpful in a small number of patients

granulosis rubra nasi
Granulosis Rubra Nasi

A rare familial disease of children

Occurring on the nose, cheeks, and chin

Characterized by diffuse redness, persistent hyperhidrosis, and a small dark red papules that disappear on diascopic pressure

The tip of the nose is red or violet

Disappears spontaneously at puberty without leaving any traces

hidradenitis
Two Types:

Neutrophilic eccrine hidradenitis(NEH)

Idiopathic plantar hidradenitis(recurrent palmoplantar hidradenitis)

A term used to describe diseases in which the histologic abnormality is primarily an inflammatory infiltrate around the eccrine glands

Hidradenitis
neutrophilic eccrine hidradenitis
Neutrophilic Eccrine Hidradenitis

Seen mainly in patients with acute myelogenous leukemia, usually beginning about 10 days after chemotherapy begins

Lesions are typically erythematous and edematous papules and plaques

Lesions are on the extremities, trunk, face(periorbital), and palms (in decreasing frequency)

Fever and neutropenia are often present

Lesions resolve over 1 to 4 weeks

Dapsone may be therapeutic

neutrophilic eccrine hidradenitis1
Neutrophilic Eccrine Hidradenitis
  • There is a sparse, perivascular, and perieccrine infiltrate seen predominantly at the dermal-subcutaneous junction
neutrophilic eccrine hidradenitis2
Neutrophilic Eccrine Hidradenitis
  • Upon closer inspection the inflammatory infiltrate is found to be predominatly around eccrine coils at the dermal-subcutaneous junction
neutrophilic eccrine hidradenitis3
Neutrophilic Eccrine Hidradenitis
  • The infiltrate is mixed but contains neutrophils. The eccrine ducts show focal pallor consistent with early necrosis
recurrent palmoplantar hidradenitis
Recurrent Palmoplantar Hidradenitis

Primarily a disorder of healthy children and young adults

Lesions are primarily painful, subcutaneous nodules on the plantar surface, resembling erythema nodosum

Children may present refusing to walk because of plantar pain

Typically recurrent, and may be triggered by ambulation

nail signs due to abnormal nail matrix function
Beau’s Lines

Pitting

Longitudinal ridging

Longitudinal fissuring

Trachyonchia

True Leukonychia (distal matrix)

Nail Signs due to Abnormal Nail Matrix Function
nail signs due to nail bed disorders
Nail Signs due to Nail Bed Disorders
  • Oncholysis
  • Subungual hyperkeratosis
  • Apparent leukoncychia
  • Splinter hemorrhages
proposed derivations of the adult structures of the nail unit
Proposed derivations of the adult structures of the nail unit
  • Thick arrows indicate epithelial derivations of the matrix
  • Thin arrows show the cornified products they form
onychomadesis
Onychomadesis
  • A periodic idiopathic shedding of the nail beginning at its proximal end
  • Temporary arrest of the function of the nail matrix may also cause this-as may penicillin allergy
  • Neurologic disorders, peritoneal dialysis, mycosis fungoides, and keratosis punctata have been reported ad causes of this type of nail loss
beau s lines
Beau’s Lines
  • Transverse furrows that begin in the matrix and progress distally as the nail grows
  • Caused by the temporary arrest of nail function of the nail matrix
  • They may result from any systemic illness or major injury
  • Shelley’s “shoreline” nails appear to be a very severe expression of essentially the same growth arrest
  • They have been reported in all 20 nails in a newborn
beau s lines1
Beau’s Lines
  • Multiple Beau’s lines caused by intermittent inflammation of the proximal nail fold with resultant injury to the underlying proximal nail matrix
muehrcke s lines
Muehrcke’s Lines
  • Narrow white transverse bands occurring in pairs were described by Muehrcke in 1956 as a sign of chronic hypoalbuminemia
  • Unlike Mees’ lines the disturbance appears to be in the nail bed and not the nail plate
  • Andrew’s believes that this change is caused edema that induces microscopic separation of the normally tightly adherent nail from its bed
mees lines
Mees’ Lines
  • Single or multiple white transverse bands described by Mees in 1919 as a sign of inorganic arsenic poisoning
  • Also have been reported in thallium poisoning, septicemia, dissecting aortic aneurysm, parasitic infections, chemotherapy, and both acute and chronic renal failure
onychorrhexis brittle nails
Onychorrhexis (Brittle Nails)
  • Brittleness with breakage of the nails may result from excessive strong soap and water exposure, from nail polish remover, hypothyroidism, or after oral retinoid therapy
  • It affects up to 20% of the population
  • Fragilitas unguium (nail fragility) is part of this process
  • B-complex vitamin biotin increases the nail thickness by 25% and may help
onychoschizia
Onychoschizia

Splitting of the distal nail plate into layers at the free edge

Is a common problem among women

Represents a dyshesion of the layers of keratin, possibly as a result of dehydration

Longitudinal splits may also occur

Nail polish should not be used

Biotin has been shown to be effective in doses of up to 2.5mg daily and frequent emollients

pitted nails stippled nails
Pitted Nails (Stippled Nails)

Small, pinpoint depressions in an otherwise normal nail

May be an early change seen in psoriasis

Also seen in aa, in early lichen planus, psoriatic arthritis, perforating granuloma annulare, or in individuals with no apparent disease

The pitting in aa tends to be shallower and more regular and has been referred to as a “Scotch plaid” (tartan) pattern

pitted nails stippled nails1
Pitted Nails (Stippled Nails)
  • Coalescence of transverse pits resulting in surface nail plate crumbling in alopecia areata
chevron nail herringbone nail
Chevron Nail (Herringbone Nail)

A rare fingernail ridging pattern of children

The ridges arise from the proximal nail fold and converge in a V-shaped pattern toward a midpoint distally

hapalonychia
Hapalonychia

=Softened nails

It results from a defect in the nail matrix that makes the nails thin and soft so that they can be easily bent

May be due to malnutrtion and debility, myxedema, leprosy, Raynaud’s phenomenon, oral retinoid therapy, or radiodermatitis

platonychia
Platonychia

The nail is abnormally flat and broad

leukonychia or white nails
Leukonychia or White Nails
  • Four forms are recognized
  • Leukonychia punctata
  • Leukonychia striata
  • Leukonychia partialis
  • Leukonychia totalis
the nail in dermatologic diseases
The Nail in Dermatologic Diseases
  • Psoriasis
  • Lichen Planus
  • Twenty Nail Dystrophy (Trachyonychia)
lichen planus of nails
Lichen Planus of Nails

Incidence is <1% to 10%

It may occur without skin changes, but 25% with nail disease will have lichen planus at other locations

Most commonly it begins during fifth or sixth decade

Treatment is unsatisfactory- intralesional injection of corticosteroids, polyethylene occlusive dressings have not been successful

Oral predisone and oral retinoids in combination with topical steroids have been successful in some

Early treatment is mandatory

lichen planus of nails1
Lichen Planus of Nails
  • Nail changes are irregular longitudinal grooving and ridging of the nail plate, thinning of the nail plate, pterygium formation, shedding of the nail plate with atrophy of the nail bed, subungual keratosis, and subungual hyperpigmentation
  • The plate may be markedly thinned, and sometimes the papules of lichen planus may involve the nail plate
psoriatic nails
Psoriatic Nails
  • Nail involvement in psoriasis varies from 10% to 50%
  • There may be pits , or furrows or transverse depressions (Beau’s lines), crumbling nail plate, or leukonychia, with a rough or smooth surface, in the nail plate
psoriatic nails1
Psoriatic Nails
  • In the nail bed splinter hemorrhages are found, reddish discoloration of a part or all of the nail bed, and horny masses
  • In the hyponychium yellowish green discoloration may occur in the area of onycholysis
  • Up to 86.5% of patients with psoriatic arthritis will have psoriatic nail changes
  • Pustular psoriasis may produce onycholysis, with lakes of pus in the nail bed or in the perionychial areas
trachyonchia twenty nail dystrophy
Trachyonchia (Twenty Nail Dystrophy)
  • Nail roughness
  • Thinning
  • Proximal nail matrix damage by alopecia areata, lichen planus, psoriasis
darier s disease
Darier’s Disease
  • Longitudinal, subungual, red or white streaks, associated with distal wedge-shaped subungual keratoses, are the nail signs diagnostic for Darier-White disease
  • Characteristic is V-shaped nicking, linear striations, onycholysis, and subungual keratotic reaction
terry s nails
Terry’s Nails
  • The distal 1 to 2 mm of the nail shows a normal pink color
  • The entire nail plate or proximal end has a white appearance as a result of changes in the nail bed
  • These changes have been noted in patients with cirrhosis, chronic congestive heart failure, and adult onset diabetes, and the very elderly
half and half nails
Half and Half Nails
  • These shoe the proximal portion of the nail white and the distal half red, pink, or brown, with a sharp line of demarcation between the two halves
  • Found in patients with renal disease associated with azotemia
slide93
Permanently dystrophic nails secondary to matrix scarring in a patient with Stevens-Johnson syndrome
racquet nails nail en raquette
Racquet Nails (Nail en Raquette)

The end of the thumb is widened and flattened

The nail plate is flattened as well

The distal phalanx is abnormally short

They occur on one or both thumbs

Inherited as AD trait

nail patella syndrome
Nail-Patella Syndrome

Comprises numerous anomalies

Characterized by the absence or hypoplsia of the patella and congenital nail dystrophy

Triangular lunulae are characteristic

nail patella syndrome1
Nail-Patella Syndrome

Other bone features:

Thickened scapulae, hyperextensible joints, radial head abnormalities, and posterior iliac horns

Skin changes may include webbing of the elbows

Eye changes such as cataracts and heterochromia of the iris

Hyperpigmentation of the pupillary margin of the iris (“Lester iris”) is a characteristic finding in 50% of cases

nail patella syndrome2
Nail-Patella Syndrome
  • Glomerulonephritis with albuminuria, hematuria, and a variety of casts –hyaline casts
  • 40% have renal dysplasia
  • 25% suffer from renal failure
  • AD trait localized to chromosome 9q34.1
  • Micronychia, triangular lunulae, and clinodactyly
anonychia
Anonychia
  • Absence of nails
  • A rare anomaly
  • May be the result of a congenital ectodermal defect, ichthyosis, severe infection, severe allergic contact dermatitis, self inflicted trauma, Raynaud’s phenomenon, lichen planus, or severe exfoliative diseases
  • Permanent anonychia has been reported as a sequel of Stevens-Johnson syndrome
anonychia1
Anonychia
  • May also be found in association with congenital developmental abnormalities such as microcephaly, and wide-spaced teeth(AR)
  • Or Cook’s syndrome(AD)- bilateral nail hypoplasia of digits 1 through 3, the absence of nails of digits 4 and 5 of the hands, total absence of all the toenails, and absence or hypoplasia of the distal phalanges of the hands and feet
onychoatrophy
Onychoatrophy
  • Faulty underdevelopment of the nail that may be congenital or acquired
  • The nail is thinner and smaller
  • A side effect of etretinate therapy
  • Also may be seen in vascular disturbances, epidermolysis bullosa, lichen planus, Darier’s disease, multicentric reticulohistiocytosis, and leprosy
  • It is also seen in the nail-patella syndrome
slide102
Permanently dystrophic nails secondary to matrix scarring in a patient with Stevens-Johnson syndrome
tumors of the nail
Signs heralding such neoplasms=

Paronychia, ingrown nail, onycholysis, pyogenic granuloma, nail plate dystrophy, bleeding, and discolorations

Symptoms of pain, itching, and throbbing may also be seen

Begin tumors of the nail include: verruca, pyogenic granuloma, fibromas, nevus cell nevi, myxoid cysts, angiofibromas (Koenen’s tumors), and epidermoid cysts

Pyogenic granuloma-like lesions may occur during treatment with isotretinoin or indinavir

Tumors of the Nail
tumors of the nail1
Tumors of the Nail
  • Pyogenic granuloma after trauma to the lateral nail fold
tumors of the nail2
Tumors of the Nail
  • Pyogenic granuloma of the nail bed mimicking hematoma or melanoma
tumors of the nail3
Tumors of the Nail
  • Pyogenic granuloma formation secondary to chronic irritation from a genetic subungual exostosis
tumors of the nail4
Tumors of the Nail
  • Advanced pyogenic granuloma formation resulting from long-standing pressure and low-grade infection
tumors
Tumors
  • Glomus tumor with a bluish hue in the distal nail bed
  • Maybe painful
tumors1
Tumors
  • Mucous cyst in its most common location exerting pressure on the matrix, resulting in a grooved nail plate
tumors2
Tumors
  • Normal finger contour is altered by focal mucinosis ( a myxoid cyst), which presses against the proximal nail matrix and results in a groove in the nail plate.
tumors3
Tumors
  • Giant cell tumor of the tendon sheath presenting lateral to the usual location on the dorsum of the digit
tumors of the nail5
Tumors of the nail
  • Advanced pyogenic granuloma formation resulting from long-standing pressure and low-grade infection
genetic subungual exostosis
Genetic Subungual Exostosis
  • Dome-shaped genetic

Subungual exostosis

A, Lateral projection

B, Dorsoplantar projection

exostosis enchondroma osteochondroma epidermoid cyst
Exostosis, Enchondroma, Osteochondroma, Epidermoid Cyst

Four benign tumors of the subungual region that involve the bone first and the nail plate second

X-ray films of these lesions should be taken first, before biopsy, because the appearance may be characteristic enough to allow for definitive surgery initially

genetic subungual exostosis1
Genetic Subungual Exostosis
  • A, Tabletop genetic subungual exostosis, hallux lateral projection
  • B, Dorsiplantar projection. Arrowheads indicate the medial origin of the exostosis, extending medial to the shaft
subungual exostosis
Subungual Exostosis
  • The growing genentic subungual exostosiss has caused expansion and tightening of the periungual skin distally and medially
genetic subungual exostosis2
Genetic Subungual Exostosis
  • Subungual nail plate hemorrhage secondary to nail plate elevation.
  • Traumatic implantation of nail matrix into nail bed is also seen
  • There are nail plate inclusions in the dermis
  • There is no granular laayer
subungual exostosis1
Subungual Exostosis
  • Genetic subungual exostosis exerts distal pressure on the hyponychium, promoting fibrosis and implantation of epidermis into dermis
  • Note kerrratinizing epidermal inclusion developing with the benefit of a granular layer, surrounded by fibrosis of the the hyponychium
premalignant and transitional tumors
Premalignant and Transitional Tumors
  • Bowen’s disease of the lateral nail groove with minimal clinical changes for 2 yrs
  • Etiologic factors: chronic infection, trauma, HPV
  • Seen more commonly in HIV infected individuals
  • Seeing an increase in Bowen’s disease in nail bed over the past decade
premalignant and transitional tumors1
Premalignant and Transitional Tumors
  • Squamous cell carcinoma
  • Actinic keratosis
  • Keratoacanthoma
  • Bowen’s disease
bowen s disease
Bowen’s Disease
  • Bowen’s disease masking as a pigmented streak along the lateral nail groove, clinically suspected of being a melanoma
bowen s disease1
Bowen’s Disease
  • Histology of the lesion prior showing cellular atypia, mitoses, and individual cell keratinization
  • The nail plate intervenes between the nail fold on top and the nail bed on the bottom in this tangential cut
subungual melanoma
Subungual Melanoma
  • Frequently diagnosed late in the course or growth, since it simulates onychomycosis or subungual hematoma
  • Amelanotic melanoma may occurand nay be mistaken for pyogenicum
  • More frequently found in Japanese than in other ethnic populations
  • Example is in vertical growth phase showing nodule formation, nail destruction, and faint Hutchinson’s sign of proximal nail fold
subungual melanoma1
Subungual Melanoma
  • Histology of acral lentiginous melanoma showing giant dendritic melanocytes with atypia
traumatic nail abnormalities
Traumatic Nail Abnormalities
  • Onychotillomania
  • Subungual Hematoma
  • Onychgryphosis
  • Pincer Nails
  • Ingrown Toenail (Onychocryptosis)
median nail dystrophy
Median Nail Dystrophy
  • AKA dystrophia unguis mediana canaliformis or solennonychia
  • Consists of longitudinal splitting or canal formation in the midline of the nail
  • The split, which often resembles a fir tree, occurs at the cuticle and proceeds outward as the nail grows
  • Trauma has been implicated, but not proven
  • A papilloma in the nail matrix forcing a tube(solenos) like structure distal to it has also been suspected
onychotillomania
Onychotillomania
  • A compulsive neurosis in which the patient picks constantly at the nails or tries to tear them of
onychogryphosis
Onychogryphosis
  • Hypertrophy may produce nails resembling claws or a ram’s horn
  • May be caused by trauma or peripheral vascular disease, or more commonly by neglect
  • Most commonly seen in the elderly
  • If the blood supply is adequate avulsion of the nail with surgical destruction of the matrix is recommened by some
onychogryphosis1
Onychogryphosis
  • Characterized by an opaque, thickened nail plate with subungual hyperkeratosis and transverse striations in which there has been exaggerated growth in an upward and lateral direction
onychogryphosis2
Onychogryphosis
  • Is an exaggerated enlargement of the nail plate.
  • Most often involves the great toenail only
  • Self-neglect is the most common cause
onychophosis
Onychophosis
  • A common finding in the elderly
  • It is a localized or diffuse hyperkeratotoic tissue that develops on the lateral or proximal nailfolds, within the space between the nailfolds and the nail plate
  • May involve the subungual area as a result of repeated trauma
  • Most frequently involves the first and fifth toes
  • Encourage the use of comfortable shoes
  • Involved areas should be debrided and treated with keratolytics
  • Emollients are also helpful
  • Precautionary measure to prevent this =wearing comfortable shoes and relieving any pressure exerted by the nail on soft tissues
onycholysis
Onycholysis
  • Spontaneous separation of the nail plate
  • Usually beginning at the free margin and progressingg proxximally
  • Rarely the lateral borders may be involved
  • Less often separation may begin proximal to the free edge, in an oval area 2 to 6 mm broad, with a yellowish brown hue (“oil spot”) this is psoriasis
onycholysis1
Onycholysis
  • The nail itself is smooth and firm
  • Underneath the nail a discoloration may occur as a result of accumulation of bacteria or yeast
  • Mostly seen in women secondary to trauma and subsequent infection with Candida
  • Systemic causes: hyperthyroidism, hypothyroidism, pregnancy, pellagra, syphillis, trauma, vaginal yeast infections with secondary spread
  • Chemical causes: solvents, nail polish base coat, artificial fingernails
  • Photoonycholysis may occur during or soon after therapy with tetracycline derivatives, psoralens, fluoroquinolones, or chloramphenicol, with subsequent exposure to light
  • Chemotherapeutic agents like mitoxantrone, may also precipitate onycholysis
  • May rarely be a sign of distal metastasis
  • May be inherited as AD trait
management
Management
  • Avoid trauma
  • Keep nail bed dry
  • Trim affected portion of the nail
onychocryptosis
Onychocryptosis
  • Aka unguis incarnatus; ingrown nail
  • One of the most frequent nail complaimts
  • Chiefly occurs on the great toe
  • Leads to excessive lateral nail growth into the nail fold, leading to PAIN and inflammation
  • Causes are: improper fitting shoes, improper nail trimming at the lateral edges so that the anterior portion cuts int the flesh as it grows distally
onychocryptosis1
Onychocryptosis
  • Rather than remove the nail a Jansey operation is frequently successful
  • This involves removing the overhanging lateral nail fold so that the nail does not cut into it
  • When healed the nail edges resembles the thumb and is very functional
  • The nail is not altered
slide138
Recurrent onychocryptosis with subsequent periungual inflammation and granulation tissue.
slide139
Partial regrowth of normal nail after previous nail plate avulsion for onychocryptosis secondary to severe overcurvature of the nail plate with painful contriction of the nail plabed (pincer nails)
slide140
Recurrent onychocryptosis with subsequent periungual inflammation and granulation tissue
jansey operation
Jansey operation

Under local anesthesia and using a rubber band tourniquet at the base of the toe, (A), a linear incision is madeparallel to nail margin, (B), A convex incision is made in a curvilinear plane parallel to the nail bed meeting the initial incision

(C), involved tissue is removed, (E), lateral flap is then approximated by 1 –2 sutures

treatment
Treatment
  • Another procedure is to apply saturated solution of phenol to the nail matrix after a portion of the ingrown nail has been removed surgically (phenolization)
  • Objective here is to permanently ablate the part of the nail matrix producing the nail that was ingrowing
  • Between 60% & 80% of patients treated with partial or complete nail plate avulsion have a recurrence
  • In mild cases, insertion of a cotton pas beneath the distal corner of the nail may resolve the problem
  • A flexible plastic tube to splint the nail is useful, the nail may be flattened through the use of a stainless steel wire brace
  • The brace fits the over-curved nail exactly and maintains constant tension over the nail
  • Adjustments are made over a period of 6 months
  • Liquid nitrogen spray to the area for 20 to 30 sec may be successful
leukonychia or white nails1
Leukonychia or White Nails
  • Four forms are recognized
  • Leukonychia punctata
  • Leukonychia striata
  • Leukonychia partialis
  • Leukonychia totalis
pterygium unguis
Pterygium Unguis
  • An abnormal extension of the cuticle over the proximal nail plate
  • Classic example is lichen planus
  • Has been reported to occur in sarcoidosis and Hansen’s disease and peripheral circulatory disturbances
pterygium inversum unguis
Pterygium Inversum Unguis
  • Characterized by adherence of the distal portion of the nail bed to the ventra lsurface of the nail plate
  • Presents at birth or is acquired and may be painful when manipulating small objects
  • Results from the extension of the zone of the nail plate, leading eventually to a more ventral and distal extension of the hyponychium
  • Most commonlysee is the secondary or acquired form caused by connective tissue disease
hangnail
Hangnail
  • An overextension of the eponychium (cuticle)
  • The cuticle becomes split and peels away from the proximal or lateral nail fold
  • Lesions are painful
  • Trim with a scissors and use emollient creams to keep the cuticle soft
pincer nails
Pincer Nails
  • AKA trumpet nails or omega
  • Common toenail disorder
  • Lateral edges of the nail slowly approach one another, compressing the nail bed and underlying dermis
  • May occur in the fingernails and is then usually asymptomatic
  • Treatment is use of commercial plastic braces after flattening the nail
  • Urea ointment under occlusion has been reported to be helpful
onychophagia
Difficult habit to cure

May apply dimethyl sulfoxide (DMSO) every day or two as a mild deterrent

Psychopharmacologic intervension may be required

Nail biting

A common compulsive behavior

May markedly shorten the nail bed

Sometimes damage the matrix and lead to pterygium formation

Onychophagia