Andrew

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Andrews Chapter 33-Part II

• David M. Bracciano D.O.

2
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

3
Folliculitis Decalvans

• 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

4
Folliculitis Decalvans

• 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

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Folliculitis Decalvans

• Early lesions show perifollicular inflammation
  composed of acute and chronic inflammatory cells

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Folliculitis Decalvans

• Later lesions shows destruction of follicular
  epithelium, dense interstitial inflammation, and
  perifollicular fibrosis, with free hairs in the
  tissue

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Folliculitis Decalvans

• There is perifollicular and interstitial fibrosis
  accompanied by an interstitial infiltrate of
  lymphocytes and plasma cells

8
Folliculitis Decalvans

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

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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

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Tinea Amiantacea

• 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

11
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 Bakers PS liquid is
  helpful to remove scale and crust
• Derma-Smoothe and FS shampoo are also effective

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Keratosis Follicularis Contagiosa

• Also known as epidemic acne, epidemic follicular
  eruption, epidemic follicular keratosis, and
  Brookes disease
• Unknown etiology
• Occurs in children

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Keratosis Follicularis Contagiosa

• 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

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Keratosis Follicularis Contagiosa

• 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

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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

16
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

17
Perforating Folliculitis

• 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.

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Perforating Folliculitis

• Resistant to treatment
• Topical tretinoin is reported to be effective
• One report of an HIV-infected man who responded
  well to thalidomide

19
Kyrles 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

20
Kyrles Disease

• There is a prediliction for the lower
  extremities, but the upper extremities, head, and
  neck may be involved
• Koebners 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

21
Kyrles Disease

• Occurs almost exclusively in adults ages 20 to 63
• No sex or racial differences seen
• Has been associated with diabetes mellitus

22
Kyrles Disease

• 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

23
Kyrles Disease

• Kyrles 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

24
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

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Reactive Perforating Collagenosis

• A cup-shaped channel containing degereated
  collagen bundles and basophilic material

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Reactive Perforating Collagenosis

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

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Reactive Perforating Collagenosis

• 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

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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

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Perforating Disease of Hemodialysis

• The disease may remit promptly after a renal
  transplant and stopping dialysis
• Topical tretinoin may be tried

30
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

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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

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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

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Disseminate and Recurrent Infundibulofolliculitis

• 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

34
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

35
Lichen Spinulosus

• 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

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Lichen Spinulosus
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Lichen Spinulosus

• 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

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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

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Hyperhidrosis

• 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(Pancoasts tumor) or
  postoperatively), sensory neuropathy (diabetes
  mellitus or subsequent to zoster), parotitis or
  parotid abscess, and surgery of the parotid gland

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Hyperhidrosis

• Other Localized Forms of Hyperhidrosis
• Localized sweating can occur over lesions of
  blue rubber bleb nevus, glomus tumors,
  hemangiomas and in POEMS syndrome, Gopalans
  syndrome, complex regional pain syndrome, and as
  a result of spinal cord tumors

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Hyperhidrosis

• 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, Parkinsons
  disease, pheochromocytoma, hypogycemia,
  salicylism, and lymphoma, and metastatic tumors
  transecting the spinal cord

42
Hyperhidrosis

• 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

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Anhidrosis absence of sweating

• May be localized or generalized
• Generalized anhidrosis occurs in anhidrotic
  ectodermal dysplasia, quinacrine anhidrosis,
  miliaria profunda, Sjogrens 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

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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

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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

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Bromidrosis

• 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

• 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

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Chromhidrosis

• 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

• 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

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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

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Fox-Fordyce Disease
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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

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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

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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

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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

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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

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Neutrophilic Eccrine Hidradenitis

• There is a sparse, perivascular, and perieccrine
  infiltrate seen predominantly at the
  dermal-subcutaneous junction

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Neutrophilic Eccrine Hidradenitis

• Upon closer inspection the inflammatory
  infiltrate is found to be predominatly around
  eccrine coils at the dermal-subcutaneous junction
  

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Neutrophilic Eccrine Hidradenitis

• The infiltrate is mixed but contains neutrophils.
  The eccrine ducts show focal pallor consistent
  with early necrosis

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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

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Sagittal view of nail unit
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Nail Signs due to Abnormal Nail Matrix Function

• Beaus Lines
• Pitting
• Longitudinal ridging
• Longitudinal fissuring
• Trachyonchia
• True Leukonychia (distal matrix)

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Nail Signs due to Proximal and Distal Matrix
function

• Onychomadesis
• Koilonychia
• Nail thinning

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Nail Signs due to Nail Bed Disorders

• Oncholysis
• Subungual hyperkeratosis
• Apparent leukoncychia
• Splinter hemorrhages

65
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

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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

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Beaus 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
• Shelleys 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

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Beaus Lines

• Multiple Beaus lines caused by intermittent
  inflammation of the proximal nail fold with
  resultant injury to the underlying proximal nail
  matrix

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Muehrckes 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
• Andrews believes that this change is caused
  edema that induces microscopic separation of the
  normally tightly adherent nail from its bed

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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

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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

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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

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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

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Pitted Nails (Stippled Nails)

• Coalescence of transverse pits resulting in
  surface nail plate crumbling in alopecia areata

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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

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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, Raynauds phenomenon, oral retinoid
  therapy, or radiodermatitis

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Platonychia

• The nail is abnormally flat and broad

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Leukonychia or White Nails

• Four forms are recognized
• Leukonychia punctata
• Leukonychia striata
• Leukonychia partialis
• Leukonychia totalis

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The Nail in Dermatologic Diseases

• Psoriasis
• Lichen Planus
• Twenty Nail Dystrophy (Trachyonychia)

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Lichen Planus of Nails

• Incidence is lt1 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

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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

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Nail Lichen Planus
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Lichen Planus
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Psoriatic Nails

• Nail involvement in psoriasis varies from 10 to
  50
• There may be pits , or furrows or transverse
  depressions (Beaus lines), crumbling nail plate,
  or leukonychia, with a rough or smooth surface,
  in the nail plate

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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

88
Trachyonchia (Twenty Nail Dystrophy)

• Nail roughness
• Thinning
• Proximal nail matrix damage by alopecia areata,
  lichen planus, psoriasis

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Dariers 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

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Dariers disease (longitudinal red and white
streaks)
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Terrys 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

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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

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• Permanently dystrophic nails secondary to matrix
  scarring in a patient with Stevens-Johnson
  syndrome

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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

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Nail-Patella Syndrome

• Comprises numerous anomalies
• Characterized by the absence or hypoplsia of the
  patella and congenital nail dystrophy
• Triangular lunulae are characteristic

96
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

97
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

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• Triangular lunulae of nail-patella syndrome

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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, Raynauds phenomenon, lichen planus, or
  severe exfoliative diseases
• Permanent anonychia has been reported as a sequel
  of Stevens-Johnson syndrome

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Anonychia

• May also be found in association with congenital
  developmental abnormalities such as microcephaly,
  and wide-spaced teeth(AR)
• Or Cooks 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

101
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, Dariers
  disease, multicentric reticulohistiocytosis, and
  leprosy
• It is also seen in the nail-patella syndrome

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• Permanently dystrophic nails secondary to matrix
  scarring in a patient with Stevens-Johnson
  syndrome

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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 (Koenens tumors),
  and epidermoid cysts
• Pyogenic granuloma-like lesions may occur during
  treatment with isotretinoin or indinavir

104
Tumors of the Nail

• Pyogenic granuloma after trauma to the lateral
  nail fold

105
Tumors of the Nail

• Pyogenic granuloma of the nail bed mimicking
  hematoma or melanoma

106
Tumors of the Nail

• Pyogenic granuloma formation secondary to chronic
  irritation from a genetic subungual exostosis

107
Tumors of the Nail

• Advanced pyogenic granuloma formation resulting
  from long-standing pressure and low-grade
  infection

108
Tumors

• Glomus tumor with a bluish hue in the distal nail
  bed
• Maybe painful

109
Tumors

• Mucous cyst in its most common location exerting
  pressure on the matrix, resulting in a grooved
  nail plate

110
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.

111
Tumors

• Giant cell tumor of the tendon sheath presenting
  lateral to the usual location on the dorsum of
  the digit

112
Tumors of the nail

• Advanced pyogenic granuloma formation resulting
  from long-standing pressure and low-grade
  infection

113
Genetic Subungual Exostosis

• Dome-shaped genetic
• Subungual exostosis
• A, Lateral projection
• B, Dorsoplantar projection

114
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

115
Differential Diagnosis
116
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

117
Subungual Exostosis

• The growing genentic subungual exostosiss has
  caused expansion and tightening of the
  periungual skin distally and medially

118
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

119
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

120
Premalignant and Transitional Tumors

• Bowens 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 Bowens disease in nail bed
  over the past decade

121
Premalignant and Transitional Tumors

• Squamous cell carcinoma
• Actinic keratosis
• Keratoacanthoma
• Bowens disease

122
Bowens Disease

• Bowens disease masking as a pigmented streak
  along the lateral nail groove, clinically
  suspected of being a melanoma

123
Bowens 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

124
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
  Hutchinsons sign of proximal nail fold

125
Subungual Melanoma

• Histology of acral lentiginous melanoma showing
  giant dendritic melanocytes with atypia

126
Traumatic Nail Abnormalities

• Onychotillomania
• Subungual Hematoma
• Onychgryphosis
• Pincer Nails
• Ingrown Toenail (Onychocryptosis)

127
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

128
Onychotillomania

• A compulsive neurosis in which the patient picks
  constantly at the nails or tries to tear them of

129
Onychogryphosis

• Hypertrophy may produce nails resembling claws or
  a rams 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

130
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

131
Onychogryphosis

• Is an exaggerated enlargement of the nail plate.
• Most often involves the great toenail only
• Self-neglect is the most common cause

132
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

133
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

134
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

135
Management

• Avoid trauma
• Keep nail bed dry
• Trim affected portion of the nail

136
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

137
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

138

• Recurrent onychocryptosis with subsequent
  periungual inflammation and granulation tissue.

139

• 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)

140

• Recurrent onychocryptosis with subsequent
  periungual inflammation and granulation tissue

141
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

142
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

143
Leukonychia or White Nails

• Four forms are recognized
• Leukonychia punctata
• Leukonychia striata
• Leukonychia partialis
• Leukonychia totalis

144
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
  Hansens disease and peripheral circulatory
  disturbances

145
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

146
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

147
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

148
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149
Onychophagia

• Nail biting
• A common compulsive behavior
• May markedly shorten the nail bed
• Sometimes damage the matrix and lead to pterygium
  formation

• Difficult habit to cure
• May apply dimethyl sulfoxide (DMSO) every day or
  two as a mild deterrent
• Psychopharmacologic intervension may be required