Andrews Diseases of the SkinChapter 10pg 239253

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Andrews Diseases of the Skin-Chapter 10-pg
239-253 Chapter 11

• Boris Ioffe, D.O.

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Recalcitrant Palmoplantar Eruptions

• Recalcitrant pustular eruptions of the hands and
  feet are often examples of psoriasis
  
• Need to then search for lesions elsewhere on the
  body(e.g., scalp, ears, glans penis)
  
• Search also for a family history to confirm your
  suspicion

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Dermatitis Repens

• Aka- acrodermatitis continua and acrodermatits
  perstans
  
• Its a chronic inflammatory disease of hands and
  feet
  
• Rarely, can become generalized
• Usually, as a pustule or paronychia

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Dermatitis Repens

• Occasionally, mucous membranes are involved
• Nails are often dystrophic or destroyed
• Lesions cause skin atrophy
• Crusted, eczematoid, and psoriasiform lesions may
  occur, and there may be moderate itching
  
• It is essentially unilateral in its beginning and
  asymmetrical throughout its entire course
  

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Dermatitis Repens

• Histology
• similar to those seen in psoriasis
• the primary lesion is epidermal
• An intraepithelial spongiform pustule is formed
  by infiltration of pmns
  
• Treatment
• topical mechlorethamine, topical steroids, PUVA,
  fluorouracil, and sulfapyridine
  
• Acitretin, low dose cyclosporine, Acitretin plus
  calcipotriol

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Palmoplantar Pustulosis

• AKA pustular psoriasis
• In contrast to dermatitis repens it is
  essentially bilateral and symmetrical
  
• Locations include thenar/hypothenar eminences or
  central portion of the palms and soles

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Palmoplantar Pustulosis

• In the course of a week, they tend to dry up,
  leaving punctate brown scabs that eventually
  exfoliate
  
• Stages of quiescence and exacerbation
  characterize the condition
  
• Meds, such as lithium, have been reported to
  induce

• Patches begin as erythematous areas in which
  pustules form
  
• Start as pinhead-sized, enlarge and coalesce to
  form small lakes of pus

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Palmoplantar Pustulosis

• Nails may become malformed, ridged, stippled,
  pitted and discolored
  
• May be associated with psoriasis vulgaris
• Some regard palmoplantar pustulosis as a form of
  psoriasis, while others consider it a separate
  entity
  
• Female predominance lack of seasonal variation
  different histopathologic features and
  
• Associated with thyroid disorders and cigarette
  smoking

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Palmoplantar Pustulosis

• May be predisposed to joint disease and possibly
  SAPHO syndrome-Synovitis, Acne, Pustulosis,
  Hyperostosis and Osteoarthritis
  
• Its resistant to most treatments

• Acitretin is reportedly effective(1mg/kg/day)
• Low-dose cyclosporine (1.25mg/kg/day-3.75mg/kg/day
  )
  
• Intramuscular Kenalog (40-60mg)may be effective
  for short-term relief
  

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Palmoplantar Pustulosis
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Pustular Bacterid

• Characterized by a symmetric, grouped, vesicular
  or pustular eruption on palms and soles
  
• Marked by exacerbations and remissions over long
  periods
  
• No involvement of webs of fingers or toes or
  flexion creases of toes
  
• WBC may be elevated
• Scaling is usually present
• Etiology is thought to be a remote focus of
  infection infection needs to be treated before
  resolution will occur

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Juvenile Plantar Dermatosis

• Usually begins as a patchy, symmetrical, smooth,
  red, glazed macule on great toes, sometimes with
  fissuring and desquamation in children aged 3-13
  
• Toe webs are rarely involved fingers may be
• Histologically, there is psoriasiform acanthosis
  and a sparse, lymphocytic infiltrate in the upper
  dermis
  
• Spongiosis is commonly present
• Tx bed rest, cotton socks and topical steroids
• Spontaneous resolution within 4 yrs is the rule

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Infantile Acropustulosis

• Intensely itchy vesicopustular eruption of hands
  and feet
  
• Begins at any age up to 10 months, clearing in a
  few weeks and recurring repeatedly until final
  resolution at 6 36 months of age
  
• Dapsone at 2mg/kg/day may help
• Potent topical steroids aid in symptomatic relief

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Infantile Acropustulosis

• Should prompt an extensive workup to eliminate
  serious infectious causes (i.e., Tzanck prep,
  gram stain, KOH prep of pustule)
  
• Some suspect that this condition may be a
  persistent reaction to prior scabies

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• Acropustulosis of infancy

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Pompholyx

• AKA dyshidrosis
• A vesicular eruption of palms and soles
  characterized by spongiotic intraepidermal
  vesicles and often accompanied by burning or
  itching
• Hyperhidrosis may be present
• Usually bilateral and symmetrical
• Bullae may form
• Contents are clear and colorless
• Attacks generally last a few weeks
• Lesions dry-up and desquamate rather than rupture

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Pomphylox

• Etiology- stress, atopy, and topical as well as
  ingested contactants
  
• Histopathology spongiotic vesicles in the
  epidermis
  
• Differential dx
• dermatophytid, contact dermatitis, atopic
  dermatitis, drug eruption, pustular psoriasis of
  palms and soles, acrodermatitis continua, and
  pustular bacterid
• Rarely, T-cell lymphoma can present with similar
  clinical findings, but biopsy of the vesicles
  will be diagnostic
  

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Pomphylox

• Tx high potency corticosteroid creams
• Triamcinolone acetonide intramuscularly or a
  short course of oral prednisone is rapidly
  effective
  
• Oral or topical psoralen UVA (PUVA) is
  effective but costly inconvenient
  
• In more severe forms, immunosuppressive
  mycophenolate mofetil has been effective
  

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Lamellar Dyshidrosis

• AKA dyshidrosis lamellosa, keratolysis
  exfoliativa
  
• A superficial exfoliative dermatosis of the palms
  and sometimes soles
  
• Referred to as recurrent palmar peeling

• Involvement is bilateral
• Can occur in association with dyshidrosis
• Often exacerbated by environmental factors
• Differential dx dermatophytosis, chronic contact
  dermatitis

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Lamellar Dyshidrosis

• Tx difficult
• Spontaneous involution can occur in a few weeks
  for some
  
• Most tends to be chronic and relapsing
• Tar creams (Zetone cream) usually helps
• 5 tar in gel (Estar Gel) is an excellent tx
• Lac-Hydrin lotion and Carmol 10 or 20 are often
  effective
  
• NB-UVB may be helpful

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Lamellar Dyshidrosis
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Palmoplantar Keratoderma

• AKA tylosis, keratosis, hyperkeratosis
• Characterized by excessive formation of keratin
  on the palms and soles
  
• Acquired
• Keratosis Punctata of the Palmar Creases
• Punctate Keratoses of the Palms and Soles
• Porokeratosis Plantaris Discreta
• Keratoderma Climactericum
• Congenital

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Punctate Keratosis of the Palms and Soles

• Primary lesion is a 1-5mm round to oval,
  dome-shaped papule distributed over left hand and
  hypothenar eminence
  
• Main symptom is pruritis
• Lesions number from 1 to 40
• Affects mainly blacks
• Theres a potential risk of developing lung and
  colon cancer

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Punctate Keratosis of the Palms and Soles
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Keratosis Punctata of the Palmar Creases

• Common most often in black pts
• Primary lesion is a 1-5mm depression filled with
  a conical keratinous plug
  
• Primarily, in creases of palms or fingers,
  occasionally in soles
  
• Lesions are multiple
• Friction aggravates lesions causing them to
  become verrucoid or surrounded by callus

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• Punctate keratoses of the palmar creases in an
  African-American
  
• PPPK-punctate palmoplantar keratoderma

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Porokeratosis Plantaris Discreta

• Occurs in adults, FemaleMale (41)
• Characterized by sharply marginated, rubbery,
  wide-based papule that does not bleed on removal
  
• Lesions are multiple, painful, 7-10mm in
  diameter
  
• Usually on wt bearing areas of sole, beneath
  metatarsal heads
  
• Tx foot pads to redistribute wt, surgical
  excision, blunt dissection

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Keratoderma Climactericum

• Characterized by hyperkeratosis of palms and
  soles beginning at about the time of menopause
  
• Descrete, thickened, hyperkeratotic patches most
  pronounced at pressure sites
  
• Fissuring may be present
• Tx keratolytics -- 10 salicylic acid, lactic
  acid creams, etc.

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Hereditary syndromes

• These have palmoplantar keratoderma as a feature
• Unna-Thost
• Papillon-Leferve

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Unna Thost

• Dominant inheritance congenital thickening of
  epidermal horny layer of the palms and soles
  
• Usually symmetrical
• Epidermis becomes thick, yellowish, verrucous,
  and horny
  
• Striate and punctate forms occur

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Unna Thost

• Occasionally nails become thickened
• 5 salicylic acid may help
• Lac Hydrin 12 may be tried
• Acitretrin or isotretinoin may be considered, but
  need for lifetime tx makes them impractical

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• Focal palmoplantar keratosis of the striate type
  on the sole

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• Diffuse non-epidermolytic palmoplantar keratosis

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• Diffuse epidermolytic palmoplantar keratosis with
  diffuse hyperkeratosis

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Papillon-Lefevre Syndrome

• Palmoplantar hyperkeratosis with peridontosis
• Usually develops within the first few months of
  life but may occur in childhood
  
• Well demarcated, erythematous, hyperkeratotic
  lesions on palms and soles
  
• Transverse grooves of fingernails may occur

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Papillon-Lefevre Syndrome

• Early onset peridontal disease has been
  attributed to damage and alteration in PMN
  function caused by Actinomyces actinomycetemcomita
  ns
• Disease associations include acroosteolysis, and
  pyogenic liver abcesses

• There are asymptomatic ectopic calcifications in
  the choroid plexus and tentorium
  
• Therapy may retard both dental and skin
  abnormalities
  
• Treatment with Acitretin in four siblings was
  reported to be effective
  

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• Papillon-Lefevre syndrome plantar keratoderma

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Mutilating Keratoderma of Vohwinkel

• Palmoplantar hyperkeratosis of the honeycomb
  type-associated with starfish-like keratosis on
  backs of hands and feet linear keratoses of the
  elbows and knees, and annular constriction
  (pseudo-ainhum) of the digits, this may progress
  to autoamputation
• More than 30 cases have been reported world-wide
• More common in women and in whites
• Onset is in infancy or early childhood

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• Vohwinkels mutilating syndrome A.) diffuse
  keratoderma of palms with B.) pseudoaainhum
  formation

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Palmoplantar Keratodermas Malignancy

• Diffuse, waxy keratoderma of palms and soles
  occurring as an AD trait associated with
  esophageal carcinoma
  
• Other related factors are oral leukoplakia,
  esophageal srictures, squamous carcinoma of
  tylotic skin, carcinoma of larynx and stomach
  
• Acquired forms of palmoplantar keratodermas have
  also been associated with carcinoma of esophagus,
  lung, breast, bladder and stomach

46

• Focal PPK in association with carcinoma of the
  esophagus

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Acrokeratoelastoidosis of Costa

• AD, more common in women
• Small, round, firm papules occurring over dorsal
  hands, knuckles, and lateral margins of palms and
  soles
  
• Appears in early childhood and progress slowly
• Most often asymptomatic
• Significant histologic finding is dermal
  elastorrhexis
  
• Therapies liquid nitrogen, salicylic acid,
  tretinoin, and prednisone have been tried

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• Focal acrokeratoelastoides multiple
  skin-colored papules at the margin of the palmar
  skin

49

• Path non-epidermolytic palmoplantar keratosis,
  acanthosis and hypergranulosis

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Exfoliative Dermatitis

• Universal or very extensive scaling and itching
  erythroderma
  
• Often associated with hair loss
• Initially with erythematous plaques, which spread
  rapidly
  
• Onset accompanied by general toxicity
• Skin becomes scarlet and swollen and may ooze a
  straw-colored exudate
  
• Desquamation is evident within a few days

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Etiology

• Most common is preexisting dermatoses (53)
• atopic dermatitis, chronic actinic dermatitis,
  psoriasis,seborrheic dermatitis, vesicular
  palmoplantar eczema, pityriasis rubra pilaris,
  and contact dermatitis
• Drug eruptions(5)
• allopurinol, gold, carbamazepine, phenytoin, and
  quinidine
  
• Cutaneous T-cell lymphoma(13) Sezary syndrome
  and mycosis fungoides

• Paraneoplstic (2) carcinoma of the lung and
  carcinoma of the stomach
  
• Leukemia cutis (1)
• Idiopathic (26)
• Mortality rate at a mean follow-up interval of 51
  months was 43

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Histology

• Most commonly, histology is nonspecific
• Hyperkeratosis focal parakeratosis
• Epidermis shows mild acanthosis, scant
  superficial upper dermal infiltrate of
  mononuclear cells
  
• May be small areas of spongiosis

53

• Generalization after withdrawal of methotrexate

54

• Exfoliation of scale with underlying erythema

55

• Generalized erythema with thick scale and crusted
  fissures on the plantar surface

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Treatment

• Topical steroids, soaks, and compresses
• Acitretin and cyclosporin-useful in psoriatic
  erythroderma, and isotretinoin in erythroderma
  caused by RPR methotrexate
  
• Systemic corticosteroids in severe cases
• Discontinuing the offending drug in drug-induced
  cases

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• Subungual hyperkeratosis and distal dystrophy

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Parapsoriasis, PityriasisRosea, Pityriasis Rubra
Pilaris
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Parapsoriasis

• Group of macular scaly eruptions with slow
  evolution
  
• These are all markedly chronic, resistant to
  treatment, and are without subjective symptoms
  
• They are divided into pityriasis lichenoides
  chronica, pityriasis lichenoides et varioliformis
  acuta, and parapsoriasis en plaques

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Pityriasis Lichenoides Chronica

• Tx- UV light is beneficial however intense doses
  may be needed for good results
  
• PUVA has been reported to be effective
• Oral tetracycline may be used with
  antihistamines
  
• PLC is a benign disease that clears spontaneously
  in a few yrs to months

• Erythematous, yellowish, scaly macules and
  lichenoid papules
  
• They persist indefinitely without change
• Mainly on sides of trunk, thighs, and upper arms
• May be confused with psoriasis and secondary
  syphilis

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Pityriasis Lichenoides Chronica
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PLEVA

• May run an acute, subacute, or chronic course
• Papules are usually yellowish or brownish-red,
  round lesions, which tend to crust, become
  necrotic and hemorrhage

• AKA parasoriasis lichenoides, Habermanns
  disease, Mucha-Habermann disease and
  parapsoriasis varioliformis acuta
  
• Sudden appearance of a polymorphous eruption
  composed of macules, papules, and occasional
  vesicles

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PLEVA
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PLEVA

• Usually a benign, self-limited disorder, but
  may be more chronic and severe
  
• Maybe a spectrum of cutaneous T-cell lymophoma
• Differential dx
• leukocytoclastic angiitis, papulonecrotic
  tuberculid, psoriasis, lichen planus, varicella,
  PR, drug eruptions, maculopapular syphilid,
  viral, rickettsial diseases, lymphomatoid
  papulosis

• When exanthem heals it leaves a smooth,
  pigmented, depressed, varioliform scar
  
• Favorite sites are anterior trunk, flexural arms,
  and axillae
  
• Palms and soles are involved infrequently-mucous
  membranes are not
  
• Generalized lymphadenopathy can occur

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PLEVA

• Histologically of PLEVA is characterized by
  epidermal necrosis, with prominent hemorrhage and
  primarily a dense perivascular infiltrate of
  lymphocytes in the superficial dermis
• Absence of neutrophils simplifies the distinction
  between leukocytoclastic angiitis
  
• Lymphomatoid papulosis differs by the presence of
  large, atypical mononuclear cells in the dermal
  infiltrate

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PLEVA-Tx

• No one tx is reliably effective
• Tetracycline and erythromycin are worth trying
• UVB and PUVA
• Methotrexate, 2.5-7.5mg every 12 hrs for 3 doses
  1 day each week
  
• Several serious reactions a few of them fatal
  have occurred with simultaneous administration of
  methotrexate and NSAIDs
  
• Dapsone and pentoxifylline(Trental), 400mg twice
  daily

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Parapsoriasis en Plaques

• Small-plaque parapsoriasis is characterized by
  non-indurated, brownish, hypopigmented, or
  yellowish red scaling patches, round to oval,
  with sharply defined borders
• Most lesions occur on the trunk and are
  between
  
• 1 5cm
• Patches may persist for years to decades and
  do not progress to lymphoma

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Large Plaque Parapsoriasis

• Has patches 5-15 cm otherwise is similar to
  small-plaque type
  
• Prognosis is benign, especially if pruritis is
  severe
  
• 10 may eventuate in T-cell lymphoma

71

• Large plaques parasporiasis large, variably
  erythematous and mildly poikilodermatous patches
  in the bathing trunk region

72

• Small plaque parasporiasis small(erythematous, slightly scaly patches

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Treatment

• First line UV radiation -- either natural or UVB
  
  
• Lubricants and Topical steroids
• PUVA but only if UVB fails
• Use of PUVA or high-potency topical streroids
  should be limited due to long-term adverse
  effects
  
• LPPP has the potential to develop lymphoma
  thus, justifying more intense tx
  
• Vitamin D2 daily250,000 units over 2-4 months
  has been effective
  

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Pityriasis Alba

• AKA-pityriasis streptogenes, furfuraceous
  impetigo, pityriasis simplex, pityriasis sicca
  faciei, and erthema streptogenes
  
• Characterized by hypopigmented, round to oval,
  scaling patches on face, upper arms, neck, or
  shoulders
  
• Color is white (but never actually depigmented)
  or light pink
  
• Scales are fine and adherent
• Patches are usually sharply demarcated edges may
  be erythematous and slightly elevated
  

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Pityriasis Alba

• Lack of any early specifically follicular
  localization helps to distinguish this lesion
  from follicular mucinosis
  
• Vellus hairs are not lost in pityriasis alba, nor
  does hypesthesia to cold occur, as often happens
  in follicular mucinosis
  
• Usually asymptomatic however there may be mild
  pruritis
  
• Disease mainly occurs in children and teenagers
• It is particularly a cosmetic problem in
  dark-skinned individuals

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Pityriasis Alba

• Etiology unknown
• Excessively dry skin appears to be contributory
• Most lesions disappear with time
• Repigmentation can be accelerated with treatment
• Emollients and bland lubricants
• Low-strength corticosteroids plus Lac-Hydrin are
  helpful
  
• Others have recommended PUVA

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Pityriasis Rosea

• Mild inflammatory exanthem of unknown origin
  ?viral
  
• Characterized by salmon-colored papules and
  patches which are oval and covered with a
  collarette of scale
  
• Disease frequently begins with a single herald
  patch, which may persist a week or more, then
  involutes

79
PR

• Appears rapidly and last from 3-8 weeks
• Peak ages 15-40
• Typically in Spring and Autumn
• More common in women

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Pityriasis Rosea

• Mainly affects the trunk
• Oral lesions are relatively uncommon, but present
  as aphthous lesions

81
Herald Patch
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Pityriasis Rosea

• Papular PR is an unusual form common in black
  chidren under age 5
  
• Inverse PR is unusual, but not rare
• Relapses and recurrences are frequently observed
• A PR-like eruption can occur as a rxn to
  captopril, arsenicals, gold, bismuth, clonidine,
  methoxypromazine, tripelennaminehydrochloride, or
  barbituates

83

• Inverse pityriasis rosea oval annular plaques in
  groin

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Treatment

• Supportive
• UVB should be used after acute inflammatory stage
  has passed
  
• Topical corticosteroids
• Antihistamines
• Emollients

85

• PR There is focal parakeratosis, mild
  acanthosis, spongiosis, perivascular lymphocytes,
  and focal erythrocyte extravasation
  

86

• PR papules and annular plaques

87

• PR oval and round plaques, some with central
  scale and others with a collarette of scale

88

• PR in darkly pigmented skin it tends to be more
  papular than in lightly pigmented skin-note
  associated hyperpigmentation

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Pityriasis Rubra Pilaris

• Chronic skin disease characterized by small
  follicular papules, disseminated yellowish pink
  scaling patches, and often, solid confluent
  palmoplantar hyperkeratosis
• Disease generally manifests itself first by
  scaliness and erythema of the scalp

90
PRP

• Involvement is usually symmetrical and diffuse,
  with islands of normal
  
• Hyperkeratosis of palms and soles called, the
  sandal
  
• Nails may be dull, rough, thick, and brittle
• Itching in some cases
• Koebners phenomenon may be present
• A number of cases have been associated with
  Kaposis sarcoma, leukemia, basal cell, lung,
  unknown primary metastatic and hepatocellular
  carcinoma

91
PRP

• PRP may classified into familal or acquired
  types
  
• in respect to the onset of the disease in
  childhood or adulthood
  
• Griffths classification Type I, the classic
  adult type, is seen most commonly, with 80
  involuting in 3 years
  
• Three types of juvenile-onset forms account for
  up to 40 of cases and have a poor prognosis for
  involution
  

92
PRP

• Etiology unknown-??AD
• Either sex affected
• Possible related to deficiency of
• vitamin A

• Histology hyperkeratosis, follicular plugging,
  and focal parakeratosis at follicular orifice
  
• Inflammatory infiltrate in dermis is composed of
  mononuclear cells

93

• PRP psoriasiform dermatitis with follicular
  plugging

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Treatment

• Methotrexate 2.5mg alternating with 5mg daily
• Monitor and treat secondary infections

• Symptomatic emollients-- Lac-Hydrin
• A several-month course of isotretinoin in doses
  of 0.5 2 mg/kg/day
  
• Vitamin A in doses of 300,00 to 500,000 untis
  daily, with possible addtion of vitamin E, 400
  units 2-3 times daily

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• Pityriasis rubra pilaris diffuse erythroderma
  with desquamation and follicular hyperkeratosis

98

• Pityriasis rubra pilaris follicular papules and
  confluent orange-red scaly plaques with islands
  of sparing

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• Pityriasis rubra pilaris orange-red waxy
  keratoderma of the palms