Perforating disorders

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Perforating disorders

• By DR.MOHAMMED ALSHAHWAN
• SUPERVISED BY DR.FAHAD ALSAIF

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• DEFINITION
• A group of conditions characterized by
• Transepithelial elimination of dermal
• Substance pathologically umbilicated
• Papules w central white keratotic crusts
• Clinically

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• IN 1916 Kyrle described (hyperkeratosis
• follicularis et parafollicularis in cutem
  penetrans)
• IN 1968 MehreganCoskey described
• Perforating folliculitis
• IN 1982 WhiteHurwitz described reactive
  perforating collagenosis

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• Rapini colleagues delineated this disorder as
  acquired perforating dermatosis.
• Miescher described (ELASTOSIS PERFORANS
  SERPIGINOSA)

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• 1.KYRLE DISEASE
• Etiology
• AR
• pathogenic event is uncoupling of
• epithelial proliferationdifferentiation

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• Pathology
• early lesions
• Focal parakeratosis,acanthosiscells of
• The basal layer increase in number and
• Disorganized.
• late lesions
• Follicular infundibulum/acrosyringium
• Dilated w funnel-shaped horny plug.

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• Advancing parakeratotic column perforate
• The BMZ(at opening of sebaceous gland
• Ductthe pit of acrosyringium)
• before perforation---mononuclear cell
• after perforation---PMNC--granuloma--scar
• perifollicular epidermis
• pefroration is not must for diagnosis and occur
  less common in kyrle disease

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• Clinical features
• chronic difficult to treat
• In 3rd-5th decades more in female
• Asymptomatic or slightly pruritic
• not associated w systemic disease
• can involve extensors,scapular area,hands,feet
  and perianal-perigenital areas
• different stages of lesions leaving atrophic
  scar
• And patchy hypo/hyperpigmentation

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• Treatment
• NO specific Tx
• topical keratolytics/retinoids
• tissue-destructive modalities e.g.
  cryotherapy,CO2 laser.
• oral retinoids very effective alone or w
• PUVA(RePUVA).

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• 2.PERFORATING FOLLICULITIS
• Etiology
• pathogenic event is follicular wall
• perforation
• chemical components of textiles or fragment or
  hairs.

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• Pathology
• early lesions--suppurative folliculitis
• late lesions---perforation occur at
• infundibular portion w keratotic plug contain
  keratotic debris,neutrophils,
• elastic fibers then gradully chronic
• inflammatory infiltrate w granloma formation
  engulfing elastic fibers

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• healing lesions---follicular epithelium
• start to sequestrate the content into
• hair follicle.
• Treatment
• NO specific Tx
• phototherapy(PUVAUVB)

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• 3.REACTIVE PERFORATING COLLAGENOSIS
• Etiology
• UNKNOWN
• abnormal cutenous response to trauma due to
  dermal vasculopathy.
• exocytosis and disintegration of PMNC releasing
  proteolytic enzymes
• foreign-body reaction to altered dermal
  substances

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• dermal microdeposits of crystalline
• substance e.g(hydrpxyapatite,uric acid)
• dysregulated metabolism of vitamins
• AD
• high serum level and dermal depostion of
• fibronectin
• 2ND to perforation.
• regenrating epidermis theory.

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• Pathology
• invaginations of the epidermis containing
• multiple perforations(Occasionally hair
• follicles/shafts or microdeposits)
• early lesionssuppurative reaction
• late lesionsgranuloma
• healing lesionsregenaration of epidermis
• long-standing lesionssigns of scrathing

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• Clinical features
• chronic
• very prurtic(ve koebner phenomenon)
• different stages of lesions wout major scaring.
• associated w CRF/-DM,CAH,CLD,HIV
• HZV,malignancy,sclerosing cholangitis
• Hypothyrodismhyperparathyrodism

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• Treatment
• Treating the associated disease e.g.
• CRF or DM
• reducing trauma (scratching) by topical
• antipruritic agents e.g.menthol,phenol or
  camphor and antiflammatory e.g topical
• steroids

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• topical keratolytic/tretinoin
• tissue-destructive treatments e.g. cryotherapy
• phototherapy(UVB,PUVA NBUVB)
• systemic therapies
• - oral retinoids e.g. isotetinoin
• - oral allopurinol 100mg OD
• other therapies systemic rifampicin,TCENS

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• 4.ELASTOSIS PERFORANS SERPIGINOSA
• Etiology
• pathogenic event is abnormal elastin
• eliminated through epidermis
• D-penicillamine
• Genosyndroms e.g.Downs,osteogenesis
  imperfecta,Marfans,EDS,cutis laxa,
• Rothmund-Thompson syndrome and
• pseudoxanthoma elasticum

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• Familial cases(AD)?
• C.T.D
• Pathology
• superficial perivascular interstital
• Mainly neutrophils
• abnormal elastic fibers in the upper dermis
• channels of epithelium through epidermis
• Containing neutrophils,depris,parakeratotic cells
• And abnormal elastic fibers

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• Clinical features
• 2mm-1cm keratotic papules arranged in
• serpiginous arcs around atrophic skin
• on the neck,extremities and rarely trunk.
• rare systemic involvement e.g. rupture
• of aorta

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• Treatment
• Isotretinoin
• cryotherapy
• ultrapulse CO2 laser

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THANK YOU