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Perforating disorders

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Perforating disorders

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    1. Perforating disorders By DR.MOHAMMED ALSHAHWAN SUPERVISED BY DR.FAHAD ALSAIF

    2. #DEFINITION: A group of conditions characterized by Transepithelial elimination of dermal Substance pathologically &umbilicated Papules w central white keratotic crusts Clinically

    3. *IN 1916 Kyrle described (hyperkeratosis follicularis et parafollicularis in cutem penetrans) *IN 1968 Mehregan&Coskey described Perforating folliculitis *IN 1982 White&Hurwitz described reactive perforating collagenosis

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

    5. 1.KYRLE DISEASE: #Etiology: *AR *pathogenic event is uncoupling of epithelial proliferation&differentiation

    6. #Pathology: *early lesions: Focal parakeratosis,acanthosis&cells of The basal layer increase in number and Disorganized. *late lesions: Follicular infundibulum/acrosyringium Dilated w funnel-shaped horny plug.

    7. Advancing parakeratotic column perforate The BMZ(at opening of sebaceous gland Duct&the 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

    8. #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

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

    10. 2.PERFORATING FOLLICULITIS: #Etiology: *pathogenic event is follicular wall perforation *chemical components of textiles or fragment or hairs.

    11. #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 . .

    12. *healing lesions---follicular epithelium start to sequestrate the content into hair follicle. #Treatment: *NO specific Tx *phototherapy(PUVA&UVB)

    13. 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

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

    15. #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

    16. #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 Hypothyrodism&hyperparathyrodism

    17. #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

    18. *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

    19. 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

    20. * 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

    21. #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

    22. #Treatment: *Isotretinoin *cryotherapy *ultrapulse CO2 laser

    23. THANK YOU

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