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40 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Name the 5 Major Perforating Disorders
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Reactive Perforating Collagenosis (RPC) - inherited
Elastosis Perforans Serpiginosa Perforating folliculitis Acquired perforating dermatosis (includes Kyrle's) Perforating periumbilical calcific elastosis |
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Key features of Reactive Perforating Collagenosis
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Childhood presentation
Follows superficial trauma Koebnerization seen Sites: arms & hands |
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Key features of Elastosis Perforans Serpiginosa
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Childhood or early adulthood presentation
40% assoc with other genetic disorders M:W 4:1 ratio Sites: Lateral neck... face, arms, flexural areas Lesions may last years |
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Name the disorders associated with EPS
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Cutis laxa
Acrogeria Rothmund Thomson Syndrome Marfan's Osteogenesis Imperfecta Penicillamine or PXE Ehlers Danlos Down Syndrome |
CARTS MOPED
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What is Kyrlie's Dz?
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"Catch all" term for acquired perforating dz arising in adults
10% of dialysis pts/renal failure Site: legs Hypothesis: Fibronectin levels are high in DM and Uremia therefore it binds to collagen IV and incites perforation |
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Name two genodermatosis assoc with Keloid formation
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Rubinstein-Taybe Syndrome
Goeminne Syndrome |
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Describe Rubinstein-Taybe Syndrome
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AR
Growth & Mental Retardation Small head Broad thumbs Characteristic facies Hypertrichosis of back High and narrow palate Crowded irregular teeth |
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Describe Goeminne Syndrome
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X-linked w/ incomplete penetrance
Torticollis Cryptorchidism Multiple keloids Renal dysplasia |
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Name cells and inflammatory factors thought to play a role in Keloid formation
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Platelets
Neutrophils Macrophages Lymphocytes Cytokines -- TGFB1 & B2 Melanocytes Mast Cells -- other factors: infection, excessive wound tension, foreign material |
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Major differences between hypertrophic scar and keloids
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Keloids can be spontaneous (no preceding injury), rarely spontaneously regress, delayed onset.
Hypertrophic scars have myofibroblasts and stay within wound margins, gradual resolution Both contain increased mast cells |
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Another name for Dupuytren's contracture
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Palmar fibromatosis
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Another name for penile fibromatosis
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Peyronie's Disease
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Another name for Lederhose's disease
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Plantar fibromatosis
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Name dzs assoc with dupuytren's contracture
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Peyronie's Disease
Lederhose's Disease Knuckle pads Alcoholism Diabetes |
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Pathogenesis of Dupuytren's contracture
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Proliferation of myofibroblasts aligning along lines of tension
Development of collagen thickening and disappearance of myofibroblasts |
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Name Diseases associated with Cutis Verticis Gyrata
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Acromegaly
Turner syndrome Fragile X syndrome Mental retardation Darier's dz Tuberous Sclerosis Myxedema Epilepsy Hyperinsulinism (* note most cases have no dz association * ) |
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Mid-dermal Elastolysis
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- acquired, non-inherited, worsened with UV light
- unknown pathogenesis, no effective RX - young women - well-circumscribed areas asymptomatic fine wrinkling - trunk, neck, upper extremities - elastic tissue absent from MID DERMIS |
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What is Anetoderma of prematurity? (aka congenital anetoderma)
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in premature infants, may be related to pressure adhesives (cutaneous monitoring leads or adhesives) or changes in flow of ions or water under monitor leads
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Diseases associated with Anetoderma?
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Lupus
Antiphospholipid antibodies Graves' disease HIV Lyme Dz (to name a few...) |
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Name two types of PRIMARY anetodermas
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Jadassohn-Pellizari type (+ inflammation)
Schweninger-Buzzi type (- inflammation) |
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What are SECONDARY anetodermas?
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-- 2ndary to a primary to dermatosis
-- 2ndary to a systemic disease (ridiculous long list...) |
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Histopathology of Anetoderma?
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Collagen normal
LOSS of ELASTIC tissue in papillary dermis or mid-reticular dermis |
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Treatment for Anetoderma?
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No successful routine rx
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Striae Distensae Histology
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Effaced, atrophic epidermis
Decreased dermal thickness and collagen bundle thinning Variable elastic tissue Absence of hair follicles and other appendages |
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Elastotic Striae (aka linear focal elastosis)
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Yellow palpable bands on the lower back
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Infection associated with Atrophoderma of Pasini and Pierni (38-53%)?
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Borrelia burgdorferi
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Where do you see Follicular atrophoderma? Assoc with what two genodermatosis?
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- follicular indentation without hairs; Back of hands or feet, on the cheeks. Also has Scrotal (fissured tongue).
1. Conradi-Hundermann-Happle syndrome (aka X-linked dominant chondrodysplasia punctata) 2. Bazex syndrome (also seen in keratosis palmoplantaris disseminata) |
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Keratosis Pilaris Atrophicans is seen in what three syndromes?
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1. Keratosis pilaris atrophicans faciei (and Ulerythema Ophryogenes)
2. Atrophodermia vermiculata 3. Keratosis pilaris follicularis spinulosa decalvans |
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Synonyms for Atrophodermia Vermiculata
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Atrophoderma vermiculata, atrophodermia ulerythematosa, folliculitis ulerythematosa reticulata, and honeycomb atrophy
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Atrophodermia Vermiculatum
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- childhood onset
- Follicular atrophoderma limited to face - honeycomb scars - defect may be follicular hyperkeratinazation of the upper third of the hair shaft leads to scarring - may be inherited (AD) - is assoc with various syndromes (down's, NF, etc) |
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Ulerythema ophyrogenes?
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lateral portion of eyebrows with pitted scars, erythema, atrophy, and alopecia
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Rombo Syndrome
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Atrophoderma Vermiculatum, cyanosis of the hands and feet, milia, telangiectases, hypotrichosis, multiple BCCs, and trichoepitheliomas
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Name the syndromes assoc with atrophoderma vermiculatum?
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Rombo syndrome
Nicolau-Balus syndrome Tuzun syndrome Braun-Falco-Marghescu syndrome |
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Nicolau-Balus Syndrome
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Atrophoderma vermiculatum, Syringomas and milia
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Tuzun syndrome
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Atrophoderma vermiculatum
Scrotal tongue |
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Braun-Falco-Marghescu syndrome
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Atrophoderma vermiculatum
PPK KP |
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Bazex Syndrome
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X-linked dominant
Multiple BCCs Hypotrichosis Localized hypohidrosis (above the neck) |
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Conradi-Hunermann-Happle Syndrome
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aka XLD chondrodysplasia puncatata
Gene = EBP (emomapil binding protein) - icthyosiform scaling erythroderma along Blashko's lines replaced by bands of follicular atrophoderma - also: cataracts, hyperpigmentation, scarring alopecia, saddle nose deformities, asymmetric limb reduction defects, and stippling calcifications of the epiphyses |
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Atrophia Maculosa Varioliformis Cutis
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Small round varioliform and linear asymptomatic facial depressions;
No preceding lesion or inflammation (spontaneous - different from atrophoderma vermiculatum) - unknown pathogenesis |
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Piezogenic Pedal Papules
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Induced by weight bearing (fat herniation through connective tissue)
Disappear with elevation Asymptomatic Common & normal |
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