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40 Cards in this Set

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Name the 5 Major Perforating Disorders
Reactive Perforating Collagenosis (RPC) - inherited
Elastosis Perforans Serpiginosa
Perforating folliculitis
Acquired perforating dermatosis (includes Kyrle's)
Perforating periumbilical calcific elastosis
Key features of Reactive Perforating Collagenosis
Childhood presentation
Follows superficial trauma
Koebnerization seen
Sites: arms & hands
Key features of Elastosis Perforans Serpiginosa
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
Name the disorders associated with EPS
Cutis laxa
Acrogeria
Rothmund Thomson Syndrome
Marfan's
Osteogenesis Imperfecta
Penicillamine or PXE
Ehlers Danlos
Down Syndrome
CARTS MOPED
What is Kyrlie's Dz?
"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
Name two genodermatosis assoc with Keloid formation
Rubinstein-Taybe Syndrome
Goeminne Syndrome
Describe Rubinstein-Taybe Syndrome
AR
Growth & Mental Retardation
Small head
Broad thumbs
Characteristic facies
Hypertrichosis of back
High and narrow palate
Crowded irregular teeth
Describe Goeminne Syndrome
X-linked w/ incomplete penetrance
Torticollis
Cryptorchidism
Multiple keloids
Renal dysplasia
Name cells and inflammatory factors thought to play a role in Keloid formation
Platelets
Neutrophils
Macrophages
Lymphocytes
Cytokines -- TGFB1 & B2
Melanocytes
Mast Cells
-- other factors: infection, excessive wound tension, foreign material
Major differences between hypertrophic scar and keloids
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
Another name for Dupuytren's contracture
Palmar fibromatosis
Another name for penile fibromatosis
Peyronie's Disease
Another name for Lederhose's disease
Plantar fibromatosis
Name dzs assoc with dupuytren's contracture
Peyronie's Disease
Lederhose's Disease
Knuckle pads
Alcoholism
Diabetes
Pathogenesis of Dupuytren's contracture
Proliferation of myofibroblasts aligning along lines of tension
Development of collagen thickening and disappearance of myofibroblasts
Name Diseases associated with Cutis Verticis Gyrata
Acromegaly
Turner syndrome
Fragile X syndrome
Mental retardation
Darier's dz
Tuberous Sclerosis
Myxedema
Epilepsy
Hyperinsulinism
(* note most cases have no dz association * )
Mid-dermal Elastolysis
- 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
What is Anetoderma of prematurity? (aka congenital anetoderma)
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
Diseases associated with Anetoderma?
Lupus
Antiphospholipid antibodies
Graves' disease
HIV
Lyme Dz
(to name a few...)
Name two types of PRIMARY anetodermas
Jadassohn-Pellizari type (+ inflammation)
Schweninger-Buzzi type (- inflammation)
What are SECONDARY anetodermas?
-- 2ndary to a primary to dermatosis
-- 2ndary to a systemic disease
(ridiculous long list...)
Histopathology of Anetoderma?
Collagen normal
LOSS of ELASTIC tissue in papillary dermis or mid-reticular dermis
Treatment for Anetoderma?
No successful routine rx
Striae Distensae Histology
Effaced, atrophic epidermis
Decreased dermal thickness and collagen bundle thinning
Variable elastic tissue
Absence of hair follicles and other appendages
Elastotic Striae (aka linear focal elastosis)
Yellow palpable bands on the lower back
Infection associated with Atrophoderma of Pasini and Pierni (38-53%)?
Borrelia burgdorferi
Where do you see Follicular atrophoderma? Assoc with what two genodermatosis?
- 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)
Keratosis Pilaris Atrophicans is seen in what three syndromes?
1. Keratosis pilaris atrophicans faciei (and Ulerythema Ophryogenes)
2. Atrophodermia vermiculata
3. Keratosis pilaris follicularis spinulosa decalvans
Synonyms for Atrophodermia Vermiculata
Atrophoderma vermiculata, atrophodermia ulerythematosa, folliculitis ulerythematosa reticulata, and honeycomb atrophy
Atrophodermia Vermiculatum
- 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)
Ulerythema ophyrogenes?
lateral portion of eyebrows with pitted scars, erythema, atrophy, and alopecia
Rombo Syndrome
Atrophoderma Vermiculatum, cyanosis of the hands and feet, milia, telangiectases, hypotrichosis, multiple BCCs, and trichoepitheliomas
Name the syndromes assoc with atrophoderma vermiculatum?
Rombo syndrome
Nicolau-Balus syndrome
Tuzun syndrome
Braun-Falco-Marghescu syndrome
Nicolau-Balus Syndrome
Atrophoderma vermiculatum, Syringomas and milia
Tuzun syndrome
Atrophoderma vermiculatum
Scrotal tongue
Braun-Falco-Marghescu syndrome
Atrophoderma vermiculatum
PPK
KP
Bazex Syndrome
X-linked dominant
Multiple BCCs
Hypotrichosis
Localized hypohidrosis (above the neck)
Conradi-Hunermann-Happle Syndrome
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
Atrophia Maculosa Varioliformis Cutis
Small round varioliform and linear asymptomatic facial depressions;
No preceding lesion or inflammation (spontaneous - different from atrophoderma vermiculatum)
- unknown pathogenesis
Piezogenic Pedal Papules
Induced by weight bearing (fat herniation through connective tissue)
Disappear with elevation
Asymptomatic
Common & normal