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This Concept Map, created with IHMC CmapTools, has information related to: Skin I, Skin I Pre-, Malignant Epidermal Tumors ACTINIC KERATOSIS -premalignant dysplasia-1%->SqCC -buildup of excess keratin -result of excess sun exposure -p53 mutation in AK and SqCC -marks inc'd risk for BCC Risk-light skin, ion. radiation, hydrocarbons, arsenicals -rough, can have 'cutaneous horn' -face, arms, hand dorsum -actinic cheilitis around mouth MORPH-cellular atypia in lower layers -basal hyperplasia -or atrophy of epidermis -INTERCELL BRIDGES-unlike BCCa -elastosis in dermis, parakeratosis -tan-brown, flesh, or red color Tx-just remove all of these, Skin I Benign Epithelial Tumors ADNEXAL (APPENDAGE) TUMORS -mostly benign -can mark internal malignancy -multiple trichilemmomas+br CA -COWDEN SYNDROME Eccrine poroma-palms, soles Cylindroma = Turban tumor-APOCRINE -jigsaw-like islands Syringomas-lower eyelids -immature eccrine gland Trichoepithelioma-face, upper trunk -keratin-filled microcysts -immature hair follicle -AD INHERITANCE Hydradenoma papilliferum-apocrine MALIGNANT: SEBACEOUS CA-eyelid glands->mets ECCRINE, APOCRINE CA-confused w/ mets adenoCA, Skin I Tumors of the Dermis DERMATOFIBROSARCOMA PROTUBERANS -firm, solid, on trunk-may ulcerate -can have protuberance w/in induration MORPH-some mitoses -storiform-pinwheel-like fib arrangement -THINNED EPIDERMIS, unlike DF -honeycomb pattern if into subQ fat, Skin I Tumors of Cellular Immigrants to the Skin MASTOCYTOSIS -mast cells in skin, other organs -systemic in 10%-adults-Poor Px -pruritus, flushing after temp, EtOH, drugs -rhinorrhea, GI/nasal bleeding, bone pain Urticaria Pigmentosa ᡪ% of MC -in kids -multiple round/oval non-scaling papules or small plaques-red-brown papules Solitary mastocytomas-pruritic, blistering Sx due to heparin, histamine degranulation Darier Sign-wheal occurs when skin is rubbed Dermatographism-edema, like hive after stroking w/ pointed instrument SYSTEMIC DZ-in 10%, worse Px-GI bleed, bone pain, pruritus, flushing, rhinorrhea MORPH-subtle or overt increase in mast cells -upper to mid dermis -fibrosis, edema, eos can be present -GIEMSA or TOLUIDINE BLUE (+) mast cells c-kit protooncogene mut->type III RTK activation, Skin I Tumors of the Dermis DERMAL VASCULAR TUMORS Capillary Hemangioma-most common -kids="strawberry heman" -usually regress by fibrosis -adults-usually enlarge -diff'ate from dermal vasc hyperplasia -from venous stasis -in superficial derm, sometimes deeper Malformations: Nevus flammeus Port-wine Stains Multifocal Angioproliferative Lesions Bacillary Angiomatosis Kaposi Sarcoma-HHV-8 Malignant - ANGIOSARCOMA, Skin I Tumors of Cellular Immigrants to the Skin MYCOSIS FUNGOIDES - CTCL (CUTANEOUS T-CELL LYMPHOMA) -can ->lymphoma -mostly stays in skin for years-good Px ᡠyo-trunk, extremities, feet, scalp -scaly, red-brown, raised, fungating Stages: patch->plaque->nodule(tumor) ->erythrodermic (Sezary Syndrome) -irreg outline, can ulcerate -if seed blood->erythroderma all over -SEZARY SYNDROME w/ scaling MORPH-Sezary-Lutzner cells-CD4 Tcells -acanthosis -can invade dermis-Pautrier u-abscesses -cerebriform nuclei -if more malig->invade deeper, vessels Tx: topical steroids, UV radiation -CTX if more advanced Px: 40% 5yr survival if erythrodermic stage, Skin I Pigmentation, Melanocyte Disorders-neural crest S-100-Melanocyte marker NEVOCELLULAR NEVUS (PIGMENTED NEVUS, MOLE) -any congen or acq'd mel'cyte neoplasm -sun exposed areas -macules-papules ɞmm Jcnal nevi-nests of mel'cytes More elevated: Compound-older-into dermis Dermal-older-epid nests lost -deeper are lighter, mature =maturation->not malig., HISTIOCYTOSIS X= LANGERHANS CELL HIST. KIDS>adults -reniform nuclei -bone marrow-derived -solitary or mult -papules, nodules, to scaling plaques -can resemble seborrheic derm. in infants MORPH-bland cells, infolded mononuc CD1a, S100 Ag staining or Birbeck grans Other Hystiocytoses LETTERER-SIZE DZ Acute, disseminated multisystemic Infancy Pulm, bone, hep'splen'meg HAND-SCHULLER-CHRISTIAN -CHRONIC, ELDERLY -progressive, multifocal Bone, DI, exopthalmos, mucocutaneous lesions EOSINOPHILIC GRANULOMA -localized benign form, Skin I Pigmentation, Melanocyte Disorders-neural crest S-100-Melanocyte marker MALIGNANT MELANOMA -also start in oral, anogenital muc, esoph, mening, EYE -men-upper back -women-back, legs -Blacks, Asians-Soles/Palms, nail beds, muc membs Risks-SUN, dysplastic nevi,light skin,XeroPig -10% run in families -1p36 (CMM1 gene) -9p21 (p16 t-supp gene)-p16INK4A -12q14-CDK4 -indoor occupation, outdoor hobbies -Late, vertical stages w/ more BRAF mut CLINICAL-can be itchy -CHANGE IN COLOR-most impt SSx Asym Border Color Diameter Elev/Enlrg MORPH Radial-lentigo maligna-sun-dmg skin -NO METS POTENTIAL Vertical-NO maturation-BRESLOW SCALE -w/ nodule growth in radial phase -cells larger than nevus cells-irreg -growth correlates w/ METS POTENTIAL, Skin I Benign Epithelial Tumors EPITHELIAL CYST (WEN) -downgrowth, cystic expansion of epidermis or hair follicle -movable nodules -filled w/ keratin, sebaceous fat MORPH-diff walls -Epidermal inclusion cyst-like epid -Pilar/Trichilemmal cyst -like hair follic epith -Dermoid cyst-like epid, but w/ appendages -Steatocystoma multiplex -seb gland duct-like wall -heritable, Skin I Epidermal Maturation D/Os ICHTHYOSIS-fish-like scales -hyperkeratosis - NOT INFLAMMATORY -at or around birth -can be acquired as adults Types: 1. Ich Vulgaris-AD or aquired 2. Congen ichthyosiform erythroderma-AR 3. Lamellar ichthyosis-AR 4. X-linked ichthyosis-def steroid sulfatase ->persistent cell-cell adhesion in s corneum MORPH-little inflam, varied epid thickening Maybe due to defect in desquamation, BASAL CELL CARCINOMA -common, slow, rare mets -sun-exposed in light skin-unrepaired DNA dmg -esp immunosupp, XERO PIGMENTOSUM -AK-damaged skin -40-60% w/ p53 mutations -30% w/ PTCH mutations -pearly papules w/ telangiectasias -some w/ melanin-mimic nevi, melanoma -can ulcerate, invade bone-"rodent ulcers" MORPH-resemble NL basal cells -no mucosa -grow, ulcerate, NO METS Multifocal growths or Nodular lesions -palisading at periphery of tumor islands -separated by stroma by clefts NEVOID BASAL CELL CA SYNDROME =GORLIN SYNDROME- AD Many BCCs ងyo Also abnl bone, nervous, eyes, reproductive organs -cleft lip/palate -palm/sole pits Mutated t-supp PATCHED (PTCH) -ligand is Sonic hedgehog -2-hit hypothesis, Skin I Pre-, Malignant Epidermal Tumors BASAL CELL CARCINOMA -common, slow, rare mets -sun-exposed in light skin-unrepaired DNA dmg -esp immunosupp, XERO PIGMENTOSUM -AK-damaged skin -40-60% w/ p53 mutations -30% w/ PTCH mutations -pearly papules w/ telangiectasias -some w/ melanin-mimic nevi, melanoma -can ulcerate, invade bone-"rodent ulcers" MORPH-resemble NL basal cells -no mucosa -grow, ulcerate, NO METS Multifocal growths or Nodular lesions -palisading at periphery of tumor islands -separated by stroma by clefts, Skin I Pigmentation, Melanocyte Disorders-neural crest S-100-Melanocyte marker MELASMA-"mask of pregnancy" -mask-like facial hyperpig'tion -sunlight accentuates -resolves spontaneously -also w/ BCP, hydantoins Epidermal-inc. melanin in basal -responds to hydroquinone -enhanced by WOODS LIGHT Dermal type-macs in pap dermis -phago'd melanin from above, Skin I Tumors of the Dermis XANTHOMAS -tumor-like w/ foamy histiocytes-chol, TGs, PLs -w/in dermis -can have fibrosis, inflam cells too (not x'lasma) 5 types: all w/ diff lipid d/os Eruptive-wax/wane w/ lipids, butt, knees, elbows Tuberous-yellow Tendinous-yellow, tendons(Achilles, fingers) Plane-w/ bil cirrhosis-skinfolds, palmar creases Xanthelasma-soft yellow, on eyelids, Skin I Benign Epithelial Tumors ACANTHOSIS NIGRICANS -thickened, hyperpigmented -in flexural areas -80% benign 1. AD occurrence 2. obesity, endocrine (DM pit, pineal tumors) 3. part of congen syndromes -can be from EGFs from CA -esp malig-esp in adenoCA MORPH-basal hyperpig -peaks and valleys -hyperkeratosis too Watch out for underlying dz, Skin I Pigmentation, Melanocyte Disorders-neural crest S-100-Melanocyte marker FRECKLE (EPHELIS) -most common lesions -reappear in winter-darken in sun -increased melanin in basal cells, Skin I Tumors of the Dermis BENIGN FIBROUS HISTIOCYTOMA-gen term -fibroblasts and histiocytes -adults, legs of young women -indolent course -tan-brown, firm, can be tender -flatten over time, can be mult cm -DIMPLE INWARD on LATERAL COMPRESSION (unlike nodular melanomas) DERMATOFIBROMA-most common-few hists -nonencapsulated in mid-dermis -epidermal hyperplasia-"dirty fingers" SCLEROSING HEMANGIOMAS-w/ vessels, hemosiderin, Skin I Pre-, Malignant Epidermal Tumors SQUAMOUS CELL CARCINOMA -older yo, sun-drenched -MEN>WOMEN, except lower leg -Head and neck Risk-sun, tars/oils, chronic ulcers, draining osteomyelitis, burn scars, arsenicals, ion radiation, tobacco, betel nut chewing -immunosupp or XP, HPV 36 -DNA dmg by UV light MORPH-keratin pearls/eddies -in situ CA-red scaling plaques -hyperkeratosis-mimic keratoacan -can be leukoplakia if oral -enlarged, hyperchrom nuc at ALL levels of epid -invasive-diff'd or anaplastic CLINICAL ɝ% mets by discovery time EPIDERMODYSPLASIA VERRUCIFORMIS -many cutaneous SqCC -via HPV 5, 8, Skin I Benign Epithelial Tumors SEBORRHEIC KERATOSES -middle-aged or older -on TRUNK, limbs, H/N -"dermatosis papulosa nigra" -if multiple on face -plaques mm-cm-coin lesions -PSEUDO-HORN CYSTS -uniform color, velvety -small, keratinoid (horn)cysts -hyperkeratosis -downward growth if w/ hair -"inverted follic keratoses" Explosively in paraneoplastic syn=sign of Leser-Trelat -due to TGFa