Index. Cystic lymphatic malformations, 54, 67 clinical features, 67

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1 INDEX Acquired acral fibrokeratoma, 279 Acquired elastotic hemangioma, 174 clinical features, 174 histopathologic features, immunohistochemistry, 174 treatment, 175 Acral pseudolymphomatous angiokeratoma in children (APACHE) histopathologic features, 309, 310 treatment, 309 Acroangiodermatitis of Mali, , Acrosyringium, 2 Aggressive angiomyxoma cutaneous angiomyxoma and, 295, 296 histopathologic features, 296, 297, 298 immunohistochemistry, 296 Alopecia areata, 37 Amniotic fluid, I Angioblastoma, 164 Angioendothelioma endovascular papillary, see Dabska's tumor papillary intralymphatic, see Dabska's tumor Angioendotheliomatosis, 128 malignant, 128, see also Intravascular lymphomatosis reactive, see Reactive angioendotheiiomatosis Angiofibroblastoma of the vulva, 296 Angiofibroma clinical features, histopathologic features, immunohistochemistry, 282 treatment, 282 Angiokeratoma, 47, 64, 86 acral pseudolymphomatous in children (APACHE), 309, 310 circumscriptum, 86 clinical features, corporis diffusum, 87-88, 89,90,91, see also Fabry's disease Fordyce's, 86, 90 histopathologic features, 89-91, 92 Mibelli's, 87 treatment, 91 Angioleiomyoma clinical features, 284 epithelioid, 286 histopathologic features, immunohistochemistry, 284, 286 intravascular, 286 palisaded, 286 treatment, 286 Angiolipoma cellular, 288 clinical features, 287 histopathologic features, infiltrating, 288 lipoma vs., 287 multiple, 287 subcutaneous, 288 treatment, 289 Angiolymphoid hyperplasia with eosinophilia (AHE) clinical features, differential diagnosis, 102 HHV8 and, 99 histopathologic features, immunohistochemistry, 102 Kimura's disease and, 99, 102, nevus flammeus and, 43 treatment, 102 Angioma cherry, see Cherry angioma choroidal, 39 spider, see Spider angioma sudoriparous, 23 tufted, see Tufted angioma Angioma serpiginosum, 133 atypical, 134 clinical features, histopathologic features, 134 treatment, 134 Angiomatosis bacillary, see Bacillary angiomatosis bilateral retinal, 42 epithelioid, 112 homolateral leptomeningeal, ipsilateral leptomeningeal,

2 312 Angiomyxoma, 293, see also Aggressive angiomyxoma; Cutaneous angiomyxoma Angiosarcoma CD31 and, 8 CD34 and, 8 cutaneous, associated with lymphedema, see Cutaneous angiosarcoma associated with lymphedema cutaneous, of the face and scalp, see Cutaneous angiosarcoma of the face and scalp cutaneous, radiation-induced, see Radiationinduced cutaneous angiosarcoma epithelioid, see Epithelioid angiosarcoma VEGFR-3 and, 8 vwfand,7 Wilson Jones', see Cutaneous angiosarcoma of the face and scalp Antigens CDl,2 factor VIII-related, 7 human leukocyte,-dr, 2 Apocrine glands, 2 Arteries adventitia, 5 intima, 4-5 media, 5 Arteriovenous hemangioma, 154 clinical features, 154, 155 histopathologic features, 154, 155 inflammatory, 99 treatment, 154 Ataxia-telangiectasia, 82, 84 Atypical pyogenic granuloma, 99 Atypical vascular lesions, 195 Bacillary angiomatosis, 112 clinical features, 112 histopathologic features, 113, 114 Kaposi's sarcoma and, 112 treatment, 113 verruga peru ana and, 116 Bannayan-Riley-Ruvalcaba syndrome, 57, 59 Bannayan-Zonana syndrome, 56 Bartonella bacilliformis, 116 henselae, 112 quintana, 112 Bartonellosis, 116 Beckwith-Wiedemann syndrome, 42 Benign atypical vascular lesions of the lip, 120 Index Benign lymphangioendothelioma, 191, 195 clinical features, differential diagnosis, 192 histopathologic features, 192, 193 hobnail hemangioma and, 157 immunohistochemistry, 192 Kaposi's sarcoma and, 192, 196,229 treatment, 192 Benign lymphangiomatous papules, 195 Benign neonatal hemangiomatosis, 141 Benign vascular proliferations Benign vascular proliferations, in irradiated skin, 195 clinical features, 195 histopathologic features, treatment, 197 Birbeck granules, 2 Birt-Hogg-Dube syndrome, 287 Blue capillary sponge blebs, 51 Blue rubber bleb nevus syndrome, 52-53, 57, 59, 198 Bockenheimer's syndrome, 34 Cafe-au-lait macules, 54 Campbell de Morgan spots, 151 Capillary aneurysm, 76 clinical features, 76 histopathologic features, treatment, 78 Carcinoembryonic antigen (CEA), 8 Carcinomas, 7 Carney's complex, 293 Carrion's disease, 116 Castleman's disease, 169,219 Cat scratch disease, 112 CDI,2 CD31, 7, 8 CD34, 7, 8 Cherry angioma clinical features, 151 histopathologic features, immunohistochemistry, 151 treatment, 152 Chondrosarcoma, Choroidal angioma, 39 Cirsoid aneurysm, 154 Coats' disease, 42 Cobb's syndrome, 41

3 Index Collagen, embryological, 2 Composite hemangioendothelioma clinical features, 250 histopathologic features, 250 immunohistochemistry, 250 treatment, 250 Cornification, 1-2 Cowden disease, 57 Crow-Fukase syndrome, 169 Cutaneous angiolipoleiomyoma clinical features, 290 histopathologic features, immunohistochemistry, 290 treatment, 292 Cutaneous angiomyxoma aggressive angiomyxoma and, 295, 296 Carney's complex and, 293 clinical features, 293 immunohistochemistry, 295 treatment, 295 Cutaneous angiosarcoma, radiation-induced, see Radiation-induced cutaneous angiosarcoma Cutaneous angiosarcoma associated with lymphedema,258 clinical features, 258 differential diagnosis, histopathologic features, immunohistochemistry, treatment, 260 Cutaneous angiosarcoma of the face and scalp, 251 clinical features, , 253 fig immunohistochemistry, 253, 255 Kaposi's sarcoma and, treatment, 255 Cutaneous collagenous vasculopathy, 82, 83, 84 Cutaneous histiocytoid hemangioma, 99 Cutaneous myofibroma, 212 clinical features, 212 histopathologic features, immunohistochemistry, 213 pericytes and, 215 treatment, 215 Cutaneous pseudolymphoma, 309 Cutis marmorata telangiectatic a congenita (CMTC),32 anomalies associated with, 33 clinical features, differential diagnosis, 34 histopathologic features, treatment, 34 Cystic hygromas, see Cystic lymphatic malformations Cystic lymphatic malformations, 54, 67 clinical features, 67 histopathologic features, 67, 68 fig. superficial lymphatic malformations vs., 67 treatment, 68 Cytogenetic studies, 12 Cytokeratin, 7 Cytomegalovirus (CMV), Dabska's tumor angiosarcoma and, 243 clinical features, 241 glomeruloid hemangioma and, 172, 243 histopathologic features, hobnail hemangioendothelioma and, 241, 243 hobnail hemangioma and, 157 immunohistochemistry, 243 retiform hemangioendothelioma and, 243 treatment, VEGFR-3 and, 8, 243 Dermatofibroma epithelioid, 236 multinucleate cell angiohistiocytoma and, Dermatofibrosarcoma protuberans, 8 Dermis blood supply, 2-3 composition, 2 embryological development of, 1, 2 Digital verrucous fibroangioma, 47 Disseminated eruptive hemangiomas, 139 Disseminated hemangiomatosis, 139 Eccrine angiomatous hamartoma (EAR), 19,23 clinical features, histopathologic features, 24 immunohistochemistry, 24 treatment, 24 Eccrine glands, 2 Ectoderm, 1 Embryo, skin of, 1-2 Endothelial cells, 5 Endovascular papillary angioendothelioma, see Dabska's tumor Epidermis, 1, 2 Epithelioid angioleiomyoma, 286 Epithelioid angiomatosis, 112 Epithelioid angiosarcoma, 7 clinical features, 268 histopathologic features, immunohistochemistry, 268 treatment, 270 Epithelioid dermatofibroma, 236 Epithelioid hemangioendothelioma, 236 carcinoma and, 7 clinical features,

4 314 differential diagnosis, 237, 239 eccrine syringofibroadenoma and, 237 GLUT-1 and, 10 histopathologic features, immunohistochemistry, 237, 239 kaposiform hemangioendothelioma and, 236 retiform hemangioendothelioma and, 236 spindle-cell hemangioma and, 186,236 treatment, 239 vwfand,7 Epithelioid hemangioma, 99 Epithelium, embryologic, 1 Epulis gravidarum, 106 Fabry's disease, 56, 88-89, 90, 91, 92, see also Angiokeratoma: corporis diffusum Factor VIII-related antigen, 7 Fibroangioma digital verrucous, 47 Fibrokeratoma acquired acral, 279 Fibrous papule of the nose, 279, 280 Fordyce's angiokeratoma, 86, 90 Generalized essential telangiectasia, 80, 83 histopathologic features, Giant-cell angioblastoma clinical features, 184 hemangiopericytoma and, 184 histopathologic features, 184, 185 treatment, 184 Glaucoma, Glomangiomas, 57, 198,201 Glomangiomyoma,201 Glomangiosarcoma, clinical features, 271 differential diagnosis, 273 hemangiopericytoma and, 273 histopathologic features, immunohistochemistry, 271, 273 leiomyosarcoma and, 273 nodular hidradenoma and, 273 treatment, 273 Glomeruloid hemangioma, 169 clinical features, 169 differential diagnosis, endovascular papillary angioendothelioma and, 172,243 histogenesis, histopathologic features, immunochemistry, 169 intravascular papillary endothelial hyperplasia and, intravascular pyogenic granuloma and, 171 treatment, 172 Glomus bodies, 5 Glomus cells, 5 Glomus tumors, 198 apocrine hidradenomas and, 202 blue rubber bleb nevus syndrome and, 198 clinical features, ,201 fig. glomangiomas and, 198,201 glomangiomyomas and, 201 histopathologic features, , immunohistochemistry, Kasabach-Merritt syndrome and, 198 malignant, see Glomangiosarcoma as pericytes, 201 treatment, 202 GLUT-I, 7, 8,10 Gorham's syndrome, 56, 59 Graves' disease, 80 Index Hair follicles, 2 Hamartoma defined, 19 eccrine angiomatous, see Eccrine angiomatous hamartoma (EAH) Hemangioendothelioma, 18 CD31 and, 8 composite, see Composite hemangioendothelioma epithelioid, see Epithelioid hemangioendothelioma hobnail, see Hobnail hemangioendothelioma kaposiform, see Kaposiform hemangioendothelioma polymorphous, 250 retiform, see Retiform hemangioendothelioma spindle cell, see Spindle cell hemangioma Hemangioma, 15 acquired elastotic, see Acquired elastotic hemangioma arteriovenous, see Arteriovenous hemangioma cavernous, 51 CD31 and, 8 congenital, 142 congenital nonprogressive, cutaneous histiocytoid, 99 disseminated eruptive, 139 epithelioid, 99 glomeruloid, see Glomeruloid hemangioma histiocytoid, 99 hobnail, see Hobnail hemangioma infantile, see Infantile hemangioma

5 Index intramuscular, 288 lobular capillary, 105 microvenular, see Microvenular hemangioma miliary, l39 multiple cutaneous, non involuting congenital, 142 progressive capillary, 164 sinusoidal, see Sinusoidal hemangioma spindle cell, see Spindle cell hemangioma targetoid hemosiderotic, see Hobnail hemangioma verrucous, 47 visceral, l Hemangiomatosis benign neonatal, 141 disseminated, l39 neonatal, 139 Hemangiopericytoma, 208 clinical features, 208 giant-cell angioblastoma and, 184 glomangiosarcoma and, 273 histopathologic features, immunohistochemistry, infantile myofibromatosis and, 209 treatment, 210 Hereditary benign telangiectasia, 82, 83 Hereditary hemorrhagic telangiectasia, histopathologic features, treatment, Hibernoma, 289 Hidradenoma apocrine, 202 nodular, 273 Histiocytoid hemangioma, 99 Histochemical stains, 7 Hobnail hemangioendothelioma, 241, 243 Hobnail hemangioma, 157 benign lymphangioendothelioma and, 157 clinical features, 157 Dabska's tumor and, 157 differential diagnosis, 159 histopathologic features, immunohistochemistry, 159 Kaposi's sarcoma and, 157, 159,229 retiform hemangioendothelioma and, 157 treatment, 159 VEGPR-3 and, 8, 159 Hodgkin's disease, 106 Human hematopoietic progenitor cell antigen, see CD34 Human herpesvirus-8 (HHV-8), 219 AHE and, 99 Kaposi's sarcoma and, 11 Human leukocyte antigen-dr, Human papillomavirus (HPV), 218 Hyperkeratotic capillary-lymphatic malformations, 86 Hyperkeratotic vascular malformations, 134 Hyperkeratotic vascular stains, 47 clinical features, 47, 48 histopathologic features, 47, 49 treatment, 47 Hyperplasia, 15 Hypochromic nevus, 29 Hypomelanosis, 29 Immunohistochemical stains, 7-10, see also subheading immunohistochemistry under names of lesions Infantile hemangioma, 15, 136 capillary, 15, 18, l36 cavernous, 15, 18, 136 clinical features, l deep, l36 GLUT-1 and, 10, 145 histopathologic features, immunohistochemistry, 143, 145 kaposiform hemangioendothelioma and, 177 Kasabach-Merritt syndrome and, , mixed, l36 prevalence, 137 stages, superficial, l36 treatment, tufted angioma and, 166 VEGPR-3 and, 8, 143 Infantile myofibromatosis, 209, 212 Inflammatory angiomatous nodule, 99 Intramuscular hemangioma, 288 Intravascular angioleiomyoma, 286 Intravascular lymphomatosis clinical features, 304, 305 histopathologic features, 304, immunohistochemistry, 304 treatment, 307 Intravascular papillary endothelial hyperplasia (IPEH), 119 clinical features, 119 glomeruloid hemangioma and, histopathologic features, , 121 Kaposi's sarcoma and, 119 Stewart-Treves syndrome and, 119 treatment, 120 Intravascular pyogenic granuloma glomeruloid hemangioma and, 171 Intravenous atypical vascular proliferation, 99

6 316 Index Kaposiform hemangioendothelioma clinical features, 177 epithelioid hemangioendothelioma and, 236 GLUT-1 and, 10 histopathologic features, immunohistochemistry, 178 infantile hemangioma and, 177 Kaposi's sarcoma and, 177, 230 Kasabach-Merritt syndrome and, 177 treatment, 180 tufted angioma and, 166, 177 VEGFR-3 and, 8,178 Kaposi's sarcoma, 59, African-endemic variant, 218, 219 AIDS-associated, 218, bacillary angiomatosis and, 112 benign lymphangioendothelioma and, 192, 196, 229 CD31 and, 8 CD34 and, 8, 229 classic, 218, 219, 222 clinical features, , 223, 224 cutaneous angiosarcoma of the face and scalp and, cytomegalovirus (CMV) and, 218 differential diagnosis, HHV -8 and, 11 histogenesis, 228 histopathologic features, hobnail hemangioma and, 157, 159,229 HPV antigens and, 218 hyaline globules in, 228 immunohistochemistry, 218, immunosuppressive form of, 218, IPEH and, 119 kaposiform hemangioendothelioma and, 177, 230 microvenular hemangioma and, nodular, 225, patch stage, , 228 pathogenesis, 219 spindle cell hemangioma and, 188, 230 spindle cells, , 229 treatment, 230 VEGFR-3 and, 8, 229 clinical features, histopathologic features, treatment, 302 Klippel-Trenaunay syndrome, 19,40,55-56,59, 89, 186 Langerhans cells, 2 Leiomyosarcoma glomangiosarcoma and, 273 Linear telangiectasia, 79 Lipoblastoma, 289 Louis-Bar syndrome, 82 Lymphangiectases, 195 clinical features, 95 histopathologic features, treatment, 96 Lymphangioma, 63, 195 acquired progressive, 191 circumscriptum, 63, 195 Lymphangiomatosis, 70 clinical features, 70 histopathologic features, immunohistochemistry, 72 treatment, 72 Lymphangiosarcomas, 258 Lymphedema angiosarcoma associated with, see Cutaneous angiosarcoma associated with lymphedema cutaneous angiosarcoma associated with, see Cutaneous angiosarcoma associated with lymphedema Maffucci's syndrome, 53-55, 58-59,67,186, 188 Malformation, defined, 27 Malignant angioendotheliomatosis, 128, see also Intravascular lymphomatosis Malignant glomus tumor (MGT), see Glomangiosarcoma Melanoblasts, 2 Kaposi's sarcoma-associated herpes virus (KSHV), Melanocytes, 2, Merkel cells, 2 Kasabach-Merritt syndrome, 47, , Mesoderm, 1 glomus tumors and, 198 Mibelli's angiokeratoma, 87 kaposiform hemangioendothelioma and, 177 Microvenular hemangioma, 161 tufted angioma and, 164, 166 clinical features, 161 Keratohyalin granules, 2 differential diagnosis, Kimura's disease histopathologic features, AHE and, 99, 102, Kaposi's sarcoma and,

7 Index POEMS syndrome and, 161 treatment, 162 Wiskott-Aldrich syndrome and, 161 Miliary hemangioma, 139 Molecular techniques, Multinucleate cell angiohistiocytoma clinical features, dermatofibroma and, differential diagnosis, histopathologic features, immunohistochemistry, 276 treatment, 277 Multiple endocrine neoplasia type 1 (MEN-I), 280 My 10, 8 Myofibroma, adult, Myofibromatosis,212 infantile, 212, 213 Myopericytes,215 Naevus vascularis mixtus, 38 Neonatal hemangiomatosis, 139 Neurofibromatosis type 2, 279 Nevus blue rubber bleb, see Blue rubber bleb nevus hypochromic, 29 Spitz, 236 telangiectatic, 30 Nevus anemicus clinical features, histopathologic features, 30 phakomatosis pigmentovascularis and, 19,20, 29 port wine stains and, 38 treatment, 30 Nevus araneus, see Spider angioma Nevus flammeus, see also Port wine stain clinical features, histopathologic features, immunohistochemistry, 43 nuchal-occipital, 37 pathogenesis, 43 phakomatosis pigmentovascularis and, 19,20, 21 pyogenic granuloma (PG) and, 106 treatment, 43 tufted angioma and, 164 Nevus of Ota, 19 Nevus oligemicus, 30 Nevus pigmentosus, 19 Nevus spilus, 19 Nevus verrucosus, 19 9D9F9,8 Nodular hidradenoma glomangiosarcoma and, 273 Oculocutaneous melanosis, 19 Oroya's fever, 116 Osler-Rendu-Weber syndrome, 57, 81, 82, 84 Pachydermoperiostosis, 100 Palisaded angioleiomyoma, 286 Papillary intralymphatic angioendothelioma, see Dabska's tumor Papular angioplasia, 99 Parkes-Weber syndrome, 40, 55 Pearly penile papules, 279, 280 Pericytes cutaneous myofibroma and, 215 glomus tumors as, 201 Periderm, 1 Periungual fibroma, 279 PHACES syndrome, 139 Phakomatosis pigmentovascu1aris clinical features, histopathologic features, nevus anemic us and, 19,20,29 nevus flammeus and, 19,20,21 pathogenesis, 20 port wine stains and, 41 treatment, 21 Phlebectasia, diffuse genuine, 34 Pinocytosis, 5 Plasma cell dyscrasia, 169 Platelet endothelial cell adhesion molecule-l (PECAM1), see CD31 Pleomorphic lipoma, 288 Plexuses, 2-4 POEMS syndrome, 161, 169 Polymerase chain reaction, 10 Polymorphous hemangioendothelioma, 250 Port wine stain, 21, 37-39, see also Nevus flammeus Progressive capillary hemangioma, 164 Proteus syndrome, 41 Pseudo-Kaposi's sarcoma, 123 clinical features, histopathologic features, treatment, Pseudopyogenic granuloma, 99 Purpuric dematoses, 134 Pyogenic granuloma GLUT-1 and, 10 Pyogenic granuloma (PG) atypical, 99 clinical features, histopathologic features, immunohistochemistry, 107 intravascular, see Intravascular pyogenic granuloma 317

8 318 nevus flammeus and, 106 spider angioma and, 73, 106 treatment, 109 QB-END 10,8 Radiation-induced cutaneous angiosarcoma, atypical vascular lesions and, benign lymphangioendothelioma and, benign lymphangiomatous papules and, benign vascular proliferations and, clinical features,, 263 fig histopathologic features, -265 lymphangiectases and, lymphangioma and, treatment, 265 Reactive angioendotheliomatosis, 128 clinical features, histopathologic features, immunohistochemistry, 129 treatment, 130 Retiform hemangioendothelioma, 245 angiosarcoma and, 245 clinical features, 245, 246 endovascular papillary angioendothelioma and, 243 epithelioid hemangioendothelioma and, 236, 245 histopathologic features, , hobnail hemangioendothelioma and, 243 hobnail hemangioma and, 157 immunohistochemistry, treatment, 246 Rhadinoviruses, 219 Riley-Smith syndrome, 56 Roberts' syndrome, 42 Rochalimaea, see Bartonella Rubinstein-Taybi syndrome, 42 Ruvalcaba-Myhre-Smith syndrome, 57 Ruvalcaba-Myhre syndrome, 57 Salmon patch, 37, 43 Scleromyxedema, 293 Self-healing pseudoangiosarcoma, 191 Senile angioma, see Cherry angioma Sinusoidal hemangioma clinical features, 182 histopathologic features, 182, 183 treatment, 182 Skin embryology, 1-2 functions, 1 lymphatic channels, 5-6 Index Spider angioma clinical features, 73 histopathologic features, 73 pyogenic granuloma (PG) and, 73, 106 treatment, 73 Spindle cell hemangioendothelioma, see Spindle cell hemangioma Spindle cell hemangioma, 59, 186 clinical features, 186, 188 differential diagnosis, 188 epithelioid hemangioendothelioma and, 186, 236 histopathologic features, 187, 188 immunohistochemistry, 188 Kaposi's sarcoma and, 188,230 Klippel-Trenaunay syndrome and, 186 Maffucci's syndrome and, 186, 188 treatment, 188 Spindle cell lipoma, 288 Spitz nevus, 236 Stewart-Bluefarb syndrome, 123, 124, 126 Stewart-Treves syndrome, 119,258 Stork bite, 37 Stratum germinativum, 1 Stratum granulosum, 1 corneum, 1 Stratum spino sum, 1 Sturge-Weber syndrome, 19,38,39-40 Subungual fibroma, 279 Sucquet-Hoyer canal, 5 Sucquet-Hoyer canals, 198 Sudoriparous angioma, 23 Superficial cutaneous lymphatic malformations clinical features, histopathologic features, 64 immunohistochemistry, 64 treatment, 64 Takatsuki's syndrome, 169 Targetoid hemosiderotic hemangioma, see Hobnail hemangioma TAR syndrome, 42 Telangiectases, Tuberous sclerosis, 279 Tufted angioma, 164 clinical features, 164, 166 differential diagnosis, 166 histopathologic features, 165, 166 immunohistochemistry, 166 infantile hemangioma and, 166 kaposiform hemangioendothelioma and, 166, 177 Kasabach-Merritt syndrome and, 164, 166 nevus flammeus and, 164 treatment, 166

9 Index Ulex europaeus lectin, 7 Unilateral nevoid telangiectasia, 79-80, 82, 83 Unilateral spider nevi, 79 Unilateral telangiectasia, 79 Vascular endothelial growth factor receptor-3, see VEGFR-3 Vascular malformations, defined, 15 Vascular proliferations classification, VEGFR-3, 7, 8 Venous lake, see Capillary aneurysm Venous malformations, 51 clinical features, histopathologic features, treatment, Verruga peruana, 116 bacillary angiomatosis and, 116 clinical features, 116, 117 histopathologic features, 116, 118 immunohistochemistry, 116 treatment, 116 Visceral hemangiomas, Vitiligo, 29 von Hippel-Lindau syndrome, 42 von Willebrand factor (vwf), 7-8, see also Factor VIII-related antigen Weibel-Palade bodies, 5 Wilson Jones' angiosarcoma, see Cutaneous angiosarcoma of the face and scalp Wiskott-Aldrich syndrome, 161 Wybum-Mason syndrome, 41

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