PATHOLOGY OF VASCULAR SKIN LESIONS - PART 2 pdf

20 Sangüeza and Requena / Pathology of Vascular Skin Lesions PATHOGENESIS The most commonly accepted opinion is that phakomatosis pigmentovascularis results from developmental abnormalities of the vasomotor nerves derived from the neural crest and melanocytes (30). It is thought that an alteration in the neural regulation of blood vessels could lead to the development of the vascular abnormalities seen in this condition. This is probably the explanation for the coexistence of nevus flammeus and nevus anemicus characteristic of this disease. The abnormal melanocytic component results from alterations during the migration of the neural crest-derived melanocytes, which produces lesions such as the nevus of Ota, nevus spilus, and mongolian spot. H ISTOPATHOLOGIC FEATURES Histopathologically, there are an increased number of dilated thin-walled capillaries and venules in the upper part of the reticular dermis, although occasionally superficial areas of subcutaneous fat are also involved. The melanocytic component consists of spindled-shaped dendritic melanocytes loaded with abundant melanin in their cytoplasm scattered between the collagen bundles of the dermis (Fig. 2). Sometimes, the number of spindled melanocytes is sparse; thus lesions of phakomatosis pigmentovascularis may be misinterpreted as nevus flammeus. Immunohistochemical stains with S-100 protein or HMB-45 are helpful in highlighting the melanocytic component. Table 1 Classification of Phakomatosis Pigmentovascularis Type Features I a,b a Nevus flammeus and nevus pigmentosus et verrucosus II a,b Nevus flammeus, mongolian spots, ± nevus anemicus III a,b Nevus flammeus, nevus spilus, ± nevus anemicus IV a,b Nevus flammeus, mongolian spots, nevus spilus, ± nevus anemicus a a, cutaneous involvement only; b, cutaneous and systemic involvement. Fig. 1. Clinical features of phakomatosis pigmentovascularis. (A) The anterior abdomen and the right thigh of this newborn show a combination of erythematous areas of nevus flammeus with bluish areas of dermal melanocytosis. (B)The buttocks and the posterior thighs of the same baby show a combination of similar features. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM20 Chapter 4 / Cutaneous Vascular Hamartomas 21 It is important to remember that lesions of nevus flammeus associated with phakomatosis pigmentovascularis are indistinguishable from port wine stain not associ- ated with melanocytic abnormalities. Ultrastructural studies indicate that nevus flammeus associated with phakomatosis pigmentovascularis is surrounded by perivascular nerve fibers, which are not present in isolated nevus flammeus (31). T REATMENT The vascular component of phakomatosis pigmentovascularis may cause psychologi- cal trauma to the patient, especially when it involves the face. In that case cosmetic camouflage may be indicated. Laser therapy is also capable of producing good results in treating nevus flammeus of phakomatosis pigmentovascularis (32). There is no description of a melanoma originating in the melanocytic component of phakomatosis pigmentovascularis. References 1. Ota M, Kawanura T, Ito N. Phacomatosis pigmentovascularis (Ota). Jpn J Dermatol 1947;52:1–3. 2. Hasegawa Y, Yashura M. Phakomatosis pigmentovascularis type IVa. Arch Dermatol 1985;121:651–5. 3. Guillaume JC, Evenou P, Charpentier P, Avril MF. Phacomatose pigmento-vasculaire type IIa. Ann Dermatol Venereol 1988;115:1113–5. 4. Mahroughan M, Mehregan AH, Mehregan DA. Phakomatosis pigmentovascularis. Report of a case. Pediatr Dermatol 1996;13:36–8. 5. Stadhouders-Keet SA, Glastra A, Van Vloten WA. Phakomatosis pigmentovascularis (type IIIa). Ned Tijdschr Geneeskd 1999;143:1337. 6. Cincinnati P, Carucci T, Rutiloni C. La facomatosi pigmento-vascolare. Minerva Pediatr 1996;48:225–8. 7. Murdoch SR, Keefe M. Phakomatosis pigmentovascularis type IIA in a Caucasian child. Pediatr Dermatol 2000;17:157. 8. Toda K. A new type of phacomatosis pigmentovascularis (Ota). Jpn J Dermatol 1966;76:47–51. 9. Hasegawa Y, Yasuhara M. A variant of phakomatosis pigmentovascularis. Sin Res (Osaka) 1979;21:178–86. 10. Hasegawa Y, Yasuhara M. Phakomatosis pigmentovascularis type IVa. Arch Dermatol 1985;121:651–5. 11. Noriega Sanchez A, Markand ON, Herndon JH. Oculocutaneous melanosis associated with the Sturge- Weber syndrome. Neurology 1972;22:256–62. 12. Furukawa T, Igata A, Toyokura Y, et al. Sturge-Weber and Klippel-Trenaunay syndrome with nevus of Ota and Ito. Arch Dermatol 1970;102:640–5. Fig. 2. Histopathologic features of phakomatosis pigmentovascularis. (A) Low-power magnifica- tion shows dilated vascular structures at different levels of the dermis. (B) Higher magnification shows the dilated and congestive capillary blood vessels and scattered spindle, dendritic melano- cytes with abundant melanin interstitially arranged between collagen bundles of the dermis. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM21 22 Sangüeza and Requena / Pathology of Vascular Skin Lesions 13. Sigg C, Pelloni F. Oligosymptomatic form of Klippel-Trenaunay-Weber syndrome associated with giant nevus spilus. Arch Dermatol 1989;125:1284–5. 14. Peyron N, Dereure O, Bessis D, Guilhou JJ, Guillot B. La phacomatose pigmento-vasculaire. A propos de 2 cas associés à une angiodysplasie. J Mal Vasc 1993;18:336–9. 15. Teekhasaenee C, Ritch R. Glaucoma in phakomatosis pigmentovascularis. Ophthalmology 1997; 104:150–7. 16. Hagiwara K, Uezato H, Nonaka S. Phacomatosis pigmentovascularis type IIb associated with Sturge- Weber syndrome and pyogenic granuloma. J Dermatol 1998;25:721–9. 17. Uysal G, Guven A, Ozhan B, Ozturk MH, Mutluay AH, Tulunay O. Phakomatosis pigmentovascularis with Sturge-Weber syndrome: a case report. J Dermatol 2000;27:467–70. 18. Guiglia MC, Prendiville JS. Multiple granular cell tumors associated with giant speckled lentiginous nevus and nevus flammeus in a child. J Am Acad Dermatol 1991;24:359–63. 19. Kikuchi I, Okazaki M. Congenital temporal alopecia in phakomatosis pigmentovascularis. J Dermatol 1982;9:485–7. 20. Kim HJ, Park KB, Yang JM, Park SH, Lee ES. Congenital triangular alopecia in phakomatosis pigmentovascularis: report of 3 cases. Acta Derm Venereol 2000;80:215–6. 21. Zahorcsek Z, Schmelas A, Schneider I. Progrediente zirkumskripte Lentiginose Phakomatosis pigmentovascularis III/A. Hautarzt 1988;39:519–23. 22. Gilliam AC, Ragge NK, Perez MI, Bolognia JL. Phakomatosis pigmentovascularis type IIb with iris mammillations. Arch Dermatol 1993;129:340–2. 23. Van Gysel D, Oranje AP, Stroink H, Simonsz HJ. Phakomatosis pigmentovascularis. Pediatr Dermatol 1996;13:33–5. 24. Di Landro A, Tadini GL, Marchesi L, Cainelli T. Phakomatosis pigmentovascularis: a new case with renal angiomas and some considerations about the classification. Pediatr Dermatol 1999;16:25–30. 25. Tsuruta D, Fukai K, Seto M, et al. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. Pediatr Dermatol 1999;16:35–8. 26. Joshi A, Garg VK, Agrawal S, Agarwalla A, Thakur A. Port-wine stain (nevus flammeus), congenital Becker’s nevus, café-au-lait-macule and lentiginides: phakomatosis pigmentovascularis type Ia—a new combination. J Dermatol 1999;26:834–6. 27. Huang C, Lee P. Phakomatosis pigmentovascularis IIb with renal anomaly. Clin Exp Dermatol 2000;25:51–4. 28. Cho S, Choi JH, Sung KJ, Moon KC, Koh JK. Phakomatosis pigmentovascularis type IIB with neuro- logic abnormalities. Pediatr Dermatol 2001;18:263. 29. Bielsa I, Paradelo C, Ribera M, Ferrandiz C. Generalized nevus spilus and nevus anemicus in a patient with a primary lymphedema: a new type of phakomatosis pigmentovascularis? Pediatr Dermatol 1998;15:293–5. 30. Libow LF. Phakomatosis pigmentovascularis type IIIb. J Am Acad Dermatol 1993;29:305–7. 31. Smoller BR, Rosen S. Port wine stains: a disease of altered neural modulation of blood vessels? Arch Dermatol 1986;122:177–9. 32. Ono I, Tateshita T. Phacomatosis pigmentovascularis type IIa successfully treated with two types of laser therapy. Br J Dermatol 2000;142:358–61. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM22 Chapter 4 / Cutaneous Vascular Hamartomas 23 2. ECCRINE ANGIOMATOUS HAMARTOMA Eccrine angiomatous hamartoma (EAH) refers to a cutaneous hamartoma that com- bines a proliferation of both eccrine glands and thin-walled blood vessels, usually of a capillary nature. So far, 45 examples of EAH have been reported in the literature (1–35), although some of the reported cases may be variations of normal skin (36) or simply vascular malformations located in volar skin, where eccrine glands are normally abundant. EAH was initially reported in 1859 by Lotzbeck (1), who described a lesion of angi- omatous appearance on the cheek of a child. Histopathologically, the lesion was com- posed of numerous clusters of eccrine glands within a stroma containing prominent blood vessels. In 1895 (2), Beier used the term sudoriparous angioma to describe a painful sudoriparous skin lesion of an angiomatous nature. In 1968, Hyman et al. (3) coined the term eccrine angiomatous hamartoma for this lesion and published a literature review on the subject. They noted that similar lesions were previously described under several designations, including secreting sudoriparous angiomatous hamartoma (4), functioning sudoriparous angiomatous hamartoma (5), nevus of sweat glands with angioma (6), and cavernous angiomatosis of the sweat ducts (7). C LINICAL FEATURES Clinically, most lesions of EAH present as solitary, erythematous nodules with an angiomatous appearance, although multiple lesions have also been described (4,7–10,34). They usually appear at birth or during early childhood, and several congenital examples have been described (8,11,12). In an isolated case, the lesion developed after radio- therapy (21). The most commonly affected areas are the acral zones, in particular the palms and soles (Fig. 3), although lesions on the face, neck, and trunk have also been reported. One patient with multiple lesions of EAH on the extensor surface of the wrists also had lesions on the knuckle pads (34). Biologically, the lesions are generally slow growing and asymptomatic, but pain and hyperhidrosis may be an occasional feature of this lesion. The pain is probably owing to nerve involvement (14,15) and the hyperhidrosis presumably results from stimulation of the eccrine component owing to a local increase in temperature caused in turn by the angiomatous component (8,10,14,16). Fig. 3. Clinical features of eccrine angiomatous hamartoma. (A) Nodular lesion involving the dorsum of the right toe in a newborn. (B) Nodular lesion involving the tip of the fifth finger. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM23 24 Sangüeza and Requena / Pathology of Vascular Skin Lesions Although the lesions tend to grow slowly, as mentioned before, there are cases that increase rapidly in size. In one reported case the increase in the size of the lesion was noted during pregnancy, indicating a possible hormonal influence. In this particular case, par- tial amputation of the involved finger was necessary because of severe pain (13). H ISTOPATHOLOGIC FEATURES The characteristic histopathologic features of EAH include lobules of mature eccrine glands and ducts closely associated with thin-walled blood vessels, usually of a capillary nature (Fig. 4), although large venous channels have also been reported (12). In addition to these two components that define the lesion, the presence of other structures including fatty tissue (17,27,30), hair follicles (18,19), apocrine glands (10), neurovascular glomic-like bodies (27), and occasional epidermal hyperplasia (32) has been described and lends further support to the hamartomatous nature of the EAH. Immunohistochemi- cal studies have demonstrated that antigens commonly found in eccrine glands, such as carcinoembryonic antigen (CEA) and S-100 protein were qualitatively diminished in the eccrine component of the EAH, whereas endothelial markers such as Ulex europaeus, CD34, CD44, and factor VIII-related antigen were expressed by endothelial cells of the vascular component (10,16). Immunoreactivity for gross cystic disease fluid protein-15 was detected in the eccrine gland component of one case (33). T REATMENT Although it is benign and slow growing, EAH is often painful and may require surgical excision. There have been reports, however, of troublesome lesions in which the pain spontaneously resolved after some time (15). Fig. 4. Histopathologic features of eccrine angiomatous hamartoma. (A) Scanning magnification shows numerous dilated vascular structures involving both the upper and deeper dermis. (B) Higher magnification demonstrates an abundant number of eccrine units intermingled with dilated thin-walled vascular structures. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM24 Chapter 4 / Cutaneous Vascular Hamartomas 25 References 1. Lotzbeck C. Ein Fall von Schweissdrüsengeschwulst an der Wauge. Virchows Arch Pathol Anat 1859;16:160. 2. Beier E. Über einen Fall von Naevus Subcutaneous (Virchow) mit hochgradiqer Hyperplasie der Knäuelodrüsen. Arch Dermat Syph 1895;31:337. 3. Hyman AB, Harris H, Brownstein MH. Eccrine angiomatous hamartoma. NY State J Med 1968;68: 2803–6. 4. Vilanova X, Piñol Aguade J, Castells A. Hamartoma angiomateux sudoripare sécretant. Dermatologica 1963;127:9–16. 5. Issa O. Hamartoma angiomatoso sudoriparo funcionante. Actas Dermosifiliogr 1964;55:361–5. 6. Söltz-Szötz K. Berich über Fall von Schweissdrüsen naevus Kombiniert mit einem Angiom. Z Hautkr 1958;24:189–92. 7. Archer BWC. Multiple cavernous angiomata of the sweat ducts associated with hemiplegia. Lancet 1927;2:595–6. 8. Domonkos AN, Suarez LS. Sudoriparous angioma. Arch Dermatol 1967;96:552–3. 9. Aloi F, Tomasini C, Pippione M. Eccrine angiomatous hamartoma: a multiple variant. Dermatology 1992;184:219–22. 10. Sulica RL, Kao GF, Sulica VJ, Penneys NS. Eccrine angiomatous hamartoma (nevus): immunohis- tochemical findings and review of the literature. J Cutan Pathol 1994;21:71–5. 11. Kikuchi J, Kukari Y, Inoves S. Painful eccrine angiomatous nevus on the sole. J Dermatol 1982;9:329–32. 12. Sanmartin O, Botella R, Alegre V, Martinez A, Aliaga A. Congenital eccrine angiomatous hamartoma. Am J Dermatopathol 1992;14:161–4. 13. Gabrielsen O, Elgjo K, Sommerschild H. Eccrine angiomatous hamartoma of the finger leading to amputation. Clin Exp Dermatol 1991;16:44–5. 14. Challa VR, Jona J. Eccrine angiomatous hamartoma: a rare skin lesion with diverse histological features. Dermatologica 1977;155:206–9. 15. Wolf R, Krakowski A, Dorfman B. Eccrine angiomatous hamartoma. A painful step. Arch Dematol 1989;125:1489–90. 16. Smith VC, Montesinos E, Revert A, Ramon D, Molina I, Jorda E. Eccrine angiomatous hamartoma: report of three patients. Pediatr Dermatol 1996;13:139–42. 17. Donati P, Amantea A, Balus I. Eccrine angiomatous hamartoma. A lipomatous variant. J Cutan Pathol 1989:16:227–9. 18. Zeller DJ. Goldman RL. Eccrine-pilar angiomatous hamartoma. Dermatologica 1971;143:100–4. 19. Velasco, JA, Almeida V. Eccrine-pilar angiomatous nevus. Dermatologica 1988;177:317–22. 20. Aloi FG, Molinero A, Ronco A, Pippione M. Nevo eccrino-angiomatoso. G Ital Dermatol Venereol 1989;124:235–6. 21. Dallot A, Chemaly P, Kemeny JL, et al. Hamartome angio-eccrine chez un adulte après radiothérapie. Ann Dermatol Venereol 1992;119:903–4. 22. Diaz-Landaeta L, Kerdel FA. Hyperhidrotic, painful lesion. Eccrine angiomatous hamartoma. Arch Dermatol 1993;129:107. 23. Torres JE, Martin RF, Sanchez JL. Eccrine angiomatous hamartoma. PR Health Sci J 1994;13:159–60. 24. Nair LV, Kurien AM. Eccrine angiomatous hamartoma. Int J Dermatol 1994;33:650–1. 25. Nakayama H, Mihara M, Hattori K, Mishima E, Shimao S. Eccrine angiomatous hamartoma of the sacral region. Acta Derm Venereol 1994;74:477. 26. Calderone DC, Glass LF, Seleznick M, Fenske NA. Eccrine angiomatous hamartoma. J Dermatol Surg Oncol 1994;20:837–8. 27. Damiani S, Riccioni L. Palmar cutaneous hamartoma. Am J Dermatopathol 1998;20:65–8. 28. Michel JL, Secchi T, Balme B, Barrut D, Thomas L, Moulin G. Hamartome angio-eccrine congenital. Ann Dermatol Venereol 1997;124:623–5. 29. Kwon OC, Oh ST, Kim SW, Park GS, Cho BK. Eccrine angiomatous hamartoma. Int J Dermatol 1998;37:787–9. 30. Cebreiro C, Sanchez Aguilar D, Gomez Centeno P, Fernandez Redondo V, Toribio J. Eccrine angioma- tous hamartoma: report of seven cases. Clin Exp Dermatol 1998;23:267–70. 31. Nakatsui TC, Schloss E, Krol A, Lin AN. Eccrine angiomatous hamartoma: report of a case and literature review. J Am Acad Dermatol 1999;41:109–11. 32. Tsuji T, Sawada H. Eccrine angiomatous hamartoma with verrucous features. Br J Dermatol 1999;141:167–9. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM25 26 Sangüeza and Requena / Pathology of Vascular Skin Lesions 33. Tanaka M, Shimizu S, Miyakawa S. Hypertrophic eccrine glands in eccrine angiomatous hamartoma produce gross cystic disease fluid protein 15. Dermatology 2000;200:336–7. 34. Morell DS, Ghali FE, Stahr BJ, McCauliffe DP. Eccrine angiomatous hamartoma: a report of symmetric and painful lesions of the wrists. Pediatr Dermatol 2001;18:117–9. 35. Lee SY, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Congenital eccrine angiomatous hamartoma: report of two patients. J Dermatol 2001;28:338–40. 36. Laeng RH, Heilbrunner J, Itin PH. Late-onset eccrine angiomatous hamartoma: clinical, histological and imaging findings. Dermatology 2001;203:70–4. 04/Sangüeza/19-26/F 01/14/2003, 10:52 AM26 Chapter 5 / Cutaneous Vascular Malformations 27 5 Cutaneous Vascular Malformations CONTENTS NEVUS ANEMICUS CUTIS MARMORATA TELANGIECTATICA CONGENITA NEVUS FLAMMEUS HYPERKERATOTIC VASCULAR STAINS VENOUS MALFORMATIONS SUPERFICIAL CUTANEOUS LYMPHATIC MALFORMATIONS CYSTIC LYMPHATIC MALFORMATIONS (CYSTIC HYGROMAS) LYMPHANGIOMATOSIS Malformation denotes an abnormal structure that results from an aberration in embryologic development. Although the term malformation is conventionally used as a synonym for hamartoma, the two are different, because hamartoma refers to a potpourri of tissue elements normally present at a particular site. Vascular malformations can be either functional or anatomic. In the case of functional abnormalities, the changes are related mostly to physiologic changes, as is the case for nevus anemicus. In contrast, anatomic vascular malformations exhibit evident morphologic abnormalities of the involved vessels. Anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies (Table 1). Clinically, it is important to separate the vascular malformations into those of low flow and high flow. Low-flow 27 Table 1 Cutaneous Vascular Malformations Functional Nevus anemicus Anatomical Capillary Cutis marmorata telangiectatica congenita Nevus flammeus (port wine stain) Hyperkeratotic vascular stains Venous and arterial Lymphatic Superficial lymphatic Deep (cystic) lymphatic Lymphangiomatosis Combined vascular malformations 05/Sangüeza/27-72/F 01/14/2003, 11:21 AM27 28 Sangüeza and Requena / Pathology of Vascular Skin Lesions abnormalities include malformations of capillary, venous, lymphatic, or combination, whereas high-flow abnormalities include arteriovenous malformations (1). References 1. Mulliken JB. Classification of vascular birthmarks. In: Mulliken JB, Young AE, eds. Vascular Birth- marks. Hemangiomas and Malformations. Philadelphia, WB Saunders, 1988;24–37. 05/Sangüeza/27-72/F 01/14/2003, 11:21 AM28 Chapter 5 / Cutaneous Vascular Malformations 29 1. NEVUS ANEMICUS CLINICAL FEATURES Nevus anemicus is an uncommon congenital vascular malformation observed more frequently in women than in men. Clinically, the lesion consists of a localized circum- scribed, pale macule with irregular margins occasionally surrounded by satellite macules (1–14). Although the upper chest is the most commonly affected site (Fig. 1), it may occur in any part of the body. Under diascopic pressure, the lesion becomes indistinguishable from the blanched surrounding skin. Wood’s lamp examination does not accentuate the lesion, and the application of friction, cold, or heat does not induce erythema in the involved areas. All these maneuvers are helpful in distinguishing nevus anemicus from vitiligo, hypochromic nevus, and other hypomelanosis. Nevus anemicus can be an addi- tional feature in types II, III, and IV of phakomatosis pigmentovascularis (5,9,12,14). The presence of persistent, localized areas of livid erythema, caused by an increase in the vasoconstrictor tone of the thermoregulatory vessels of the involved skin and leading to relative stasis in the superficial nutritional vasculature, is considered a clinical variant Fig. 1. Clinical features of a nevus anemicus involving the anterior chest of an adult woman. (A) Hypochromic macule on the right anterior chest. (B) Rubbing of the lesion results in peripheral erythema secondary to vasodilation in adjacent noninvolved skin, whereas the lesion of nevus anemicus remains a whitish color. 05/Sangüeza/27-72/F 01/14/2003, 11:21 AM29 [...]... A, Hata Y Multiple anemic macules on the arms: a variant form of nevus anemicus? Dermatology 20 00 ;20 1:180–3 16 Plantin P, Schoenlaub P Multiple anemic macules on the arms: not a variant form of nevus anemicus Dermatology 20 01 ;20 2 :27 1 2 05/Sangüeza /2 7-7 2/ F 31 01/14 /20 03, 11 :21 AM 32 Sangüeza and Requena / Pathology of Vascular Skin Lesions 2 CUTIS MARMORATA TELANGIECTATICA CONGENITA Cutis marmorata telangiectatica... Catsman-Berrevoets CE Cutis marmorata telangiectatica congenita Int J Dermatol 19 92; 31 :24 9– 52 51 Pehr K, Moroz B Cutis marmorata telangiectatica congenita: long-term follow-up, review of the literature and report of a case in conjunction with congenital hypothyroidism Pediatr Dermatol 1993;10:6–11 05/Sangüeza /2 7-7 2/ F 35 01/14 /20 03, 11 :21 AM 36 Sangüeza and Requena / Pathology of Vascular Skin Lesions 52. .. stains (1, 12, 14–18) Small and superficial lesions can be treated by electrosurgery and with argon laser (22 ), but because of the depth of extension of most of these lesions (they usually extend into subcutaneous tissue), this superficial therapeutic approach is usually followed by recurrence 05/Sangüeza /2 7-7 2/ F 47 01/14 /20 03, 11 :21 AM 48 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig... Clinical features of a venous malformation involving the left side of the face of an adult woman The lesion was present at birth 05/Sangüeza /2 7-7 2/ F 51 01/14 /20 03, 11 :21 AM 52 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 14 Venous malformation involving the glans penis lesions of multiple plaque-like glomangiomas These lesions show a blue color and can mimic the appearance of venous malformations,... fluxmetry of the involved skin has shown evidence of functional disturbance, which may be the expression of an α-adrenergic innervation deficit of the cutaneous terminal blood vessels (58) 05/Sangüeza /2 7-7 2/ F 33 01/14 /20 03, 11 :21 AM 34 Sangüeza and Requena / Pathology of Vascular Skin Lesions The differential diagnosis of CMTC is with Bockenheimer’s syndrome or diffuse genuine phlebectasia ( 12, 59,60)... a review of the literature Surv Ophthalmol 1976 ;20 :415–31 22 Uram M, Zubillaga C The cutaneous manifestations of Sturge-Weber syndrome J Clin Neuroophthalmol 19 82; 2:145–8 23 Jacobs AH Sturge-Weber syndrome without port-wine nevus Pediatrics 1977; 60:785–6 24 Andriola M, Stolfi J Sturge-Weber syndrome: report of an atypical case Am J Dis Child 19 72; 123 :507–10 25 Crosley CJ, Binet EF Sturge-Weber syndrome:... Proteus syndrome—the association of hemihypertrophy, macrodactyly, verrucous epidermal nevus, vascular malformations, soft subcutaneous masses, and cerebriform overgrowth of the palmar or plantar surfaces of the hypertrophied limb (49–51) 05/Sangüeza /2 7-7 2/ F 41 01/14 /20 03, 11 :21 AM 42 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 10 Histopathologic features of nevus flammeus (A) Low power... increased number of mast cells has been described in the dermis of the lesions of nevus flammeus (58) When angiomatous nodules develop in a patch of 05/Sangüeza /2 7-7 2/ F 42 01/14 /20 03, 11 :21 AM Chapter 5 / Cutaneous Vascular Malformations 43 nevus flammeus, they are made up either of an aggregation of numerous thin-walled vessels whose lumina are of different calibers (59) or, in other instances, they are... Venereol 19 92; 72: 303–4 22 Newton JH, McGibbon DH The treatment of multiple angiokeratomata with argon laser Clin Exp Dermatol 1987; 12: 23–5 05/Sangüeza /2 7-7 2/ F 50 01/14 /20 03, 11 :21 AM Chapter 5 / Cutaneous Vascular Malformations 51 5 VENOUS MALFORMATIONS In the past, venous malformations have been inaccurately known as “cavernous” hemangiomas They are malformations, not neoplasms, and consist of slow-flowing,... 01/14 /20 03, 11 :21 AM 40 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig 8 Klippel-Trenaunay syndrome Extensive nevus flammeus involving the posterior aspect of a hypertrophic lower extremity drome, although there is no correlation between the extent of the cutaneous lesions and the extent of leptomeningeal involvement Some authors admit to the possibility of a forme fruste of Sturge-Weber . melano- cytes with abundant melanin interstitially arranged between collagen bundles of the dermis. 04/Sangüeza/1 9 -2 6/F 01/14 /20 03, 10: 52 AM21 22 Sangüeza and Requena / Pathology of Vascular Skin Lesions 13 skin, whereas the lesion of nevus anemicus remains a whitish color. 05/Sangüeza /2 7-7 2/ F 01/14 /20 03, 11 :21 AM29 30 Sangüeza and Requena / Pathology of Vascular Skin Lesions of nevus anemicus (8) expression of an α-adrenergic innervation deficit of the cutaneous terminal blood vessels (58). 05/Sangüeza /2 7-7 2/ F 01/14 /20 03, 11 :21 AM33 34 Sangüeza and Requena / Pathology of Vascular Skin Lesions The