Pathology of Vascular Skin Lesions
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122 Sangüeza and Requena / Pathology of Vascular Skin Lesions 13. Albrecht S, Kahn HJ. Immunohistochemistry of intravascular papillary endothelial hyperplasia. J Cutan Pathol 1990;17:16–21. 14. Rosai J, Akerman LR. Intravascular atypical vascular proliferation. Arch Dermatol 1974;109:704–17. 15. Cooper PH. Vascular tumors. In: Farmer R, Hood AF, eds. Pathology of the Skin. Norwalk, CT, Appleton & Lange, 1990:804–46. 16. Pins MR, Rosenthal DI, Springfield DS, Rosemberg AE. Florid extravascular papillary endothelial hyperplasia (Masson’s pseudoangiosarcoma) presenting as a soft-tissue sarcoma. Arch Pathol Lab Med 1993;117:259–63. 17. Chen KT. Extravascular papillary endothelial hyperplasia. J Surg Oncol 1987;36:52–4. 18. Borrelli L, Ciniglio M, Faffulli N, Del Torto M. Intravascular papillary endothelial hyperplasia in the hand of a fencer. Pathologica 1992;84:551–6. 19. Inaloz HS, Patel G, Knight AG. Recurrent intravascular papillary endothelial hyperplasia developing from a pyogenic granuloma. J Eur Acad Dermatol Venereol 2000;15:156–8. 20. Hashimoto H, Daimaru Y, Enjoji M. Intravascular papillary endothelial hyperplasia. A clinicopatho- logic study of 91 cases. Am J Dermatopathol 1983;5:539–46. 21. Heyden G, Dahl I, Angervall L. Intravascular papillary endothelial hyperplasia in the oral mucosa. Oral Surg Oral Med Oral Pathol 1978;45:83–7. 22. Buchner A, Merrell PW, Carpenter WM, Leider AS. Oral intravascular papillary endothelial hyperpla- sia. J Oral Pathol Med 1990;19:419–22. 23. Stern Y, Braslavsky D, Shpitzer T, Feinmesser R. Papillary endothelial hyperplasia in the tongue: a benign lesion that may be mistaken for angiosarcoma. J Otolaryngol 1994;23:81–3. 24. Tosios K, Koutlas IG, Papanicolaou SI. Intravascular papillary endothelial hyperplasia of the oral soft tissues: report of 18 cases and review of the literature. J Oral Maxillofac Surg 1994;52:1263–8. 25. De Courten A, Fuffer R, Samson J, Lombardi T. Intravascular papillary endothelial hyperplasia of the mouth: report of six cases and literature review. Oral Dis 1999;5:175–8. 26. Wehbe MA, Otto NR. Intravascular papillary endothelial hyperplasia in the hand. J Hand Surg [Am] 1986;11:275–9. 27. Schwartz IS, Parris A. Cutaneous intravascular papillary endothelial hyperplasia: a benign lesion that may simulate angiosarcoma. Cutis 1982;29:66–9. 28. Dekio S, Tsujino Y, Jidoi J. Intravascular papillary endothelial hyperplasia on the penis: report of a case. J Dermatol 1993;20:10:657–9. 29. Cisco RW, McCormac RM. Intravascular papillary endothelial hyperplasia of the foot. J Foot Ankle Surg 1994;33:610–6. 30. Kato H. Two cases of intravascular papillary endothelial hyperplasia developing on the sole. J Dermatol 1996;23:655–7. 31. Stewart M, Smoller BR. Multiple lesions of intravascular papillary endothelial hyperplasia (Masson’s lesions). Arch Pathol Lab Med 1994;118:315–6. 32. Romani J, Puig L, Costa I, de Moragas JM. Masson’s intravascular papillary endothelial hyperplasia mimicking Stewart-Treves syndrome: report of a case. Cutis 1997;59:148–50. 33. Renshaw AA, Rosai J. Benign atypical vascular lesions of the lip. A study of 12 cases. Am J Surg Pathol 1993;17:557–65. 34. Levere SM, Barsky SH, Meals RA. Intravascular papillary endothelial hyperplasia: a neoplastic “actor” representing an exaggerated attempt at recanalization mediated by basic fibroblast growth factor. J Hand Surg [Am] 1994;19:559–64. 35. Katzman B, Caliguri DA, Klein DM, Nicastri AD, Chen P. Recurrent intravascular papillary endothelial hyperplasia. J Hand Surg [Br] 1997;22:113–5. 36. Yamamoto T, Marui T, Mizuno K. Recurrent intravascular papillary endothelial hyperplasia of the toes. Dermatology 2000;200:72–4. 07/Sangüeza/99-132/F 122 01/14/2003, 12:12 PM Chapter 7 / Cutaneous Vascular Hyperplasias 123 6. PSEUDO-KAPOSI’S SARCOMA Pseudo-Kaposi’s sarcoma is an unfortunate term applied to two completely different processes, acroangiodermatitis of Mali and the Stewart-Bluefarb syndrome. Acroangio- dermatitis of Mali (1) refers to skin lesions on the lower extremities of patients with chronic venous insufficiency, and Stewart-Bluefarb syndrome (2) is an arteriovenous malformation that clinically resembles Kaposi’s sarcoma. Clinical Features Clinically, the Stewart-Bluefarb syndrome usually presents early in life and involves the lower extremities of young adults unilaterally (Fig. 13). Purple papules and macules appear, which in some instances are painful and become ulcerated. The affected limb may have an increased temperature, with varicose veins, and a palpable thrill can be felt as an expression of the underlying arteriovenous shunt. Similar changes have been described at the site of cutaneous shunts for hemodyalisis (3–5) (Figs. 14 and 15) in paralyzed extremities (6,7) and in patients with Klippel-Trenaunay syndrome (8,9). Acroangio- dermatitis of Mali is simply exaggerated stasis dermatitis. The lesions are usually bilat- eral and develop in elderly patients with chronic venous insufficiency (Fig. 16). They have a predilection for the dorsal aspect of the feet and ankles. The lesions begin as violaceous macules and patches that develop slowly into soft, nontender, red to purple papules and nodules. Patients also present with scaly and indurated purple plaques, and Fig. 13. Stewart-Bluefarb syndrome in the lower extremity of young male patient. An underlying arteriovenous shunt was present. 07/Sangüeza/99-132/F 123 01/14/2003, 12:12 PM 124 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 14. Acroangiodermatitis involving the forearm and the hand, distally to the site of a cutaneous arteriovenous shunt for hemodialysis. Fig. 15. Acroangiodermatitis involving the inner aspect of the forearm distally to the site of a cutaneous arteriovenous shunt for hemodialysis. The lesion showed the appearance of a purpu- ric plaque. changes of stasis dermatitis are evident on the adjacent skin. Lesions identical to those of acroangiodermatitis of Mali may be seen in the distal part of an amputation stump (10,11) (Fig. 17) induced by a suction-socket prosthesis (12). HISTOPATHOLOGIC FEATURES Both types of pseudo-Kaposi’s sarcoma resemble Kaposi’s sarcoma clinically, but histopathologically they are completely different. In the Mali’s variant, the histopatho- logic findings are those of stasis dermatitis, namely, there is an increased number of thick- walled vessels lined by plump endothelial cells, extravasation of erythrocytes, and deposits of hemosiderin (Fig. 18). These changes are confined to the upper half of the dermis. In Stewart-Bluefarb syndrome, the entire dermis may be affected and, in large specimens, an arteriovenous shunt may be identified. Histopathologically, the differen- tial diagnosis with early stages of Kaposi’s sarcoma is usually straightforward, keeping in mind that the patch and plaque stages of Kaposi’s sarcoma are characterized by a proliferation of irregular jagged blood vessels, which are present around preexisting venules and adnexa and are lined by thin endothelial cells. As a rule, the papillary dermis is spared in the early stages of Kaposi’s sarcoma. Recently, the expression of CD34 antigen has been proposed as a feature to histopathologically distinguish lesions of pseudo-Kaposi’s sarcoma from authentic Kaposi’s sarcoma. CD34 positivity is detected in both endothelial cells and perivascular spindle cells of Kaposi’s sarcoma, whereas no such expression is seen in pseudo-Kaposi’s sarcoma (13). Furthermore; HHV-8 is not demonstrated in lesions of pseudo-Kaposi’s sarcoma (14). 07/Sangüeza/99-132/F 124 01/14/2003, 12:12 PM Chapter 7 / Cutaneous Vascular Hyperplasias 125 Fig. 16. Acroangiodermatitis of Mali involving the inner aspect of the ankle of an elderly male. Fig. 17. Acroangiodermatitis of Mali involving the distal part of an amputation stump. 07/Sangüeza/99-132/F 125 01/14/2003, 12:12 PM 126 Sangüeza and Requena / Pathology of Vascular Skin Lesions Fig. 18. Histopathologic features of acroangiodermatitis. (A) Scanning magnification show lobu- lar proliferations of capillaries at the superficial dermis. (B) Higher magnification demonstrates that the lobules are composed of plump endothelial cells with extravasation of erythrocytes and deposits of hemosiderin. TREATMENT Treatment of acroangiodermatitis of Mali is unsatisfactory and often unnecessary. If it is required, treatment of venous insufficiency of the lower extremities may be followed by slow improvement of the cutaneous lesions. Patients with Stewart-Bluefarb syndrome should consult with a vascular surgeon in order to embolize or excise the arteriovenous shunt under angiographic control (15). References 1. Mali JWH, Kuiper JT, Hamers AA. Acro-angiodermatitis of the foot. Arch Dermatol 1965;92:515–8. 2. Bluefarb SM, Adams LA. Arteriovenous malformation with angiodermatitis. Stasis dermatitis simulat- ing Kaposi’s disease. Arch Dermatol 1967;96:176–81. 3. Goldblum OM, Kraus E, Bronner AK. Pseudo-Kaposi’s sarcoma of the hand associated with an acquired, iatrogenic arteriovenous fistula. Arch Dermatol 1985;121:1038–40. 4. Landthaler M, Stolz W, Eckert F, Schmoeckel C, Braun-Falco O. Pseudo-Kaposi’s sarcoma occurring after placement of arteriovenous shunt. A case report with DNA content analysis. J Am Acad Dermatol 1989;21:499–505. 07/Sangüeza/99-132/F 126 01/14/2003, 12:12 PM Chapter 7 / Cutaneous Vascular Hyperplasias 127 5. Kim TH, Kim KH, Kang JS, Kim JH, Hwang IY. Pseudo-Kaposi’s sarcoma associated with acquired arteriovenous fistula. J Dermatol 1997;24:28–33. 6. Meynadier J, Malbos S, Guilhon JJ, et al. Pseudo-angiosarcomatose de Kaposi sur membre paralytique. Dermatologica 1980;16:190–7. 7. Landthaler M, Langehenke H, Holzmann H, Braun Falco O. Akroangiodermatitis Mali (“Pseudo- Kaposi”) and gelähmten Beinen. Hautarzt 1988;39:304–7. 8. Lund Kofoed M, Klemp P, Thestrup-Pedersen K. The Klippel-Trenaunay syndrome with acro- angiodermatitis (pseudo-Kaposi’s sarcoma). Acta Derm Venereol 1985;65:75–7. 9. Lyle WG, Given KS. Acroangiodermatitis (pseudo-Kaposi’s sarcoma) associated with Klippel- Trenaunay syndrome. Ann Plast Surg 1996;37:654–6. 10. Kolde G, Wörheide J, Baumgartner R, Bröcker EB.