Selim A

Sarcoidosis initially was described by Sir Jonathan Hutchinson in 1875, and cutaneous sarcoidosis (lupus pernio) was described by Besnier 1 in 1899. Sarcoidosis is a multisystem disease that may involve almost any organ system and, therefore, may present with various clinical manifestations.2 Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis. Recognition of cutaneous lesions is important because the lesions provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination.3 Because lesions can exhibit many different morphologies, cutaneous sarcoidosis is known as one of the "great imitators" in dermatology.4 Lesions of cutaneous sarcoidosis also can appear in preexisting scars, a condition known as scar sarcoidosis.5 The latter condition may be caused by mechanical trauma such as venipuncture, scars caused by infection such as herpes zoster,6 and tattoos.7 Treatment of cutaneous lesions can be frustrating. For patients with widespread disease, the most effective treatment is systemic glucocorticoids. The prognosis of sarcoidosis usually is good, in particular, if the condition predominantly or solely affects the skin.8 

Case Report
A 34-year-old man presented with a progressively enlarging lesion on his left calf. He reported that about 3 months prior he had developed a small ulceration at this location following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow beyond the borders of the previous ulceration and became raised with violaceous discoloration. The patient denied any history of excessive scarring or keloid formation after skin surgeries or trauma. There were no personal or family histories of granulomatous diseases. Results of a physical examination showed an erythematous-to-dusky plaque measuring approximately 4X3 cm (Figure 1) on the left calf with well-defined irregular borders and discrete papules on the internal aspect of the knee. No tender nodules on the shins were noticed, and no lymphadenopathy was present. Results from a review of systems and a routine chest x-ray were unremarkable. Results of a punch biopsy revealed changes consistent with sarcoid naked granulomas (Figure 2). The patient was started on topical potent corticosteroid tapes and experienced marked improvement.

Comment

Sarcoidosis occurs more frequently in females than in males, with reported ratios as high as 5:1. In the United States, black individuals are affected 3 to 4 times more often than white individuals.9 Sarcoidosis is found worldwide and in every race, though the incidence varies dramatically. In Europe, the disease affects white individuals more commonly than other races, and it affects Western Europeans more than Eastern Europeans. People from Scandinavia have one of the highest incidence rates at 64 cases per 100,000 population; in Poland, the incidence is 3 cases per 100,000 population. The disease is rare in Eskimos, Southeast Asians, New Zealand Maoris, and native Canadian populations.10,11 The difference in prevalence among certain populations in varying geographic locations suggests that ethnic susceptibility factors, as well as environmental factors, contribute to the etiology of sarcoidosis.11 Sarcoidosis is a multisystem disorder characterized by noncaseating, naked, epithelioid granulomas and commonly involves the hilar lymph nodes, lungs, skin, and eyes. The frequency of skin involvement in sarcoidosis is 10% to 30% of all cases, but the prevalence of particular types of cutaneous lesions varies among races, as well as among individual cases.12 Clinically, there is spontaneous development of livid or reddish-brown plaques on scars that were previously and mostly atrophic; this phenomenon occurs at varying intervals. Therefore, sarcoidosis should be considered in the differential diagnosis of an enlarging previously inactive scar. Lesions can develop in scars caused by mechanical trauma, such as in Kveim test sites, tuberculin test sites,5 sites that have received hyaluronic acid injection for wrinkles,13 sites of cosmetic tattoos,¹⁴ sites of previous laser surgery,15 and sites used for desensitization injections.16 Scar sarcoidosis has been reported following herpes zoster infection.17 Correctly diagnosing sarcoidosis may be a challenge. Unfortunately, no single test can lead to diagnosis of the condition. Patients are diagnosed with sarcoidosis when a compatible clinical or radiologic picture is present, along with histologic evidence of noncaseating granulomas, and when other potential causes, such as infections, are excluded. Cutaneous sarcoidosis varies greatly in its clinical presentation and has been labeled as one of the great dermatologic masqueraders.4,18 Maculopapular lesions can appear as xanthelasma, acne rosea, lupus erythematosus, or adenoma sebaceum. The differential diagnoses of plaques include lupus vulgaris, necrobiosis lipoidica, leprosy, leishmaniasis, psoriasis, and discoid lupus.4,18 The etiology of sarcoidosis is unknown, but several immune aberrations have been noted and are thought to play a role in its pathogenesis.19 Immune dysregulation has been theorized to result from a persistent antigen of low virulence that is poorly cleared by the immune system, leading to a chronic T cell of the TH1 subtype response and causing granuloma formation. Proposed antigens fall into 3 categories that include infectious, environmental, and autoantigens.20 The most common infectious agents implicated are Mycobacterium tuberculosis, Mycoplasma species, Corynebacterium species, spirochetes, atypical mycobacteria, Propionibacterium acnes, Borrelia burgdorferi, herpes simplex virus, Epstein-Barr virus, cytomegalovirus, coxsackievirus, rubella virus, Histoplasma species, Cryptococcus species, coccidioidomycosis, and sporotrichosis.21 Environmental antigens implicated include metals (eg, zirconium, aluminum, beryllium), organic dusts (eg, pine, pollen), inorganic dusts (eg, clay, soil, talc), and autoantigens (AV 2S3+ and HLA-DR17+).22 Genetic factors also are thought to play a role in the disease process.23 Familial clustering of cases has been reported. Monozygotic twins are 2 to 4 times more likely to have the disease than dizygotic twins.23 Certain HLA associations have been demonstrated; the most common allele found in sarcoidosis is HLA-B8. Other associated alleles include HLA-A1 and HLA-DR3.24 Most authors divide cutaneous lesions into specific and nonspecific categories.25 Specific skin lesions display noncaseating granulomas on biopsy. Nonspecific skin lesions display no granulomas on biopsy. Scar sarcoidosis is a specific form of cutaneous sarcoidosis in which old scars become infiltrated with noncaseating epithelioid cell granulomas. Typical sarcoid lesions are characterized by the presence of circumscribed granulomas of epithelioid cells with little or no necrosis. Granulomas usually are in the superficial dermis but may involve the full thickness of the dermis and extend to the subcutaneous tissue. Islands of epithelioid cells may have a few Langerhans giant cells.25 Giant cells may contain asteroid or Schaumann bodies; asteroid bodies are star-shaped eosinophilic structures; and Schaumann bodies are round or oval laminated structures that usually are calcified at the periphery.26 Granulomas are referred to as naked because they have only a sparse lymphocytic infiltrate at the margins of the granulomas. Fibrosis, if present, usually starts at the periphery and advances toward the center.26 The treatment of cutaneous sarcoidosis often is frustrating, and the condition often is refractory to therapy or recurs following successful treatment. Therapeutic approaches range from topical, intralesional, and systemic use of corticosteroids to systemic medications such as cytostatic drugs, chloroquine,27 allopurinol (300 mg/d),28 and thalidomide.29 For localized involvement of cutaneous sarcoidosis, topical or intralesional steroids are used. Physicians frequently use superpotent topical corticosteroids because the drugs occasionally are effective.30-32 However, the corticosteroid often does not adequately penetrate the skin lesion. Intralesional corticosteroids (eg, triamcinolone acetonide in a dose of 5 mg/mL) typically are more effective, with injections repeated at 2- to 3-week intervals.30,31 Alternative therapies include oral psoralen plus UVA, surgical excision, and laser treatment.32 The Q-switched ruby laser appears to be a rapid and effective means of treating scar sarcoidosis in traumatic tattoos without adverse effects.33 Surgical excision of small lesions or excision of larger lesions with skin grafting can be attempted but may cause the recurrence of hypertrophic and keloidal scarring.34 Systemic agents are reserved for widespread progressive lesions or for lesions that impair function. Systemic glucocorticoids are the most effective agents and are commonly used at slow tapering dosages, starting at 20 to 60 mg/d of oral prednisone for 4 to 5 weeks. However, there are many drawbacks to this therapy. Aside from the well-known complications of chronic steroid use, not all patients respond to systemic steroids.35 Patients who do respond frequently experience disease flare-ups after cessation of therapy. Many other medications may be used in refractory cases, including agents such as hydroxychloroquine sulfate,36 methotrexate,37 and thalidomide.29 Although randomized controlled trials are lacking, multiple anecdotal reports suggest the efficacy of these agents. The course and prognosis of sarcoidosis correlates with the mode of onset of the disease, the patient's race, and the presenting stage. In general, the prognosis of cutaneous sarcoidosis depends on systemic involvement. The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.³⁸ Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% of patients have a more chronic or progressive course. The mortality rate is 1% to 6%. Sarcoidosis can lead to death either from severe involvement of lung parenchyma, which leads to pulmonary fibrosis and respiratory failure,38,39 or from myocardial involvement, which leads to arrhythmias and cardiac failure.39 Other causes of significant morbidity and mortality include central nervous system involvement, blindness, pulmonary hemorrhage, renal insufficiency, hypopituitarism, and liver disease.35 Cutaneous sarcoidosis usually has a prolonged course. Papules and nodules tend to resolve over months or years, though plaques may be more resistant.19 As treatment is withdrawn, relapses are frequent, especially in black patients who tend to have more severe and prolonged symptoms.11 

Sarcoidosis initially was described by Sir Jonathan Hutchinson in 1875, and cutaneous sarcoidosis (lupus pernio) was described by Besnier 1 in 1899. Sarcoidosis is a multisystem disease that may involve almost any organ system and, therefore, may present with various clinical manifestations.2 Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis. Recognition of cutaneous lesions is important because the lesions provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination.3 Because lesions can exhibit many different morphologies, cutaneous sarcoidosis is known as one of the "great imitators" in dermatology.4 Lesions of cutaneous sarcoidosis also can appear in preexisting scars, a condition known as scar sarcoidosis.5 The latter condition may be caused by mechanical trauma such as venipuncture, scars caused by infection such as herpes zoster,6 and tattoos.7 Treatment of cutaneous lesions can be frustrating. For patients with widespread disease, the most effective treatment is systemic glucocorticoids. The prognosis of sarcoidosis usually is good, in particular, if the condition predominantly or solely affects the skin.8 

Case Report
A 34-year-old man presented with a progressively enlarging lesion on his left calf. He reported that about 3 months prior he had developed a small ulceration at this location following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow beyond the borders of the previous ulceration and became raised with violaceous discoloration. The patient denied any history of excessive scarring or keloid formation after skin surgeries or trauma. There were no personal or family histories of granulomatous diseases. Results of a physical examination showed an erythematous-to-dusky plaque measuring approximately 4X3 cm (Figure 1) on the left calf with well-defined irregular borders and discrete papules on the internal aspect of the knee. No tender nodules on the shins were noticed, and no lymphadenopathy was present. Results from a review of systems and a routine chest x-ray were unremarkable. Results of a punch biopsy revealed changes consistent with sarcoid naked granulomas (Figure 2). The patient was started on topical potent corticosteroid tapes and experienced marked improvement.

Comment

Sarcoidosis occurs more frequently in females than in males, with reported ratios as high as 5:1. In the United States, black individuals are affected 3 to 4 times more often than white individuals.9 Sarcoidosis is found worldwide and in every race, though the incidence varies dramatically. In Europe, the disease affects white individuals more commonly than other races, and it affects Western Europeans more than Eastern Europeans. People from Scandinavia have one of the highest incidence rates at 64 cases per 100,000 population; in Poland, the incidence is 3 cases per 100,000 population. The disease is rare in Eskimos, Southeast Asians, New Zealand Maoris, and native Canadian populations.10,11 The difference in prevalence among certain populations in varying geographic locations suggests that ethnic susceptibility factors, as well as environmental factors, contribute to the etiology of sarcoidosis.11 Sarcoidosis is a multisystem disorder characterized by noncaseating, naked, epithelioid granulomas and commonly involves the hilar lymph nodes, lungs, skin, and eyes. The frequency of skin involvement in sarcoidosis is 10% to 30% of all cases, but the prevalence of particular types of cutaneous lesions varies among races, as well as among individual cases.12 Clinically, there is spontaneous development of livid or reddish-brown plaques on scars that were previously and mostly atrophic; this phenomenon occurs at varying intervals. Therefore, sarcoidosis should be considered in the differential diagnosis of an enlarging previously inactive scar. Lesions can develop in scars caused by mechanical trauma, such as in Kveim test sites, tuberculin test sites,5 sites that have received hyaluronic acid injection for wrinkles,13 sites of cosmetic tattoos,¹⁴ sites of previous laser surgery,15 and sites used for desensitization injections.16 Scar sarcoidosis has been reported following herpes zoster infection.17 Correctly diagnosing sarcoidosis may be a challenge. Unfortunately, no single test can lead to diagnosis of the condition. Patients are diagnosed with sarcoidosis when a compatible clinical or radiologic picture is present, along with histologic evidence of noncaseating granulomas, and when other potential causes, such as infections, are excluded. Cutaneous sarcoidosis varies greatly in its clinical presentation and has been labeled as one of the great dermatologic masqueraders.4,18 Maculopapular lesions can appear as xanthelasma, acne rosea, lupus erythematosus, or adenoma sebaceum. The differential diagnoses of plaques include lupus vulgaris, necrobiosis lipoidica, leprosy, leishmaniasis, psoriasis, and discoid lupus.4,18 The etiology of sarcoidosis is unknown, but several immune aberrations have been noted and are thought to play a role in its pathogenesis.19 Immune dysregulation has been theorized to result from a persistent antigen of low virulence that is poorly cleared by the immune system, leading to a chronic T cell of the TH1 subtype response and causing granuloma formation. Proposed antigens fall into 3 categories that include infectious, environmental, and autoantigens.20 The most common infectious agents implicated are Mycobacterium tuberculosis, Mycoplasma species, Corynebacterium species, spirochetes, atypical mycobacteria, Propionibacterium acnes, Borrelia burgdorferi, herpes simplex virus, Epstein-Barr virus, cytomegalovirus, coxsackievirus, rubella virus, Histoplasma species, Cryptococcus species, coccidioidomycosis, and sporotrichosis.21 Environmental antigens implicated include metals (eg, zirconium, aluminum, beryllium), organic dusts (eg, pine, pollen), inorganic dusts (eg, clay, soil, talc), and autoantigens (AV 2S3+ and HLA-DR17+).22 Genetic factors also are thought to play a role in the disease process.23 Familial clustering of cases has been reported. Monozygotic twins are 2 to 4 times more likely to have the disease than dizygotic twins.23 Certain HLA associations have been demonstrated; the most common allele found in sarcoidosis is HLA-B8. Other associated alleles include HLA-A1 and HLA-DR3.24 Most authors divide cutaneous lesions into specific and nonspecific categories.25 Specific skin lesions display noncaseating granulomas on biopsy. Nonspecific skin lesions display no granulomas on biopsy. Scar sarcoidosis is a specific form of cutaneous sarcoidosis in which old scars become infiltrated with noncaseating epithelioid cell granulomas. Typical sarcoid lesions are characterized by the presence of circumscribed granulomas of epithelioid cells with little or no necrosis. Granulomas usually are in the superficial dermis but may involve the full thickness of the dermis and extend to the subcutaneous tissue. Islands of epithelioid cells may have a few Langerhans giant cells.25 Giant cells may contain asteroid or Schaumann bodies; asteroid bodies are star-shaped eosinophilic structures; and Schaumann bodies are round or oval laminated structures that usually are calcified at the periphery.26 Granulomas are referred to as naked because they have only a sparse lymphocytic infiltrate at the margins of the granulomas. Fibrosis, if present, usually starts at the periphery and advances toward the center.26 The treatment of cutaneous sarcoidosis often is frustrating, and the condition often is refractory to therapy or recurs following successful treatment. Therapeutic approaches range from topical, intralesional, and systemic use of corticosteroids to systemic medications such as cytostatic drugs, chloroquine,27 allopurinol (300 mg/d),28 and thalidomide.29 For localized involvement of cutaneous sarcoidosis, topical or intralesional steroids are used. Physicians frequently use superpotent topical corticosteroids because the drugs occasionally are effective.30-32 However, the corticosteroid often does not adequately penetrate the skin lesion. Intralesional corticosteroids (eg, triamcinolone acetonide in a dose of 5 mg/mL) typically are more effective, with injections repeated at 2- to 3-week intervals.30,31 Alternative therapies include oral psoralen plus UVA, surgical excision, and laser treatment.32 The Q-switched ruby laser appears to be a rapid and effective means of treating scar sarcoidosis in traumatic tattoos without adverse effects.33 Surgical excision of small lesions or excision of larger lesions with skin grafting can be attempted but may cause the recurrence of hypertrophic and keloidal scarring.34 Systemic agents are reserved for widespread progressive lesions or for lesions that impair function. Systemic glucocorticoids are the most effective agents and are commonly used at slow tapering dosages, starting at 20 to 60 mg/d of oral prednisone for 4 to 5 weeks. However, there are many drawbacks to this therapy. Aside from the well-known complications of chronic steroid use, not all patients respond to systemic steroids.35 Patients who do respond frequently experience disease flare-ups after cessation of therapy. Many other medications may be used in refractory cases, including agents such as hydroxychloroquine sulfate,36 methotrexate,37 and thalidomide.29 Although randomized controlled trials are lacking, multiple anecdotal reports suggest the efficacy of these agents. The course and prognosis of sarcoidosis correlates with the mode of onset of the disease, the patient's race, and the presenting stage. In general, the prognosis of cutaneous sarcoidosis depends on systemic involvement. The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.³⁸ Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% of patients have a more chronic or progressive course. The mortality rate is 1% to 6%. Sarcoidosis can lead to death either from severe involvement of lung parenchyma, which leads to pulmonary fibrosis and respiratory failure,38,39 or from myocardial involvement, which leads to arrhythmias and cardiac failure.39 Other causes of significant morbidity and mortality include central nervous system involvement, blindness, pulmonary hemorrhage, renal insufficiency, hypopituitarism, and liver disease.35 Cutaneous sarcoidosis usually has a prolonged course. Papules and nodules tend to resolve over months or years, though plaques may be more resistant.19 As treatment is withdrawn, relapses are frequent, especially in black patients who tend to have more severe and prolonged symptoms.11 

Sarcoidosis initially was described by Sir Jonathan Hutchinson in 1875, and cutaneous sarcoidosis (lupus pernio) was described by Besnier 1 in 1899. Sarcoidosis is a multisystem disease that may involve almost any organ system and, therefore, may present with various clinical manifestations.2 Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis. Recognition of cutaneous lesions is important because the lesions provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination.3 Because lesions can exhibit many different morphologies, cutaneous sarcoidosis is known as one of the "great imitators" in dermatology.4 Lesions of cutaneous sarcoidosis also can appear in preexisting scars, a condition known as scar sarcoidosis.5 The latter condition may be caused by mechanical trauma such as venipuncture, scars caused by infection such as herpes zoster,6 and tattoos.7 Treatment of cutaneous lesions can be frustrating. For patients with widespread disease, the most effective treatment is systemic glucocorticoids. The prognosis of sarcoidosis usually is good, in particular, if the condition predominantly or solely affects the skin.8 

Case Report
A 34-year-old man presented with a progressively enlarging lesion on his left calf. He reported that about 3 months prior he had developed a small ulceration at this location following a fall. With local wound care, the ulceration healed with a scar. The scar, however, continued to grow beyond the borders of the previous ulceration and became raised with violaceous discoloration. The patient denied any history of excessive scarring or keloid formation after skin surgeries or trauma. There were no personal or family histories of granulomatous diseases. Results of a physical examination showed an erythematous-to-dusky plaque measuring approximately 4X3 cm (Figure 1) on the left calf with well-defined irregular borders and discrete papules on the internal aspect of the knee. No tender nodules on the shins were noticed, and no lymphadenopathy was present. Results from a review of systems and a routine chest x-ray were unremarkable. Results of a punch biopsy revealed changes consistent with sarcoid naked granulomas (Figure 2). The patient was started on topical potent corticosteroid tapes and experienced marked improvement.

Comment

Sarcoidosis occurs more frequently in females than in males, with reported ratios as high as 5:1. In the United States, black individuals are affected 3 to 4 times more often than white individuals.9 Sarcoidosis is found worldwide and in every race, though the incidence varies dramatically. In Europe, the disease affects white individuals more commonly than other races, and it affects Western Europeans more than Eastern Europeans. People from Scandinavia have one of the highest incidence rates at 64 cases per 100,000 population; in Poland, the incidence is 3 cases per 100,000 population. The disease is rare in Eskimos, Southeast Asians, New Zealand Maoris, and native Canadian populations.10,11 The difference in prevalence among certain populations in varying geographic locations suggests that ethnic susceptibility factors, as well as environmental factors, contribute to the etiology of sarcoidosis.11 Sarcoidosis is a multisystem disorder characterized by noncaseating, naked, epithelioid granulomas and commonly involves the hilar lymph nodes, lungs, skin, and eyes. The frequency of skin involvement in sarcoidosis is 10% to 30% of all cases, but the prevalence of particular types of cutaneous lesions varies among races, as well as among individual cases.12 Clinically, there is spontaneous development of livid or reddish-brown plaques on scars that were previously and mostly atrophic; this phenomenon occurs at varying intervals. Therefore, sarcoidosis should be considered in the differential diagnosis of an enlarging previously inactive scar. Lesions can develop in scars caused by mechanical trauma, such as in Kveim test sites, tuberculin test sites,5 sites that have received hyaluronic acid injection for wrinkles,13 sites of cosmetic tattoos,¹⁴ sites of previous laser surgery,15 and sites used for desensitization injections.16 Scar sarcoidosis has been reported following herpes zoster infection.17 Correctly diagnosing sarcoidosis may be a challenge. Unfortunately, no single test can lead to diagnosis of the condition. Patients are diagnosed with sarcoidosis when a compatible clinical or radiologic picture is present, along with histologic evidence of noncaseating granulomas, and when other potential causes, such as infections, are excluded. Cutaneous sarcoidosis varies greatly in its clinical presentation and has been labeled as one of the great dermatologic masqueraders.4,18 Maculopapular lesions can appear as xanthelasma, acne rosea, lupus erythematosus, or adenoma sebaceum. The differential diagnoses of plaques include lupus vulgaris, necrobiosis lipoidica, leprosy, leishmaniasis, psoriasis, and discoid lupus.4,18 The etiology of sarcoidosis is unknown, but several immune aberrations have been noted and are thought to play a role in its pathogenesis.19 Immune dysregulation has been theorized to result from a persistent antigen of low virulence that is poorly cleared by the immune system, leading to a chronic T cell of the TH1 subtype response and causing granuloma formation. Proposed antigens fall into 3 categories that include infectious, environmental, and autoantigens.20 The most common infectious agents implicated are Mycobacterium tuberculosis, Mycoplasma species, Corynebacterium species, spirochetes, atypical mycobacteria, Propionibacterium acnes, Borrelia burgdorferi, herpes simplex virus, Epstein-Barr virus, cytomegalovirus, coxsackievirus, rubella virus, Histoplasma species, Cryptococcus species, coccidioidomycosis, and sporotrichosis.21 Environmental antigens implicated include metals (eg, zirconium, aluminum, beryllium), organic dusts (eg, pine, pollen), inorganic dusts (eg, clay, soil, talc), and autoantigens (AV 2S3+ and HLA-DR17+).22 Genetic factors also are thought to play a role in the disease process.23 Familial clustering of cases has been reported. Monozygotic twins are 2 to 4 times more likely to have the disease than dizygotic twins.23 Certain HLA associations have been demonstrated; the most common allele found in sarcoidosis is HLA-B8. Other associated alleles include HLA-A1 and HLA-DR3.24 Most authors divide cutaneous lesions into specific and nonspecific categories.25 Specific skin lesions display noncaseating granulomas on biopsy. Nonspecific skin lesions display no granulomas on biopsy. Scar sarcoidosis is a specific form of cutaneous sarcoidosis in which old scars become infiltrated with noncaseating epithelioid cell granulomas. Typical sarcoid lesions are characterized by the presence of circumscribed granulomas of epithelioid cells with little or no necrosis. Granulomas usually are in the superficial dermis but may involve the full thickness of the dermis and extend to the subcutaneous tissue. Islands of epithelioid cells may have a few Langerhans giant cells.25 Giant cells may contain asteroid or Schaumann bodies; asteroid bodies are star-shaped eosinophilic structures; and Schaumann bodies are round or oval laminated structures that usually are calcified at the periphery.26 Granulomas are referred to as naked because they have only a sparse lymphocytic infiltrate at the margins of the granulomas. Fibrosis, if present, usually starts at the periphery and advances toward the center.26 The treatment of cutaneous sarcoidosis often is frustrating, and the condition often is refractory to therapy or recurs following successful treatment. Therapeutic approaches range from topical, intralesional, and systemic use of corticosteroids to systemic medications such as cytostatic drugs, chloroquine,27 allopurinol (300 mg/d),28 and thalidomide.29 For localized involvement of cutaneous sarcoidosis, topical or intralesional steroids are used. Physicians frequently use superpotent topical corticosteroids because the drugs occasionally are effective.30-32 However, the corticosteroid often does not adequately penetrate the skin lesion. Intralesional corticosteroids (eg, triamcinolone acetonide in a dose of 5 mg/mL) typically are more effective, with injections repeated at 2- to 3-week intervals.30,31 Alternative therapies include oral psoralen plus UVA, surgical excision, and laser treatment.32 The Q-switched ruby laser appears to be a rapid and effective means of treating scar sarcoidosis in traumatic tattoos without adverse effects.33 Surgical excision of small lesions or excision of larger lesions with skin grafting can be attempted but may cause the recurrence of hypertrophic and keloidal scarring.34 Systemic agents are reserved for widespread progressive lesions or for lesions that impair function. Systemic glucocorticoids are the most effective agents and are commonly used at slow tapering dosages, starting at 20 to 60 mg/d of oral prednisone for 4 to 5 weeks. However, there are many drawbacks to this therapy. Aside from the well-known complications of chronic steroid use, not all patients respond to systemic steroids.35 Patients who do respond frequently experience disease flare-ups after cessation of therapy. Many other medications may be used in refractory cases, including agents such as hydroxychloroquine sulfate,36 methotrexate,37 and thalidomide.29 Although randomized controlled trials are lacking, multiple anecdotal reports suggest the efficacy of these agents. The course and prognosis of sarcoidosis correlates with the mode of onset of the disease, the patient's race, and the presenting stage. In general, the prognosis of cutaneous sarcoidosis depends on systemic involvement. The course is variable, ranging from self-limited acute episodes to a chronic debilitating disease that may result in death.³⁸ Spontaneous remissions occur in nearly two thirds of patients, but 10% to 30% of patients have a more chronic or progressive course. The mortality rate is 1% to 6%. Sarcoidosis can lead to death either from severe involvement of lung parenchyma, which leads to pulmonary fibrosis and respiratory failure,38,39 or from myocardial involvement, which leads to arrhythmias and cardiac failure.39 Other causes of significant morbidity and mortality include central nervous system involvement, blindness, pulmonary hemorrhage, renal insufficiency, hypopituitarism, and liver disease.35 Cutaneous sarcoidosis usually has a prolonged course. Papules and nodules tend to resolve over months or years, though plaques may be more resistant.19 As treatment is withdrawn, relapses are frequent, especially in black patients who tend to have more severe and prolonged symptoms.11 

Angiosarcoma is an aggressive neoplasm that predominantly affects elderly patients. Most cases appear on the scalp and face de novo; however, trauma, longstanding lymphedema, and irradiation are predisposing factors. Management includes a multidisciplinary team and may involve a combination of surgery, radiation, and chemotherapy tailored to the patient’s age and associated comorbidities.

Bruiselike patches on the face and scalp of elderly patients should raise the index of suspicion for angiosarcoma. A biopsy of the lesion and a workup for other organ involvement should be considered. We present the case of an elderly patient with an angiosarcoma and discuss the management and follow-up along with a brief review of the current literature. 

Case Report
In February 2002, an 80-year-old white man presented with approximately a 3-month history of a growing raised area on the nose (Figure). The patient first noticed a sudden onset of redness on the right side of his nose in December 2001. The redness gradually darkened over the next 3 months.

Examination revealed a 30-X20-mm purplish red plaque on the right side of the patient's nose. Results of a 4-mm punch biopsy showed an angiosarcoma. The patient was referred to an ear, nose, and throat physician with a special interest in oncology. He also was evaluated by an oncologist. Results of the patient's physical examination were unremarkable except for the skin lesion on his nose. Results of a complete blood count, x-ray, and computed tomography of the chest, as well as an abdominal ultrasound and magnetic resonance imaging of the head and neck, were unremarkable for masses, lymphadenopathy, or other significant findings. In March 2002, the patient underwent excision of the tumor followed by skin grafting. Six weeks after the operation, the patient was started on external beam radiation therapy. He was treated with 98 Gy over 49 days (2.0 Gy daily). Results of follow-up physical examinations by the multidisciplinary team did not reveal any signs or symptoms of tumor recurrence. On the patient's last follow-up visit in April 2005, he showed no evidence of recurrence of the lesion or metastasis. No follow-up photographs were taken. 

Comment

Cutaneous angiosarcoma of the face and scalp is a distinct entity among the angiosarcomas. It was first described in detail by Jones1 in 1964 as malignant angioendothelioma of the skin.2 This aggressive vascular neoplasm predominantly affects elderly patients (average age, 70 years).3 Men are affected twice as frequently as women. Men also tend to develop the disease at an earlier age. The tumor is localized mostly to the upper half of the face and the scalp.3 Predisposing factors in the onset of angiosarcoma include trauma, longstanding lymphedema, and irradiation of benign vascular lesions; however, most cases present with no obvious etiology.4 The clinical presentation of angiosarcoma is variable. Ill-defined bruiselike areas or facial edema with minimal erythema are the initial signs. Progressively more indurated plaques appear with nodular or ulcerated components. The neoplasm spreads quickly, centrifugally, and transdermally.5,6 Multifocality also is possible. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion.7 Unusual presentations have included yellowish plaques over the upper eyelids that resemble xanthelasma and cause ptosis,8 rosacealike lesions,9 and lesions presenting with scarring alopecia.10 Angiosarcoma presenting with intermittent angioedema of the face that comes and goes is another uncommon manifestation.11 Rhinophymalike features also should be considered as an unusual clinical manifestation of cutaneous angiosarcoma.12 Diagnosis often is delayed by the variable presentation and the benign appearance of the lesion, which simulates a bruise or a hemangioma. Retrospective studies show that clinical diagnosis of cutaneous angiosarcomas often is difficult.13 Other lesions that may need to be differentiated from angiosarcoma on the face include hemangiomas, Kaposi sarcomas, malignant melanomas, metastases, and vascular venous malformations. A summary of the characteristic features is included in the Table.

Pathologically, 2 main patterns of angiomas are recognized: angiomatous and solid.9 The angiomatous pattern is characterized by irregular, anastomosing vascular channels that dissect through the collagen. The vessels are lined by endothelial cells with features that range from normal-appearing endothelial cells to pleomorphic, hyperchromatic cells that exhibit multi-layering. Papillary processes may be present within the lumen. Numerous normal or abnormal mitotic figures are present as well. A dense mononuclear cell infiltrate is present and correlates with a better prognosis. In the solid form of angiosarcoma, tumor cells may be spindle or polygonal shaped. Vascular architecture may not be identified in the poorly differentiated areas. Reticulin staining highlights the vascular channels.9 Possible immunohistochemical markers for angiosarcoma include ulex europaeus 1 lectin (sensitive marker; used in conjunction with epithelial membrane antigen and cytokeratin to exclude epithelial tumor); factor VIII antigen (highly specific; low detection sensitivity); CD34 cells (highly sensitive; stains dermal dendrocytes, sweat gland basement membrane, and hematopoietic progenitor cells); and CD31 cells (highly sensitive; good specificity).7,19 Several differential diagnoses should be considered on pathology.20 Unlike benign vascular lesions, the well-differentiated angiomatous areas in angiosarcoma display cytologic atypia; multilayering, papillary structures; and irregular anastomosing blood vessels. In Kaposi sarcoma, cytologic atypia is less prominent and there is no endothelial multilayering.20 The optimal treatment of cutaneous angiosarcoma has not been defined.21 Generally, radical surgery and postoperative radiotherapy are advocated to treat patients with these tumors. In many patients, surgery often is not feasible because of the tumor's multifocal nature and local spread pattern.21 Therefore, the results have been poor. In a study by Mark et al,22 only 1 of 12 patients had the disease locally controlled. Holden et al23 reported 1 cure in 7 patients treated with surgery alone. The surgical aim is to resect all clinically identifiable disease.24 In areas of doubt, microscopic control of surgical margins may have a role in guiding the extent of resection. Achieving a negative margin frequently is difficult in angiosarcoma patients because of the extensive microscopic spread that is so common in this disease. Therefore, in trying to achieve a negative margin, a wound is created that almost never can be closed primarily. The reconstructive surgeon has a number of options for initial temporary coverage, as well as definitive reconstruction, including homograft skin, autologous skin graft, rotation flaps, and free flaps.24 Angiosarcomas usually respond to radiotherapy to some degree and most studies suggest that a combination of surgery and radiotherapy offers the best chance for long-term control.3,21 In 1 series of 28 angiosarcomas of the head and neck, Mark et al22 reported better survival after a median 32 months with combined surgery and radiotherapy compared with surgery alone. In cases unsuited for surgery, radiotherapy alone may be considered; however, usually only partial responses are achieved. Morrison et al21 suggested that moderate doses of radiation could control subclinical disease. Because angiosarcoma is a systemic disease, chemotherapy may be useful in its management.25 The efficacy of chemotherapy is undefined, with some studies reporting a beneficial effect and others suggesting no survival benefit. Chemotherapeutic agents used have included doxorubicin, cyclophosphamide, dacarbazine, actinomycin D, methotrexate, and vincristine.25 Systemic paclitaxel therapy has been used with encouraging results in 3 patients with angiosarcoma with regression of the lesions.26 Spieth and colleagues27 reported the effectiveness of 13-cis-retinoic acid and interferon alfa-2a combination therapy in a patient with recurring cutaneous angiosarcoma of the head after radical radiation treatment. There is no optimal treatment for angiosarcoma and the search for effective systemic treatment is needed. The prognosis in angiosarcoma is poor because of its high potential for metastasis.28 The 5-year survival rate is about 12%. Prognostic factors include the size of the tumor and mitotic counts, with tumors less than 10 cm in diameter and those with low mitotic counts having a better prognosis.28

Conclusion

Angiosarcomas are rare, aggressive tumors of vascular origin. They occur most often in areas of long-term sun-exposed skin in elderly patients, patients with longstanding lymphedema, or patients who have completed radiation therapy. The prognosis is poor and radical surgery is often required. In addition, radiation or chemotherapy may be considered as therapeutic options.

Angiosarcoma is an aggressive neoplasm that predominantly affects elderly patients. Most cases appear on the scalp and face de novo; however, trauma, longstanding lymphedema, and irradiation are predisposing factors. Management includes a multidisciplinary team and may involve a combination of surgery, radiation, and chemotherapy tailored to the patient’s age and associated comorbidities.

Bruiselike patches on the face and scalp of elderly patients should raise the index of suspicion for angiosarcoma. A biopsy of the lesion and a workup for other organ involvement should be considered. We present the case of an elderly patient with an angiosarcoma and discuss the management and follow-up along with a brief review of the current literature. 

Case Report
In February 2002, an 80-year-old white man presented with approximately a 3-month history of a growing raised area on the nose (Figure). The patient first noticed a sudden onset of redness on the right side of his nose in December 2001. The redness gradually darkened over the next 3 months.

Examination revealed a 30-X20-mm purplish red plaque on the right side of the patient's nose. Results of a 4-mm punch biopsy showed an angiosarcoma. The patient was referred to an ear, nose, and throat physician with a special interest in oncology. He also was evaluated by an oncologist. Results of the patient's physical examination were unremarkable except for the skin lesion on his nose. Results of a complete blood count, x-ray, and computed tomography of the chest, as well as an abdominal ultrasound and magnetic resonance imaging of the head and neck, were unremarkable for masses, lymphadenopathy, or other significant findings. In March 2002, the patient underwent excision of the tumor followed by skin grafting. Six weeks after the operation, the patient was started on external beam radiation therapy. He was treated with 98 Gy over 49 days (2.0 Gy daily). Results of follow-up physical examinations by the multidisciplinary team did not reveal any signs or symptoms of tumor recurrence. On the patient's last follow-up visit in April 2005, he showed no evidence of recurrence of the lesion or metastasis. No follow-up photographs were taken. 

Comment

Cutaneous angiosarcoma of the face and scalp is a distinct entity among the angiosarcomas. It was first described in detail by Jones1 in 1964 as malignant angioendothelioma of the skin.2 This aggressive vascular neoplasm predominantly affects elderly patients (average age, 70 years).3 Men are affected twice as frequently as women. Men also tend to develop the disease at an earlier age. The tumor is localized mostly to the upper half of the face and the scalp.3 Predisposing factors in the onset of angiosarcoma include trauma, longstanding lymphedema, and irradiation of benign vascular lesions; however, most cases present with no obvious etiology.4 The clinical presentation of angiosarcoma is variable. Ill-defined bruiselike areas or facial edema with minimal erythema are the initial signs. Progressively more indurated plaques appear with nodular or ulcerated components. The neoplasm spreads quickly, centrifugally, and transdermally.5,6 Multifocality also is possible. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion.7 Unusual presentations have included yellowish plaques over the upper eyelids that resemble xanthelasma and cause ptosis,8 rosacealike lesions,9 and lesions presenting with scarring alopecia.10 Angiosarcoma presenting with intermittent angioedema of the face that comes and goes is another uncommon manifestation.11 Rhinophymalike features also should be considered as an unusual clinical manifestation of cutaneous angiosarcoma.12 Diagnosis often is delayed by the variable presentation and the benign appearance of the lesion, which simulates a bruise or a hemangioma. Retrospective studies show that clinical diagnosis of cutaneous angiosarcomas often is difficult.13 Other lesions that may need to be differentiated from angiosarcoma on the face include hemangiomas, Kaposi sarcomas, malignant melanomas, metastases, and vascular venous malformations. A summary of the characteristic features is included in the Table.

Pathologically, 2 main patterns of angiomas are recognized: angiomatous and solid.9 The angiomatous pattern is characterized by irregular, anastomosing vascular channels that dissect through the collagen. The vessels are lined by endothelial cells with features that range from normal-appearing endothelial cells to pleomorphic, hyperchromatic cells that exhibit multi-layering. Papillary processes may be present within the lumen. Numerous normal or abnormal mitotic figures are present as well. A dense mononuclear cell infiltrate is present and correlates with a better prognosis. In the solid form of angiosarcoma, tumor cells may be spindle or polygonal shaped. Vascular architecture may not be identified in the poorly differentiated areas. Reticulin staining highlights the vascular channels.9 Possible immunohistochemical markers for angiosarcoma include ulex europaeus 1 lectin (sensitive marker; used in conjunction with epithelial membrane antigen and cytokeratin to exclude epithelial tumor); factor VIII antigen (highly specific; low detection sensitivity); CD34 cells (highly sensitive; stains dermal dendrocytes, sweat gland basement membrane, and hematopoietic progenitor cells); and CD31 cells (highly sensitive; good specificity).7,19 Several differential diagnoses should be considered on pathology.20 Unlike benign vascular lesions, the well-differentiated angiomatous areas in angiosarcoma display cytologic atypia; multilayering, papillary structures; and irregular anastomosing blood vessels. In Kaposi sarcoma, cytologic atypia is less prominent and there is no endothelial multilayering.20 The optimal treatment of cutaneous angiosarcoma has not been defined.21 Generally, radical surgery and postoperative radiotherapy are advocated to treat patients with these tumors. In many patients, surgery often is not feasible because of the tumor's multifocal nature and local spread pattern.21 Therefore, the results have been poor. In a study by Mark et al,22 only 1 of 12 patients had the disease locally controlled. Holden et al23 reported 1 cure in 7 patients treated with surgery alone. The surgical aim is to resect all clinically identifiable disease.24 In areas of doubt, microscopic control of surgical margins may have a role in guiding the extent of resection. Achieving a negative margin frequently is difficult in angiosarcoma patients because of the extensive microscopic spread that is so common in this disease. Therefore, in trying to achieve a negative margin, a wound is created that almost never can be closed primarily. The reconstructive surgeon has a number of options for initial temporary coverage, as well as definitive reconstruction, including homograft skin, autologous skin graft, rotation flaps, and free flaps.24 Angiosarcomas usually respond to radiotherapy to some degree and most studies suggest that a combination of surgery and radiotherapy offers the best chance for long-term control.3,21 In 1 series of 28 angiosarcomas of the head and neck, Mark et al22 reported better survival after a median 32 months with combined surgery and radiotherapy compared with surgery alone. In cases unsuited for surgery, radiotherapy alone may be considered; however, usually only partial responses are achieved. Morrison et al21 suggested that moderate doses of radiation could control subclinical disease. Because angiosarcoma is a systemic disease, chemotherapy may be useful in its management.25 The efficacy of chemotherapy is undefined, with some studies reporting a beneficial effect and others suggesting no survival benefit. Chemotherapeutic agents used have included doxorubicin, cyclophosphamide, dacarbazine, actinomycin D, methotrexate, and vincristine.25 Systemic paclitaxel therapy has been used with encouraging results in 3 patients with angiosarcoma with regression of the lesions.26 Spieth and colleagues27 reported the effectiveness of 13-cis-retinoic acid and interferon alfa-2a combination therapy in a patient with recurring cutaneous angiosarcoma of the head after radical radiation treatment. There is no optimal treatment for angiosarcoma and the search for effective systemic treatment is needed. The prognosis in angiosarcoma is poor because of its high potential for metastasis.28 The 5-year survival rate is about 12%. Prognostic factors include the size of the tumor and mitotic counts, with tumors less than 10 cm in diameter and those with low mitotic counts having a better prognosis.28

Conclusion

Angiosarcomas are rare, aggressive tumors of vascular origin. They occur most often in areas of long-term sun-exposed skin in elderly patients, patients with longstanding lymphedema, or patients who have completed radiation therapy. The prognosis is poor and radical surgery is often required. In addition, radiation or chemotherapy may be considered as therapeutic options.

Angiosarcoma is an aggressive neoplasm that predominantly affects elderly patients. Most cases appear on the scalp and face de novo; however, trauma, longstanding lymphedema, and irradiation are predisposing factors. Management includes a multidisciplinary team and may involve a combination of surgery, radiation, and chemotherapy tailored to the patient’s age and associated comorbidities.

Bruiselike patches on the face and scalp of elderly patients should raise the index of suspicion for angiosarcoma. A biopsy of the lesion and a workup for other organ involvement should be considered. We present the case of an elderly patient with an angiosarcoma and discuss the management and follow-up along with a brief review of the current literature. 

Case Report
In February 2002, an 80-year-old white man presented with approximately a 3-month history of a growing raised area on the nose (Figure). The patient first noticed a sudden onset of redness on the right side of his nose in December 2001. The redness gradually darkened over the next 3 months.

Examination revealed a 30-X20-mm purplish red plaque on the right side of the patient's nose. Results of a 4-mm punch biopsy showed an angiosarcoma. The patient was referred to an ear, nose, and throat physician with a special interest in oncology. He also was evaluated by an oncologist. Results of the patient's physical examination were unremarkable except for the skin lesion on his nose. Results of a complete blood count, x-ray, and computed tomography of the chest, as well as an abdominal ultrasound and magnetic resonance imaging of the head and neck, were unremarkable for masses, lymphadenopathy, or other significant findings. In March 2002, the patient underwent excision of the tumor followed by skin grafting. Six weeks after the operation, the patient was started on external beam radiation therapy. He was treated with 98 Gy over 49 days (2.0 Gy daily). Results of follow-up physical examinations by the multidisciplinary team did not reveal any signs or symptoms of tumor recurrence. On the patient's last follow-up visit in April 2005, he showed no evidence of recurrence of the lesion or metastasis. No follow-up photographs were taken. 

Comment

Cutaneous angiosarcoma of the face and scalp is a distinct entity among the angiosarcomas. It was first described in detail by Jones1 in 1964 as malignant angioendothelioma of the skin.2 This aggressive vascular neoplasm predominantly affects elderly patients (average age, 70 years).3 Men are affected twice as frequently as women. Men also tend to develop the disease at an earlier age. The tumor is localized mostly to the upper half of the face and the scalp.3 Predisposing factors in the onset of angiosarcoma include trauma, longstanding lymphedema, and irradiation of benign vascular lesions; however, most cases present with no obvious etiology.4 The clinical presentation of angiosarcoma is variable. Ill-defined bruiselike areas or facial edema with minimal erythema are the initial signs. Progressively more indurated plaques appear with nodular or ulcerated components. The neoplasm spreads quickly, centrifugally, and transdermally.5,6 Multifocality also is possible. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion.7 Unusual presentations have included yellowish plaques over the upper eyelids that resemble xanthelasma and cause ptosis,8 rosacealike lesions,9 and lesions presenting with scarring alopecia.10 Angiosarcoma presenting with intermittent angioedema of the face that comes and goes is another uncommon manifestation.11 Rhinophymalike features also should be considered as an unusual clinical manifestation of cutaneous angiosarcoma.12 Diagnosis often is delayed by the variable presentation and the benign appearance of the lesion, which simulates a bruise or a hemangioma. Retrospective studies show that clinical diagnosis of cutaneous angiosarcomas often is difficult.13 Other lesions that may need to be differentiated from angiosarcoma on the face include hemangiomas, Kaposi sarcomas, malignant melanomas, metastases, and vascular venous malformations. A summary of the characteristic features is included in the Table.

Pathologically, 2 main patterns of angiomas are recognized: angiomatous and solid.9 The angiomatous pattern is characterized by irregular, anastomosing vascular channels that dissect through the collagen. The vessels are lined by endothelial cells with features that range from normal-appearing endothelial cells to pleomorphic, hyperchromatic cells that exhibit multi-layering. Papillary processes may be present within the lumen. Numerous normal or abnormal mitotic figures are present as well. A dense mononuclear cell infiltrate is present and correlates with a better prognosis. In the solid form of angiosarcoma, tumor cells may be spindle or polygonal shaped. Vascular architecture may not be identified in the poorly differentiated areas. Reticulin staining highlights the vascular channels.9 Possible immunohistochemical markers for angiosarcoma include ulex europaeus 1 lectin (sensitive marker; used in conjunction with epithelial membrane antigen and cytokeratin to exclude epithelial tumor); factor VIII antigen (highly specific; low detection sensitivity); CD34 cells (highly sensitive; stains dermal dendrocytes, sweat gland basement membrane, and hematopoietic progenitor cells); and CD31 cells (highly sensitive; good specificity).7,19 Several differential diagnoses should be considered on pathology.20 Unlike benign vascular lesions, the well-differentiated angiomatous areas in angiosarcoma display cytologic atypia; multilayering, papillary structures; and irregular anastomosing blood vessels. In Kaposi sarcoma, cytologic atypia is less prominent and there is no endothelial multilayering.20 The optimal treatment of cutaneous angiosarcoma has not been defined.21 Generally, radical surgery and postoperative radiotherapy are advocated to treat patients with these tumors. In many patients, surgery often is not feasible because of the tumor's multifocal nature and local spread pattern.21 Therefore, the results have been poor. In a study by Mark et al,22 only 1 of 12 patients had the disease locally controlled. Holden et al23 reported 1 cure in 7 patients treated with surgery alone. The surgical aim is to resect all clinically identifiable disease.24 In areas of doubt, microscopic control of surgical margins may have a role in guiding the extent of resection. Achieving a negative margin frequently is difficult in angiosarcoma patients because of the extensive microscopic spread that is so common in this disease. Therefore, in trying to achieve a negative margin, a wound is created that almost never can be closed primarily. The reconstructive surgeon has a number of options for initial temporary coverage, as well as definitive reconstruction, including homograft skin, autologous skin graft, rotation flaps, and free flaps.24 Angiosarcomas usually respond to radiotherapy to some degree and most studies suggest that a combination of surgery and radiotherapy offers the best chance for long-term control.3,21 In 1 series of 28 angiosarcomas of the head and neck, Mark et al22 reported better survival after a median 32 months with combined surgery and radiotherapy compared with surgery alone. In cases unsuited for surgery, radiotherapy alone may be considered; however, usually only partial responses are achieved. Morrison et al21 suggested that moderate doses of radiation could control subclinical disease. Because angiosarcoma is a systemic disease, chemotherapy may be useful in its management.25 The efficacy of chemotherapy is undefined, with some studies reporting a beneficial effect and others suggesting no survival benefit. Chemotherapeutic agents used have included doxorubicin, cyclophosphamide, dacarbazine, actinomycin D, methotrexate, and vincristine.25 Systemic paclitaxel therapy has been used with encouraging results in 3 patients with angiosarcoma with regression of the lesions.26 Spieth and colleagues27 reported the effectiveness of 13-cis-retinoic acid and interferon alfa-2a combination therapy in a patient with recurring cutaneous angiosarcoma of the head after radical radiation treatment. There is no optimal treatment for angiosarcoma and the search for effective systemic treatment is needed. The prognosis in angiosarcoma is poor because of its high potential for metastasis.28 The 5-year survival rate is about 12%. Prognostic factors include the size of the tumor and mitotic counts, with tumors less than 10 cm in diameter and those with low mitotic counts having a better prognosis.28

Conclusion

Angiosarcomas are rare, aggressive tumors of vascular origin. They occur most often in areas of long-term sun-exposed skin in elderly patients, patients with longstanding lymphedema, or patients who have completed radiation therapy. The prognosis is poor and radical surgery is often required. In addition, radiation or chemotherapy may be considered as therapeutic options.