Rheumatol Int DOI 10.1007/s00296-014-3063-6
Letter to the Editor
A case of cutaneous sarcoid vasculitis with livedo and review of the literature Koya Obara · Hideki Maejima · Sumiyuki Mii · Chieko Katayama · Hiroshi Takasu · Yasuyuki Amoh
Received: 10 March 2014 / Accepted: 2 June 2014 © Springer-Verlag Berlin Heidelberg 2014
To the Editor, Cutaneous sarcoid vasculitis (CSV) is rare. Systemic vasculitis is an uncommon complication of sarcoidosis [1]. Sarcoid vasculitis (SV) is sometimes present in pulmonary sarcoid lesions [2], but rarely occurs in the skin. As the clinical features of CSV are variable [3–6], the diagnosis of CSV relies on histopathological examination of the cutaneous sarcoid lesion, and features depend on the depth and extent of vascular disease. We herein describe a case of CSV with granulomatous vasculitis and livedo. A 32-year-old woman presented with a 2-month history of asymptomatic livedo on both legs and lowered visual activity (Fig. 1). Laboratory investigations revealed elevated serum levels of angiotensin-converting enzyme (42.7 U/ml [normal range 8.3–21.4 U/ml]) and lysozyme (17.8 μg/ml [normal range 5.0–10.2 μg/ml]); other blood tests, including liver and renal function tests, were normal. The tuberculin test was negative. Computed tomography of the chest demonstrated bilateral hilar lymphadenopathy (BHL). Ophthalmologic examination revealed bilateral uveitis. The skin biopsy specimen of the livedo showed numerous naked granulomas in the mid-dermis and subcutaneous fat surrounded by small vessels. The walls of the small vessels had fibrinoid necrosis, and their lumens were narrowed by infiltrated granulomas lack of neutrophiles and eosinophiles (Fig. 2a, b). No pathogens were detected by Periodic acid-Schiff and Ziehl-Neelsen staining, polarization microscopic examination revealed no foreign body, K. Obara (*) · H. Maejima · S. Mii · C. Katayama · H. Takasu · Y. Amoh Department of Dermatology, Kitasato University School of Medicine, 1‑15‑1 Kitasato, Sagamihara, Kanagawa 252‑0374, Japan e-mail: [email protected]
and CD68 was positive for granuloma cells. The histopathological features were considered to be those of CSV. The abovementioned clinical and histopathological findings and laboratory results led to the diagnosis of CSV with granulomatous vasculitis. The livedo and BHL disappeared without any treatment approximately 2 months after onset. She was treated as steroid ophthalmic solution for uveitis, and her visual acuity improved. These diseases did not show recurrence until now. Livedo is caused by impaired perfusion and hypoxia in the skin; therefore, it may occur in the setting of vessel wall injury or intravascular obstruction that interrupts the circulation. Livedo occurs in peripheral atherosclerosis, autoimmune and collagen diseases, thrombosis, and in cholesterol embolization syndrome [7], but rarely in sarcoidosis [8, 9]. Cutaneous manifestations of sarcoidosis include lupus pernio, infiltrated plaques, maculopapular eruptions, subcutaneous nodules, infiltration of old scars, and erythema
Fig. 1 Asymptomatic livedo on the right lower leg
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Rheumatol Int
Fig. 2 a Microscopic examination of the livedo reveals multiple granulomas throughout the dermis (hematoxylin–eosin stain, original magnification ×40). b Necrotizing vasculitis in the dermis with
focal involvement of the small blood vessel walls (hematoxylin–eosin stain, original magnification ×200)
nodosum [9]. Livedo is an atypical manifestation of cutaneous sarcoidosis. Livedo in cutaneous sarcoidosis may be attributed to SV or peripheral microcirculatory disturbance due to infiltrated sarcoid granuloma without vasculitis [8, 10]. Sarcoid vasculitis is categorized as vasculitis associated with systemic disease in International Chapel Hill Consensus Conference criteria revised in 2012 [11]. Histologically, SV includes granulomatous vasculitis and leukocytoclastic vasculitis [1]. The skin lesions of SV are variable and include livedo [3, 8], erythema nodosum-like lesion [4], ulcers [5], and purpuric ulcerations [6], which are mostly located on the lower legs. The association between CSV and Propionibacterium acnes was reported by Asahina et al. [8]. Sarcoid vasculitis may resemble granulomatosis with polyangiitis (Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), but histopathological examination shows neutrophil-infiltrated granulomatous vasculitis in Wegener’s granulomatosis and eosinophil-infiltrated granulomatous vasculitis in Churg–Strauss syndrome, while naked granulomas are seen in SV. Clinical manifestations of SV range from glomerulonephritis with renal failure, transient cerebral ischemia, Takayasu arteritis-like disease with aortitis, pulmonary vessel involvement, aortic dissection, and involvement of the primary branches of the aorta manifesting as aneurysms and stenosis [1]. The SV patients who had involvement of large vessels may develop poor prognosis if they are not treated with corticosteroids or immunosuppressive agents. Even small vessel of CSV sometimes has required with immunosuppressive therapy [1, 3, 12]. Therefore, CSV is a one of the important clinical features of sarcoidosis and may be recognized systemic disease.
Conflict of interest We have no conflict of interests.
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References 1. Fernandes SRM, Singsen BH, Hoffman GS (2000) Sarcoidosis and systemic vasculitis. Semin Arthritis Rheum 30:33–46 2. Takemura T, Matsui Y, Saiki S, Mikami R (1992) Pulmonary vascular involvement in sarcoidosis: a report of 40 autopsy cases. Hum Pathol 23:1216–1223 3. Hayashi S, Hatamochi A, Hamasaki Y, Kitamura Y, Ishii Y, Fukuda T et al (2009) A case of sarcoidosis with livedo. Int J Dermatol 48:1217–1221 4. Takenoshita H, Yamamoto T (2010) Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjogren’s syndrome. Eur J Dermatol 20:640–641 5. Poonawalla T, Colome-Grimmer MI, Kelly B (2007) Ulcerative sarcoidosis in the leg with granulomatous vasculitis. Clin Exp Dermatol 33:282–286 6. Wei CH, Huang YH, Shih YC, Tseng FW, Yang CH (2010) Sarcoidosis with cutaneous granulomatous vasculitis. Australas J Dermatol 51:198–201 7. Mañá J, Marcoval J, Graells J, Salazar A, Peyrí J (1997) Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol 133:882–888 8. Asahina A, Miura K, Saito I, Oshikata C, Ishii N, Eishi Y (2013) Cutaneous sarcoidosis with livedoid lesions: evidence of the involvement of Propionibacterium acnes. J Dermatol 40:501–502 9. Maejima H, Nakahara C, Akira W, Katsuoka K (2010) A Case with four clinical cutaneous associations with sarcoidosis. Eur J Dermatol 20:505–506 10. Janette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitis. Arthritis Rheum 65:1–11 11. Carlson JA, Chen KR (2007) Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndrome. Am J Dermatopathol 29:32–43 12. Kawakami T, Soma Y (2011) Successful use of mizoribine in a patient with sarcoidosis and cutaneous vasculitis. Acta Derm Venereol 91:582–583
Letter to the Editor
A case of cutaneous sarcoid vasculitis with livedo and review of the literature Koya Obara · Hideki Maejima · Sumiyuki Mii · Chieko Katayama · Hiroshi Takasu · Yasuyuki Amoh
Received: 10 March 2014 / Accepted: 2 June 2014 © Springer-Verlag Berlin Heidelberg 2014
To the Editor, Cutaneous sarcoid vasculitis (CSV) is rare. Systemic vasculitis is an uncommon complication of sarcoidosis [1]. Sarcoid vasculitis (SV) is sometimes present in pulmonary sarcoid lesions [2], but rarely occurs in the skin. As the clinical features of CSV are variable [3–6], the diagnosis of CSV relies on histopathological examination of the cutaneous sarcoid lesion, and features depend on the depth and extent of vascular disease. We herein describe a case of CSV with granulomatous vasculitis and livedo. A 32-year-old woman presented with a 2-month history of asymptomatic livedo on both legs and lowered visual activity (Fig. 1). Laboratory investigations revealed elevated serum levels of angiotensin-converting enzyme (42.7 U/ml [normal range 8.3–21.4 U/ml]) and lysozyme (17.8 μg/ml [normal range 5.0–10.2 μg/ml]); other blood tests, including liver and renal function tests, were normal. The tuberculin test was negative. Computed tomography of the chest demonstrated bilateral hilar lymphadenopathy (BHL). Ophthalmologic examination revealed bilateral uveitis. The skin biopsy specimen of the livedo showed numerous naked granulomas in the mid-dermis and subcutaneous fat surrounded by small vessels. The walls of the small vessels had fibrinoid necrosis, and their lumens were narrowed by infiltrated granulomas lack of neutrophiles and eosinophiles (Fig. 2a, b). No pathogens were detected by Periodic acid-Schiff and Ziehl-Neelsen staining, polarization microscopic examination revealed no foreign body, K. Obara (*) · H. Maejima · S. Mii · C. Katayama · H. Takasu · Y. Amoh Department of Dermatology, Kitasato University School of Medicine, 1‑15‑1 Kitasato, Sagamihara, Kanagawa 252‑0374, Japan e-mail: [email protected]
and CD68 was positive for granuloma cells. The histopathological features were considered to be those of CSV. The abovementioned clinical and histopathological findings and laboratory results led to the diagnosis of CSV with granulomatous vasculitis. The livedo and BHL disappeared without any treatment approximately 2 months after onset. She was treated as steroid ophthalmic solution for uveitis, and her visual acuity improved. These diseases did not show recurrence until now. Livedo is caused by impaired perfusion and hypoxia in the skin; therefore, it may occur in the setting of vessel wall injury or intravascular obstruction that interrupts the circulation. Livedo occurs in peripheral atherosclerosis, autoimmune and collagen diseases, thrombosis, and in cholesterol embolization syndrome [7], but rarely in sarcoidosis [8, 9]. Cutaneous manifestations of sarcoidosis include lupus pernio, infiltrated plaques, maculopapular eruptions, subcutaneous nodules, infiltration of old scars, and erythema
Fig. 1 Asymptomatic livedo on the right lower leg
13
Rheumatol Int
Fig. 2 a Microscopic examination of the livedo reveals multiple granulomas throughout the dermis (hematoxylin–eosin stain, original magnification ×40). b Necrotizing vasculitis in the dermis with
focal involvement of the small blood vessel walls (hematoxylin–eosin stain, original magnification ×200)
nodosum [9]. Livedo is an atypical manifestation of cutaneous sarcoidosis. Livedo in cutaneous sarcoidosis may be attributed to SV or peripheral microcirculatory disturbance due to infiltrated sarcoid granuloma without vasculitis [8, 10]. Sarcoid vasculitis is categorized as vasculitis associated with systemic disease in International Chapel Hill Consensus Conference criteria revised in 2012 [11]. Histologically, SV includes granulomatous vasculitis and leukocytoclastic vasculitis [1]. The skin lesions of SV are variable and include livedo [3, 8], erythema nodosum-like lesion [4], ulcers [5], and purpuric ulcerations [6], which are mostly located on the lower legs. The association between CSV and Propionibacterium acnes was reported by Asahina et al. [8]. Sarcoid vasculitis may resemble granulomatosis with polyangiitis (Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome), but histopathological examination shows neutrophil-infiltrated granulomatous vasculitis in Wegener’s granulomatosis and eosinophil-infiltrated granulomatous vasculitis in Churg–Strauss syndrome, while naked granulomas are seen in SV. Clinical manifestations of SV range from glomerulonephritis with renal failure, transient cerebral ischemia, Takayasu arteritis-like disease with aortitis, pulmonary vessel involvement, aortic dissection, and involvement of the primary branches of the aorta manifesting as aneurysms and stenosis [1]. The SV patients who had involvement of large vessels may develop poor prognosis if they are not treated with corticosteroids or immunosuppressive agents. Even small vessel of CSV sometimes has required with immunosuppressive therapy [1, 3, 12]. Therefore, CSV is a one of the important clinical features of sarcoidosis and may be recognized systemic disease.
Conflict of interest We have no conflict of interests.
13
References 1. Fernandes SRM, Singsen BH, Hoffman GS (2000) Sarcoidosis and systemic vasculitis. Semin Arthritis Rheum 30:33–46 2. Takemura T, Matsui Y, Saiki S, Mikami R (1992) Pulmonary vascular involvement in sarcoidosis: a report of 40 autopsy cases. Hum Pathol 23:1216–1223 3. Hayashi S, Hatamochi A, Hamasaki Y, Kitamura Y, Ishii Y, Fukuda T et al (2009) A case of sarcoidosis with livedo. Int J Dermatol 48:1217–1221 4. Takenoshita H, Yamamoto T (2010) Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjogren’s syndrome. Eur J Dermatol 20:640–641 5. Poonawalla T, Colome-Grimmer MI, Kelly B (2007) Ulcerative sarcoidosis in the leg with granulomatous vasculitis. Clin Exp Dermatol 33:282–286 6. Wei CH, Huang YH, Shih YC, Tseng FW, Yang CH (2010) Sarcoidosis with cutaneous granulomatous vasculitis. Australas J Dermatol 51:198–201 7. Mañá J, Marcoval J, Graells J, Salazar A, Peyrí J (1997) Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol 133:882–888 8. Asahina A, Miura K, Saito I, Oshikata C, Ishii N, Eishi Y (2013) Cutaneous sarcoidosis with livedoid lesions: evidence of the involvement of Propionibacterium acnes. J Dermatol 40:501–502 9. Maejima H, Nakahara C, Akira W, Katsuoka K (2010) A Case with four clinical cutaneous associations with sarcoidosis. Eur J Dermatol 20:505–506 10. Janette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitis. Arthritis Rheum 65:1–11 11. Carlson JA, Chen KR (2007) Cutaneous vasculitis update: neutrophilic muscular vessel and eosinophilic, granulomatous, and lymphocytic vasculitis syndrome. Am J Dermatopathol 29:32–43 12. Kawakami T, Soma Y (2011) Successful use of mizoribine in a patient with sarcoidosis and cutaneous vasculitis. Acta Derm Venereol 91:582–583
