130
Letters to the Editor / Joint Bone Spine 82 (2015) 129–137 [4] Hamaoka T, Madewell JE, Podoloff DA, et al. Bone imaging in metastatic breast cancer. J Clin Oncol 2004;22:2942–53. [5] Whitlock JP, Evans AJ, Jackson L, et al. Imaging of metastatic breast cancer: distribution and radiological assessment at presentation. Clin Oncol (R Coll Radiol) 2001;13:181–6. [6] Umeoka S, Koyama T, Miki Y, et al. Pictorial review of tuberous sclerosis in various organs. Radiographics 2008;28:e32. [7] Baskin Jr HJ. The pathogenesis and imaging of the tuberous sclerosis complex. Pediatr Radiol 2008;38:936–52. [8] Song L, Zhang Y, Zhang W. Bone scintigraphy may help differentiate bone sclerotic lesions from osteoblastic metastases in tuberous sclerosis patients with concomitant pulmonary adenocarcinoma. Clin Imaging 2013;37:382–5.
Constance de Margerie-Mellon a,∗ Rachid Kaci b Jean-Denis Laredo a Valérie Bousson a a Department of Osteoarticular Radiology, Université Paris Diderot, hôpital Lariboisière, 2, rue Ambroise-Paré, Sorbonne Paris Cité, 75475 Paris cedex 10, France b Department of Pathology, université Paris Diderot, hôpital Lariboisière, AP–HP, 2, rue Ambroise-Paré, Sorbonne Paris Cité, 75475 Paris cedex 10, France ∗ Corresponding
author. Department of radiology, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75475 Paris cedex 10, France. E-mail address: [email protected] (C. de Margerie-Mellon) Accepted 5 August 2014 Available online 17 September 2014
http://dx.doi.org/10.1016/j.jbspin.2014.08.002
Severe skin involvement in type II cryoglobulinemia successfully treated with thalidomide
a r t i c l e Fig. 2. Thoraco-abdomino-pelvic CT scan, sagittal reformation. Diffuse small blastic lesions of vertebral bodies and pelvic bones. Lytic lesion of T12 vertebral body (arrow).
In conclusion, since breast cancer is relatively frequent, atypical presentation should be recognized. Most of the time, bone metastases are heterogeneous with a lytic pattern. However, bone lesions can be small, blastic and homogeneous, and the breast tumor hidden. Rare diagnosis as TS can only be made after definitive elimination of frequent diseases and verification of complete compliance with the diagnostic criteria. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13: 624–8. [2] Orvieto E, Maiorano E, Bottiglieri L, et al. Clinicopathologic characteristics of invasive lobular carcinoma of the breast: results of an analysis of 530 cases from a single institution. Cancer 2008;113:1511–20. [3] Sastre-Garau X, Jouve M, Asselain B, et al. Infiltrating lobular carcinoma of the breast: clinicopathologic analysis of 975 cases with reference to data on conservative therapy and metastatic patterns. Cancer 1998;77:113–20.
i n f o
Keywords: Mixed cryoglobulinemia Ulcers Auricles Thalidomide
Mixed cryoglobulinemia (MC) is a small vessel vasculitis associated with serum immune-complexes containing immunoglobulins that precipitate at temperatures below 37 ◦ C [1]. We report the case of a patient with severe skin involvement successfully treated with thalidomide after the failure of conventional therapy. A 68-years-old man came to our attention on 2009 with a 3 year-history of lower limb purpuric-livedoid plaques and ulcers (Fig. S1; see the supplementary material associated with this article online). A diagnosis of erythema elevatum diutinum had been made based on a histological finding of leukocytoclastic vasculitis with microthrombosis. He had been treated with dapsone (50 mg/day) and low-dose methylprednisolone. Some periodical relapses had been well controlled with an increase of steroids, until an extensive ulceration of the lower limbs and abdomen arised. A biopsy confirmed a leukocytoclastic vasculitis with cryoglobulin deposition. Laboratory tests revealed a type II cryoglobulinemia with monoclonal IgG Kappa and polyclonal IgG with negative rheumatoid factor, C3 and C4. A work-up excluded a hematologic or connective tissue disease and confirmed an immune profile due to a past B hepatitis. A diagnosis of MC was made. The patient was treated with two rituximab cycles (1-g intravenous
Letters to the Editor / Joint Bone Spine 82 (2015) 129–137
infusion on day 1 and 15), methotrexate (10 mg/week) and low-dose aspirin for a worsening in lower limb ulcers. These lesions considerably improved, but new purpuric-livedoid plaques appeared in upper limbs and face requiring multiple courses of high-dose intravenous steroids. In 2011, the patient presented livedoid helix lesions that progressively evolved to ulcerative-necrotic lesions (Fig. S2; see the supplementary material associated with this article online). Thalidomide (50 mg/day) was started with sudden disappearance of the pain. A consecutive increase to 100 mg/day induced a complete regression of all the lesions. During these last 3 years the patient experienced recurrent relapses of skin ulcers (both in auricles and lower limbs) that were readily treated with brief thalidomide cycles. Purpura and ulcers are the typical skin manifestations of MC, whereas skin necrosis is more common in type I cryoglobulinemia [2,3]. Auricle involvement is unusual in cryoglobulinemia, but few reports have been published both in type I and III disease [4–6]. Our patient is to our knowledge the first case of MC with skin involvement responsive to thalidomide. He is also unusual for the presence of monoclonal IgG-polyclonal IgG immune-complexes without rheumatoid factor activity, not activating complement and severe skin lesions in atypical sites. Based on the failure of recommended medications for severe disease (glucocorticoids, rituximab) [3], the presence of skin manifestations that were closer to a type I than a type II cryoglobulinemia and the previously reported efficacy of thalidomide in type I cryoglobulinemia [3,7–9] we chose to treat our patient with thalidomide. A low dosage of this medication was extremely fast in healing all types of lesions. The efficacy was confirmed by the relapse of lesions at its interruption and by the disappearance at its reintroduction. Our case report suggests that thalidomide might be an option to be considered for the treatment of severe and resistant cryoglobulinemic cutaneous vasculitis.
131
Mara Taraborelli a,b,∗ Paola Monari c Ilaria Cavazzana a Giulio Gualdi c Piergiacomo Calzavara-Pinton b,c Franco Franceschini a a Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, piazzale Spedali Civili di Brescia 1, 25123 Brescia, Italy b University of Brescia, piazza Mercato 15, 25121 Brescia, Italy c Dermatology Department, Spedali Civili and University of Brescia, piazzale Spedali Civili di Brescia 1, 25123 Brescia, Italy ∗ Corresponding author at: Reumatologia e Immunologia Clinica, Spedali Civili, piazzale Spedali Civili 1, 25123 Brescia, Italy. E-mail address: [email protected] (M. Taraborelli)
Accepted 18 May 2014 Available online 27 June 2014 http://dx.doi.org/10.1016/j.jbspin.2014.05.005
Chronic expanding hematoma of the calf
a r t i c l e
i n f o
Keywords: Chronic expanding hematoma Baker’s cyst
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
Appendix A. Supplementary material Supplementary materials (Figs. S1–S2) associated with this article can be found at http://www.sciencedirect.com, in the online version, at http://dx.doi.org/10.1016/j.jbspin.2014.05.005.
References [1] Lospalluto J, Dorward B, Miller Jr W, et al. Cryoglobulinemia based on interaction between a gamma macroglobulin and 7S gamma globulin. Am J Med 1962;32:142–5. [2] Ramos-Casals M, Stone JH, Cid MC, et al. The cryoglobulinaemias. Lancet 2012;379:348–60. [3] Terrier B, Karras A, Kahn JE, et al. The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases. Medicine (Baltimore) 2013;92:61–8. [4] Vila AT, Barnadas MA, Ballarin J, et al. Cutaneous ulcers with type I cryoglobulinemia treated with plasmapheresis. Eur J Dermatol 2004;14:186–9. [5] Martens HA, Bijl M. Clinical images: blue-purple ears. Arthritis Rheum 2007;56:673. [6] Nan DN, Fernández-Ayala M, García-Palomo D, et al. Atypical skin lesions associated with mixed cryoglobulinaemia and hepatitis C virus infection in a cocaine-consuming patient. Br J Dermatol 2000;143:1330–1. [7] Calabrese C, Faiman B, Martin D, et al. Type 1 cryoglobulinemia: response to thalidomide and lenalidomide. J Clin Rheumatol 2011;17:145–7. [8] Sampson A, Callen JP. The cutting edge: thalidomide for type 1 cryoglobulinemic vasculopathy. Arch Dermatol 2006;142:972–4. [9] Cem Ar M, Soysal T, Hatemi G, et al. Successful management of cryoglobulinemiainduced leukocytoclastic vasculitis with thalidomide in a patient with multiple myeloma. Ann Hematol 2005;84:609–13.
Chronic expanding hematoma (CEH) is the manifestation of a hematoma that is present and continues to enlarge for more than one month after the initial hemorrhage [1]. It was suggested that CEH might result from irritant effect of blood and its breakdown products, leading to repeated exudation or bleeding from the capillaries in the granulation tissue [1]. There have been only a few reports of CEH involving the lower limb, one of them recently published in the Joint Bone Spine journal [2]. On the other side, a hemorrhagic rupture of a Baker’s cyst occasionally may occur and is usually diffused in the surrounding tissues [3,4]. In this letter to the editor, we would like to share our experience with a very interesting case of a CEH caused most likely by a hemorrhagic rupture of a Baker’s cyst. A 65-year-old man presented with a 3-month history of slow growing right calf mass after hyperextension injury with subsequent development of ecchymosis, mild edema and discomfort of the affected area. Physical exam revealed palpable mass over the posteromedial side of the lower leg with normal overlaying skin. Ultrasound (US) and magnetic resonance imaging (MRI) were subsequently performed showing a 5 × 11 × 3.5 cm cystic lesion. (Fig. 1A–E) Review of the MRI report from three years earlier revealed degenerative knee changes and moderate size Baker’s cyst. Intraoperatively, the mass was located between the subcutaneous tissue and the underlying muscle and had a thick capsule that was not well-defined form the adjacent tissues and adherent to the superficial fascia and underlying muscle (Fig. 1F). The content of the cyst consisted of yellowish fluid and white proteinaceous-looking material mostly adherent to the inner capsular wall. Granulation tissue in conjunction with metaplastic cartilaginous tissue, abundant acellular fibrin and evidence of pseudocapsule consistent with chronic organizing hematoma were
Letters to the Editor / Joint Bone Spine 82 (2015) 129–137 [4] Hamaoka T, Madewell JE, Podoloff DA, et al. Bone imaging in metastatic breast cancer. J Clin Oncol 2004;22:2942–53. [5] Whitlock JP, Evans AJ, Jackson L, et al. Imaging of metastatic breast cancer: distribution and radiological assessment at presentation. Clin Oncol (R Coll Radiol) 2001;13:181–6. [6] Umeoka S, Koyama T, Miki Y, et al. Pictorial review of tuberous sclerosis in various organs. Radiographics 2008;28:e32. [7] Baskin Jr HJ. The pathogenesis and imaging of the tuberous sclerosis complex. Pediatr Radiol 2008;38:936–52. [8] Song L, Zhang Y, Zhang W. Bone scintigraphy may help differentiate bone sclerotic lesions from osteoblastic metastases in tuberous sclerosis patients with concomitant pulmonary adenocarcinoma. Clin Imaging 2013;37:382–5.
Constance de Margerie-Mellon a,∗ Rachid Kaci b Jean-Denis Laredo a Valérie Bousson a a Department of Osteoarticular Radiology, Université Paris Diderot, hôpital Lariboisière, 2, rue Ambroise-Paré, Sorbonne Paris Cité, 75475 Paris cedex 10, France b Department of Pathology, université Paris Diderot, hôpital Lariboisière, AP–HP, 2, rue Ambroise-Paré, Sorbonne Paris Cité, 75475 Paris cedex 10, France ∗ Corresponding
author. Department of radiology, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75475 Paris cedex 10, France. E-mail address: [email protected] (C. de Margerie-Mellon) Accepted 5 August 2014 Available online 17 September 2014
http://dx.doi.org/10.1016/j.jbspin.2014.08.002
Severe skin involvement in type II cryoglobulinemia successfully treated with thalidomide
a r t i c l e Fig. 2. Thoraco-abdomino-pelvic CT scan, sagittal reformation. Diffuse small blastic lesions of vertebral bodies and pelvic bones. Lytic lesion of T12 vertebral body (arrow).
In conclusion, since breast cancer is relatively frequent, atypical presentation should be recognized. Most of the time, bone metastases are heterogeneous with a lytic pattern. However, bone lesions can be small, blastic and homogeneous, and the breast tumor hidden. Rare diagnosis as TS can only be made after definitive elimination of frequent diseases and verification of complete compliance with the diagnostic criteria. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13: 624–8. [2] Orvieto E, Maiorano E, Bottiglieri L, et al. Clinicopathologic characteristics of invasive lobular carcinoma of the breast: results of an analysis of 530 cases from a single institution. Cancer 2008;113:1511–20. [3] Sastre-Garau X, Jouve M, Asselain B, et al. Infiltrating lobular carcinoma of the breast: clinicopathologic analysis of 975 cases with reference to data on conservative therapy and metastatic patterns. Cancer 1998;77:113–20.
i n f o
Keywords: Mixed cryoglobulinemia Ulcers Auricles Thalidomide
Mixed cryoglobulinemia (MC) is a small vessel vasculitis associated with serum immune-complexes containing immunoglobulins that precipitate at temperatures below 37 ◦ C [1]. We report the case of a patient with severe skin involvement successfully treated with thalidomide after the failure of conventional therapy. A 68-years-old man came to our attention on 2009 with a 3 year-history of lower limb purpuric-livedoid plaques and ulcers (Fig. S1; see the supplementary material associated with this article online). A diagnosis of erythema elevatum diutinum had been made based on a histological finding of leukocytoclastic vasculitis with microthrombosis. He had been treated with dapsone (50 mg/day) and low-dose methylprednisolone. Some periodical relapses had been well controlled with an increase of steroids, until an extensive ulceration of the lower limbs and abdomen arised. A biopsy confirmed a leukocytoclastic vasculitis with cryoglobulin deposition. Laboratory tests revealed a type II cryoglobulinemia with monoclonal IgG Kappa and polyclonal IgG with negative rheumatoid factor, C3 and C4. A work-up excluded a hematologic or connective tissue disease and confirmed an immune profile due to a past B hepatitis. A diagnosis of MC was made. The patient was treated with two rituximab cycles (1-g intravenous
Letters to the Editor / Joint Bone Spine 82 (2015) 129–137
infusion on day 1 and 15), methotrexate (10 mg/week) and low-dose aspirin for a worsening in lower limb ulcers. These lesions considerably improved, but new purpuric-livedoid plaques appeared in upper limbs and face requiring multiple courses of high-dose intravenous steroids. In 2011, the patient presented livedoid helix lesions that progressively evolved to ulcerative-necrotic lesions (Fig. S2; see the supplementary material associated with this article online). Thalidomide (50 mg/day) was started with sudden disappearance of the pain. A consecutive increase to 100 mg/day induced a complete regression of all the lesions. During these last 3 years the patient experienced recurrent relapses of skin ulcers (both in auricles and lower limbs) that were readily treated with brief thalidomide cycles. Purpura and ulcers are the typical skin manifestations of MC, whereas skin necrosis is more common in type I cryoglobulinemia [2,3]. Auricle involvement is unusual in cryoglobulinemia, but few reports have been published both in type I and III disease [4–6]. Our patient is to our knowledge the first case of MC with skin involvement responsive to thalidomide. He is also unusual for the presence of monoclonal IgG-polyclonal IgG immune-complexes without rheumatoid factor activity, not activating complement and severe skin lesions in atypical sites. Based on the failure of recommended medications for severe disease (glucocorticoids, rituximab) [3], the presence of skin manifestations that were closer to a type I than a type II cryoglobulinemia and the previously reported efficacy of thalidomide in type I cryoglobulinemia [3,7–9] we chose to treat our patient with thalidomide. A low dosage of this medication was extremely fast in healing all types of lesions. The efficacy was confirmed by the relapse of lesions at its interruption and by the disappearance at its reintroduction. Our case report suggests that thalidomide might be an option to be considered for the treatment of severe and resistant cryoglobulinemic cutaneous vasculitis.
131
Mara Taraborelli a,b,∗ Paola Monari c Ilaria Cavazzana a Giulio Gualdi c Piergiacomo Calzavara-Pinton b,c Franco Franceschini a a Rheumatology and Clinical Immunology Department, Spedali Civili of Brescia, piazzale Spedali Civili di Brescia 1, 25123 Brescia, Italy b University of Brescia, piazza Mercato 15, 25121 Brescia, Italy c Dermatology Department, Spedali Civili and University of Brescia, piazzale Spedali Civili di Brescia 1, 25123 Brescia, Italy ∗ Corresponding author at: Reumatologia e Immunologia Clinica, Spedali Civili, piazzale Spedali Civili 1, 25123 Brescia, Italy. E-mail address: [email protected] (M. Taraborelli)
Accepted 18 May 2014 Available online 27 June 2014 http://dx.doi.org/10.1016/j.jbspin.2014.05.005
Chronic expanding hematoma of the calf
a r t i c l e
i n f o
Keywords: Chronic expanding hematoma Baker’s cyst
Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
Appendix A. Supplementary material Supplementary materials (Figs. S1–S2) associated with this article can be found at http://www.sciencedirect.com, in the online version, at http://dx.doi.org/10.1016/j.jbspin.2014.05.005.
References [1] Lospalluto J, Dorward B, Miller Jr W, et al. Cryoglobulinemia based on interaction between a gamma macroglobulin and 7S gamma globulin. Am J Med 1962;32:142–5. [2] Ramos-Casals M, Stone JH, Cid MC, et al. The cryoglobulinaemias. Lancet 2012;379:348–60. [3] Terrier B, Karras A, Kahn JE, et al. The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases. Medicine (Baltimore) 2013;92:61–8. [4] Vila AT, Barnadas MA, Ballarin J, et al. Cutaneous ulcers with type I cryoglobulinemia treated with plasmapheresis. Eur J Dermatol 2004;14:186–9. [5] Martens HA, Bijl M. Clinical images: blue-purple ears. Arthritis Rheum 2007;56:673. [6] Nan DN, Fernández-Ayala M, García-Palomo D, et al. Atypical skin lesions associated with mixed cryoglobulinaemia and hepatitis C virus infection in a cocaine-consuming patient. Br J Dermatol 2000;143:1330–1. [7] Calabrese C, Faiman B, Martin D, et al. Type 1 cryoglobulinemia: response to thalidomide and lenalidomide. J Clin Rheumatol 2011;17:145–7. [8] Sampson A, Callen JP. The cutting edge: thalidomide for type 1 cryoglobulinemic vasculopathy. Arch Dermatol 2006;142:972–4. [9] Cem Ar M, Soysal T, Hatemi G, et al. Successful management of cryoglobulinemiainduced leukocytoclastic vasculitis with thalidomide in a patient with multiple myeloma. Ann Hematol 2005;84:609–13.
Chronic expanding hematoma (CEH) is the manifestation of a hematoma that is present and continues to enlarge for more than one month after the initial hemorrhage [1]. It was suggested that CEH might result from irritant effect of blood and its breakdown products, leading to repeated exudation or bleeding from the capillaries in the granulation tissue [1]. There have been only a few reports of CEH involving the lower limb, one of them recently published in the Joint Bone Spine journal [2]. On the other side, a hemorrhagic rupture of a Baker’s cyst occasionally may occur and is usually diffused in the surrounding tissues [3,4]. In this letter to the editor, we would like to share our experience with a very interesting case of a CEH caused most likely by a hemorrhagic rupture of a Baker’s cyst. A 65-year-old man presented with a 3-month history of slow growing right calf mass after hyperextension injury with subsequent development of ecchymosis, mild edema and discomfort of the affected area. Physical exam revealed palpable mass over the posteromedial side of the lower leg with normal overlaying skin. Ultrasound (US) and magnetic resonance imaging (MRI) were subsequently performed showing a 5 × 11 × 3.5 cm cystic lesion. (Fig. 1A–E) Review of the MRI report from three years earlier revealed degenerative knee changes and moderate size Baker’s cyst. Intraoperatively, the mass was located between the subcutaneous tissue and the underlying muscle and had a thick capsule that was not well-defined form the adjacent tissues and adherent to the superficial fascia and underlying muscle (Fig. 1F). The content of the cyst consisted of yellowish fluid and white proteinaceous-looking material mostly adherent to the inner capsular wall. Granulation tissue in conjunction with metaplastic cartilaginous tissue, abundant acellular fibrin and evidence of pseudocapsule consistent with chronic organizing hematoma were
