BRIEF REPORT
Postirradiation Pseudosclerodermatous Panniculitis of the Leg: Report of a Case and Review of the Literature Mauricio Sandoval, MD,* Laura Giesen, MD,* Karina Cataldo, MD,* and Sergio Gonzalez, MD†
Abstract: Postirradiation pseudosclerodermatous panniculitis presents as an erythematous indurated plaque, on a previously irradiated area. Histopathologically, thick and sclerotic septa and inflammatory infiltrate with foamy histiocytes and adiponecrosis are seen. To date, 12 cases have been reported worldwide. Hereby, we report the case of a 68-yearold woman who developed an indurated painful erythematous plaque on her right leg after radiotherapy for a soft tissue sarcoma, with a septal panniculitis and lobular adiponecrosis on histopathological examination. This is the 13th case reported and the second of extrathoracic location. Key Words: panniculitis, radiotherapy, diagnosis, differential (Am J Dermatopathol 2015;37:587–589)
INTRODUCTION
In 1993, Winkelmann et al1 described 4 cases of patients who received radiotherapy due to breast cancer and developed erythematous and edematous indurated plaques on the irradiated skin, which appeared 1 to 6 months after treatment. A histiocytic infiltrate in the dermis and subcutaneous fat was observed. These authors named this entity pseudosclerodermatous panniculitis after irradiation. Eight cases have been reported since then,2–4 all of them on the thorax in breast cancer patients, except for 1 case on the left inguinal area due to radiotherapy for breast cancer metastasis on the femur’s head. Hereby, we report an additional case of this condition after radiotherapy for a soft tissue sarcoma.
CASE REPORT A 68-year-old woman, with a right patellar myxoid liposarcoma, treated with neoadjuvant radiotherapy on December 2008, surgical excision on March 2009, and chemotherapy and a new surgery due to iliac and sacral metastasis on 2011. Five years after radiotherapy, she complained of an indurated painful erythematous plaque on her right leg of 4 months of evolution. She was first seen by her orthopedic surgeon who diagnosed cellulitis and prescribed antibiotics, without results. On physical examination, a large dully erythematous and warm indurated plaque was noted (Fig. 1).
From the *Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago de Chile, Chile; and †Department of Pathology, Pontificia Universidad Católica de Chile, Santiago de Chile, Chile. The authors declare no conflicts of interest. Reprints: Mauricio Sandoval, MD, Department of Dermatology, Centro Médico San Joaquín, Avenida Vicuña Mackenna 4686, Macul, Santiago de Chile, Chile 7820436 (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Am J Dermatopathol Volume 37, Number 7, July 2015
Laboratory findings were otherwise within normal limits. The skin biopsy showed a mostly lobular panniculitis with thickened sclerotic septa. Lobular adiponecrosis was present. Lipophagic granulomas and foamy histiocytes were not seen. No epidermal, blood vessels, or connective tissue changes were noted (Figs. 2, 3).
DISCUSSION Postirradiation pseudosclerodermatous panniculitis appears as an erythematous indurated plaque, on a previously radiated area. It can appear months1,2 or even years3,4 after radiotherapy. This case presented several years after radiotherapy. To date, 12 cases have been reported, all of them in female patients with breast cancer and after radiotherapy (Table 1). We also report a case in a woman, with a sarcoma on a different anatomic site, being the first postirradiation pseudosclerodermatous panniculitis case report not associated with breast cancer and the second extrathoracic case reported in the literature. It is interesting that only women are affected, and that most of them were previously treated for breast cancer, suggesting a hormonal cause. The wide variation in latency between reported cases is not clear at this time; there might be more clinical variables involved in the length between radiotherapy and postirradiation pseudosclerodermatous panniculitis onset, other than sex, age, and associated neoplasia, which have not been addressed. Postirradiation pseudosclerodermatous panniculitis is a panniculitis without vasculitis, of histiocytic predominance.5 Subcutaneous septa are thick and sclerotic, but the main findings can be seen in fat lobules: an inflammatory infiltrate with foamy histiocytes and few lymphocytes and plasma cells, necrotic adypocytes, and lipophagic granulomas at the periphery of the lobules,2–4 ie, adiponecrosis. Winkelmann et al,1 Dalle et al,3 and Pielasinski et al4 noted irradiaton-induced changes in the dermis, such as dilated hyalinized blood vessels, endothelial cells hyperplasia, fibrosis with atypical fibroblasts, and skin adnexa involution. However, Carrasco et al2 did not observe radiodermatitis. Currently, radiotherapy techniques have improved and diminished chronic radiodermatitis incidence,2 but the deep tissue damage has increased.4 The most important clinical differential diagnosis is breast cancer cutaneous metastasis, so if there is diagnostic doubt, skin biopsy is needed. Other possible diagnoses are cellulitis and connective tissue diseases. Histopathologically, the main differential diagnosis considers morphea profunda and lupus panniculitis. Postirradiation morphea has been described, but this condition www.amjdermatopathology.com |
587
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Am J Dermatopathol Volume 37, Number 7, July 2015
Sandoval et al
TABLE 1. Clinicopathological Findings of Each Postirradiation Pseudosclerodermatous Panniculitis Case Report Ref
Case
Sex
Age
Neoplasia
Latency
1
1
F
58
Breast
1–6 mo
2
2 3 4 5
F F F F
69 73 73 56
Breast Breast Breast Breast
8 mo
6
F
67
Breast
6 mo
7
F
58
Breast
6 mo
8
F
44
Breast bone metastases
4 mo
9
F
60
Breast
4 mo
3
4
This case
Clinical Findings
Lymphocytic inflammation within fat lobules Lipophagic granulomas Collagenization of fat lobules
Subcutaneous induration of the anterior chest Subcutaneous induration of the anterior chest
Few dilated lymphatic vessels throughout the dermis Mostly lobular panniculitis, with adiponecrosis and dense inflammatory infiltrates of foamy histiocytes Lipophagic granulomas
Subcutaneous induration of the anterior chest Subcutaneous induration of the left inguinal area Painful erythema and progressive infiltration of the right breast
10
F
64
Breast
17 yrs
Erythematous indurate plaque
11
F
41
Breast
3 yrs
Subcutaneous asymptomatic nodule
12
F
71
Breast
6 yrs
Indurate plaque with ulcerated surface
F
68
Myxoid liposarcoma
5 yrs
Histopathological Findings
Erythema, edema and induration of the breast tissue
Indurated painful erythematous plaque
Thickened septa of the subcutaneous tissue Atrophic epidermis Dilated and hyalinized blood vessels, endothelial cell hyperplasia, dermal fibrosis, involution of appendages, and dermal lymphocytic inflammatory infiltrate Lobular panniculitis and thickened, sclerotic septa Lymphocytic inflammation within fat lobules, with adiponecrosis, lipophagic granulomas, and foamy histiocytes Dermal radiodermatitis Lipophagic granulomas Sclerotic septa of the subcutis Dermal radiodermatitis Lymphocytic infiltrate involving the entire dermis Lipophagic granulomas Sclerotic septa of the subcutis Dermal radiodermatitis Dermal sclerosis extending to subcutaneous tissue Hyalinization of the vessel walls in venules and arterioles Lipophagic granulomas Mostly lobular panniculitis with thickened, sclerotic septa Lobular adiponecrosis
mostly affects the dermis.3 Morphea profunda is mostly a septal panniculitis, with an inflammatory infiltrate of lymphocytes and plasma cells6; septal sclerosis and hyalinization may be observed, with replacement of the subcutaneous fat.6 Lupus panniculitis is a lobular panniculitis, without vasculitis, with lymphocytic inflammatory infiltrates, lymphoid follicles in the subcutaneous septa, and lymphocytic nuclear dust; adipocytes necrosis or lipophagic granulomas are not seen.5 In half of these cases, there are epidermal and dermal changes of discoid lupus erythematosus, and most of them
FIGURE 1. Large dully erythematous and warm indurated plaque on the right leg.
588
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
| www.amjdermatopathology.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Am J Dermatopathol Volume 37, Number 7, July 2015
FIGURE 2. Microphotograph showing septal fibrosis and lobular atrophy. Hematoxylin-eosine, ·100.
exhibit linear deposition of IgM and c3 along the dermoepidermal junction.5 Spontaneous resolution,2 persistence without improve4 ment, and response to systemic corticosteroids3 have been reported in postirradiation pseudosclerodermatous panniculitis; more information about therapeutic options are scarce. REFERENCES 1. Winkelmann RK, Grado GL, Quimby SR, et al. Pseudosclerodermatous panniculitis after irradiation: an unusual complication of megavoltage treatment of breast carcinoma. Mayo Clin Proc. 1993;68:122–127. 2. Carrasco L, Moreno C, Pastor MA, et al. Postirradiation pseudosclerodermatous panniculitis. Am J Dermatopathol. 2001;23:283–287.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Postirradiation Pseudosclerodermatous Panniculitis
FIGURE 3. Microphotograph showing dense septal fibrosis with telangiectasis without inflammatory infiltrates and atrophy of lobular adipocytes. Hematoxylin-eosine, ·200. 3. Dalle S, Skowron F, Ronger-Savie S, et al. Pseudosclerodermatous panniculitis after irradiation and bronchiolitis obliterans organizing pneumonia: simultaneous onset suggesting a common origin. Dermatology. 2004; 209:138–141. 4. Pielasinski U, Machan S, Camacho D, et al. Postirradiation pseudosclerodermatous panniculitis: three new cases with additional histopathologic features supporting the radiotherapy etiology. Am J Dermatopathol. 2013; 35:129–134. 5. Requena L, Sánchez Yus E. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol. 2001;45:325–361. 6. Requena L, Sánchez Yus E. Panniculitis. Part I. Mostly septal panniculitis. J Am Acad Dermatol. 2001;45:163–183.
www.amjdermatopathology.com |
589
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Postirradiation Pseudosclerodermatous Panniculitis of the Leg: Report of a Case and Review of the Literature Mauricio Sandoval, MD,* Laura Giesen, MD,* Karina Cataldo, MD,* and Sergio Gonzalez, MD†
Abstract: Postirradiation pseudosclerodermatous panniculitis presents as an erythematous indurated plaque, on a previously irradiated area. Histopathologically, thick and sclerotic septa and inflammatory infiltrate with foamy histiocytes and adiponecrosis are seen. To date, 12 cases have been reported worldwide. Hereby, we report the case of a 68-yearold woman who developed an indurated painful erythematous plaque on her right leg after radiotherapy for a soft tissue sarcoma, with a septal panniculitis and lobular adiponecrosis on histopathological examination. This is the 13th case reported and the second of extrathoracic location. Key Words: panniculitis, radiotherapy, diagnosis, differential (Am J Dermatopathol 2015;37:587–589)
INTRODUCTION
In 1993, Winkelmann et al1 described 4 cases of patients who received radiotherapy due to breast cancer and developed erythematous and edematous indurated plaques on the irradiated skin, which appeared 1 to 6 months after treatment. A histiocytic infiltrate in the dermis and subcutaneous fat was observed. These authors named this entity pseudosclerodermatous panniculitis after irradiation. Eight cases have been reported since then,2–4 all of them on the thorax in breast cancer patients, except for 1 case on the left inguinal area due to radiotherapy for breast cancer metastasis on the femur’s head. Hereby, we report an additional case of this condition after radiotherapy for a soft tissue sarcoma.
CASE REPORT A 68-year-old woman, with a right patellar myxoid liposarcoma, treated with neoadjuvant radiotherapy on December 2008, surgical excision on March 2009, and chemotherapy and a new surgery due to iliac and sacral metastasis on 2011. Five years after radiotherapy, she complained of an indurated painful erythematous plaque on her right leg of 4 months of evolution. She was first seen by her orthopedic surgeon who diagnosed cellulitis and prescribed antibiotics, without results. On physical examination, a large dully erythematous and warm indurated plaque was noted (Fig. 1).
From the *Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago de Chile, Chile; and †Department of Pathology, Pontificia Universidad Católica de Chile, Santiago de Chile, Chile. The authors declare no conflicts of interest. Reprints: Mauricio Sandoval, MD, Department of Dermatology, Centro Médico San Joaquín, Avenida Vicuña Mackenna 4686, Macul, Santiago de Chile, Chile 7820436 (e-mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Am J Dermatopathol Volume 37, Number 7, July 2015
Laboratory findings were otherwise within normal limits. The skin biopsy showed a mostly lobular panniculitis with thickened sclerotic septa. Lobular adiponecrosis was present. Lipophagic granulomas and foamy histiocytes were not seen. No epidermal, blood vessels, or connective tissue changes were noted (Figs. 2, 3).
DISCUSSION Postirradiation pseudosclerodermatous panniculitis appears as an erythematous indurated plaque, on a previously radiated area. It can appear months1,2 or even years3,4 after radiotherapy. This case presented several years after radiotherapy. To date, 12 cases have been reported, all of them in female patients with breast cancer and after radiotherapy (Table 1). We also report a case in a woman, with a sarcoma on a different anatomic site, being the first postirradiation pseudosclerodermatous panniculitis case report not associated with breast cancer and the second extrathoracic case reported in the literature. It is interesting that only women are affected, and that most of them were previously treated for breast cancer, suggesting a hormonal cause. The wide variation in latency between reported cases is not clear at this time; there might be more clinical variables involved in the length between radiotherapy and postirradiation pseudosclerodermatous panniculitis onset, other than sex, age, and associated neoplasia, which have not been addressed. Postirradiation pseudosclerodermatous panniculitis is a panniculitis without vasculitis, of histiocytic predominance.5 Subcutaneous septa are thick and sclerotic, but the main findings can be seen in fat lobules: an inflammatory infiltrate with foamy histiocytes and few lymphocytes and plasma cells, necrotic adypocytes, and lipophagic granulomas at the periphery of the lobules,2–4 ie, adiponecrosis. Winkelmann et al,1 Dalle et al,3 and Pielasinski et al4 noted irradiaton-induced changes in the dermis, such as dilated hyalinized blood vessels, endothelial cells hyperplasia, fibrosis with atypical fibroblasts, and skin adnexa involution. However, Carrasco et al2 did not observe radiodermatitis. Currently, radiotherapy techniques have improved and diminished chronic radiodermatitis incidence,2 but the deep tissue damage has increased.4 The most important clinical differential diagnosis is breast cancer cutaneous metastasis, so if there is diagnostic doubt, skin biopsy is needed. Other possible diagnoses are cellulitis and connective tissue diseases. Histopathologically, the main differential diagnosis considers morphea profunda and lupus panniculitis. Postirradiation morphea has been described, but this condition www.amjdermatopathology.com |
587
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Am J Dermatopathol Volume 37, Number 7, July 2015
Sandoval et al
TABLE 1. Clinicopathological Findings of Each Postirradiation Pseudosclerodermatous Panniculitis Case Report Ref
Case
Sex
Age
Neoplasia
Latency
1
1
F
58
Breast
1–6 mo
2
2 3 4 5
F F F F
69 73 73 56
Breast Breast Breast Breast
8 mo
6
F
67
Breast
6 mo
7
F
58
Breast
6 mo
8
F
44
Breast bone metastases
4 mo
9
F
60
Breast
4 mo
3
4
This case
Clinical Findings
Lymphocytic inflammation within fat lobules Lipophagic granulomas Collagenization of fat lobules
Subcutaneous induration of the anterior chest Subcutaneous induration of the anterior chest
Few dilated lymphatic vessels throughout the dermis Mostly lobular panniculitis, with adiponecrosis and dense inflammatory infiltrates of foamy histiocytes Lipophagic granulomas
Subcutaneous induration of the anterior chest Subcutaneous induration of the left inguinal area Painful erythema and progressive infiltration of the right breast
10
F
64
Breast
17 yrs
Erythematous indurate plaque
11
F
41
Breast
3 yrs
Subcutaneous asymptomatic nodule
12
F
71
Breast
6 yrs
Indurate plaque with ulcerated surface
F
68
Myxoid liposarcoma
5 yrs
Histopathological Findings
Erythema, edema and induration of the breast tissue
Indurated painful erythematous plaque
Thickened septa of the subcutaneous tissue Atrophic epidermis Dilated and hyalinized blood vessels, endothelial cell hyperplasia, dermal fibrosis, involution of appendages, and dermal lymphocytic inflammatory infiltrate Lobular panniculitis and thickened, sclerotic septa Lymphocytic inflammation within fat lobules, with adiponecrosis, lipophagic granulomas, and foamy histiocytes Dermal radiodermatitis Lipophagic granulomas Sclerotic septa of the subcutis Dermal radiodermatitis Lymphocytic infiltrate involving the entire dermis Lipophagic granulomas Sclerotic septa of the subcutis Dermal radiodermatitis Dermal sclerosis extending to subcutaneous tissue Hyalinization of the vessel walls in venules and arterioles Lipophagic granulomas Mostly lobular panniculitis with thickened, sclerotic septa Lobular adiponecrosis
mostly affects the dermis.3 Morphea profunda is mostly a septal panniculitis, with an inflammatory infiltrate of lymphocytes and plasma cells6; septal sclerosis and hyalinization may be observed, with replacement of the subcutaneous fat.6 Lupus panniculitis is a lobular panniculitis, without vasculitis, with lymphocytic inflammatory infiltrates, lymphoid follicles in the subcutaneous septa, and lymphocytic nuclear dust; adipocytes necrosis or lipophagic granulomas are not seen.5 In half of these cases, there are epidermal and dermal changes of discoid lupus erythematosus, and most of them
FIGURE 1. Large dully erythematous and warm indurated plaque on the right leg.
588
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
| www.amjdermatopathology.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Am J Dermatopathol Volume 37, Number 7, July 2015
FIGURE 2. Microphotograph showing septal fibrosis and lobular atrophy. Hematoxylin-eosine, ·100.
exhibit linear deposition of IgM and c3 along the dermoepidermal junction.5 Spontaneous resolution,2 persistence without improve4 ment, and response to systemic corticosteroids3 have been reported in postirradiation pseudosclerodermatous panniculitis; more information about therapeutic options are scarce. REFERENCES 1. Winkelmann RK, Grado GL, Quimby SR, et al. Pseudosclerodermatous panniculitis after irradiation: an unusual complication of megavoltage treatment of breast carcinoma. Mayo Clin Proc. 1993;68:122–127. 2. Carrasco L, Moreno C, Pastor MA, et al. Postirradiation pseudosclerodermatous panniculitis. Am J Dermatopathol. 2001;23:283–287.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Postirradiation Pseudosclerodermatous Panniculitis
FIGURE 3. Microphotograph showing dense septal fibrosis with telangiectasis without inflammatory infiltrates and atrophy of lobular adipocytes. Hematoxylin-eosine, ·200. 3. Dalle S, Skowron F, Ronger-Savie S, et al. Pseudosclerodermatous panniculitis after irradiation and bronchiolitis obliterans organizing pneumonia: simultaneous onset suggesting a common origin. Dermatology. 2004; 209:138–141. 4. Pielasinski U, Machan S, Camacho D, et al. Postirradiation pseudosclerodermatous panniculitis: three new cases with additional histopathologic features supporting the radiotherapy etiology. Am J Dermatopathol. 2013; 35:129–134. 5. Requena L, Sánchez Yus E. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol. 2001;45:325–361. 6. Requena L, Sánchez Yus E. Panniculitis. Part I. Mostly septal panniculitis. J Am Acad Dermatol. 2001;45:163–183.
www.amjdermatopathology.com |
589
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
![[Phleboliths revealing multiple malformations of the leg: report of a case and review of the literature].](/assets/img/hold.png)
