June 2012

Panniculitis Associated With Dermatomyositis and Recurrent Ovarian Cancer

Author Affiliations

Author Affiliations: Harvard Medical School, Boston, Massachusetts (Ms Girouard and Drs Velez, Penson, Massarotti and Vleugels); Department of Dermatology (Drs Velez and Vleugels) and Department of Medicine, Division of Rheumatology, Immunology, and Allergy (Dr Massarotti), Brigham and Women's Hospital, Boston; Department of Medicine, Division of Medical Gynecologic Oncology (Dr Penson), Massachusetts General Hospital, Boston; and Division of Allergy and Immunology, Children's Hospital Boston (Dr Vleugels).

Arch Dermatol. 2012;148(6):740-744. doi:10.1001/archdermatol.2012.288

Background Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM), typically presenting as tender, erythematous subcutaneous nodules. Complications, such as pain, calcinosis, and lipoatrophy, are associated with high morbidity. While it has been suggested that panniculitis in DM may correlate with a better prognosis, our understanding of its true significance, prognostic implications, and management is limited by the paucity of reported cases. We describe the first reported case to our knowledge of DM-associated panniculitis in the setting of ovarian adenocarcinoma as well as in association with a recurrent malignancy.

Observations A 63-year-old woman with a history of DM and recurrent ovarian adenocarcinoma presented with multiple painful, erythematous subcutaneous nodules on the bilateral upper arms, thighs, and buttocks. Histologic examination showed lymphoplasmacytic panniculitis with associated dermal mucin deposition, consistent with lobular panniculitis in association with connective-tissue disease. Treatment with oral methotrexate resulted in sustained clinical improvement over a 10-month period.

Conclusions Although panniculitis in DM has previously been suggested to be a good prognostic indicator, our case report describes an association with ovarian adenocarcinoma and a recurrent malignancy. Methotrexate may be an effective treatment for panniculitis in DM.

Dermatomyositis (DM) is a connective tissue disease characterized by inflammatory myopathy and distinct cutaneous findings, including Gottron's papules and a periorbital heliotrope eruption. Panniculitis, a rare cutaneous manifestation of DM, is associated with tender, erythematous nodules. A limited number of case reports describe an association between DM and panniculitis, the significance of which is unknown. Early diagnosis and treatment of panniculitis is critical because its complications, which include pain, lipoatrophy, and calcinosis, can be severe and irreversible. However, no standard treatment regimen exists given the paucity of available therapeutic data for this entity. Herein, we report a case of panniculitis in a patient with DM and recurrent ovarian adenocarcinoma that responded to treatment with methotrexate.


A 63-year-old woman with a history of recurrent ovarian adenocarcinoma and DM presented with multiple painful, erythematous subcutaneous nodules on her bilateral arms, thighs, and buttocks. Twenty-five months earlier she had been diagnosed as having DM, both clinically and with confirmation from skin and muscle biopsies, after presenting with symmetric proximal muscle weakness, elevated muscle enzyme levels, periorbital violaceous erythema, and lichenoid violaceous papules overlying the knuckles on the dorsal hands. Immunologic studies showed a positive antinuclear antibody (ANA) titer (1:160, speckled pattern), and negative findings for anti-RNP, anti-SS-A, anti-SS-B, and anti-Jo-1 antibodies. She was treated with systemic corticosteroids, methotrexate, and intravenous immunoglobulin (IVIG), all of which were subsequently tapered and then discontinued after remission of her disease was achieved.

Almost 18 years earlier, she had been diagnosed with stage IIIB papillary serous cystadenocarcinoma of the right ovary and treated with surgical resection, pelvic node dissection, and chemotherapy. Her first recurrence of metastatic adenocarcinoma occurred 4 years later in a paraaortic lymph node and was successfully treated with carboplatin. She was then in remission for 11 years prior to presenting with cutaneous and muscle findings consistent with DM. After her diagnosis of DM, cancer antigen 125 (CA-125) and imaging studies were performed with increased frequency. Six months later, concomitant with a rise in CA-125 levels, she was noted to have recurrence of ovarian carcinoma in the paraaortic lymph nodes and received additional chemotherapy. Fifteen months later, she had a third recurrence in the left paraaortic lymph node, which was treated with radiofrequency ablation.

Four months after her third recurrence of ovarian adenocarcinoma, she presented with new cutaneous findings. On physical examination, multiple tender, soft, subcutaneous nodules with overlying erythema were palpated on her arms and thighs (Figure 1A). The contours of her arms, thighs, and buttocks were characterized by alternating areas of subcutaneous nodules and depressions. Examination was also remarkable for faint upper eyelid erythema, ragged cuticles, and livedo reticularis on the extremities.

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Figure 1. Clinical images. A, Confluent violaceous to erythematous patches with underlying soft, tender subcutaneous nodules before treatment with methotrexate. B, Improvement after treatment with methotrexate, including resolution of erythema, decreased nodularity, and absence of pain. Of note, lipoatrophy resulting from dermatomyositis-associated panniculitis is often permanent.

A biopsy of a subcutaneous nodule on the right upper extremity showed lobular lymphoplasmacytic panniculitis with associated dermal mucin deposition, consistent with lobular panniculitis in association with connective tissue disease (Figure 2). Special stains for bacteria, mycobacteria, and fungi were negative.

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Figure 2. Hematoxylin-eosin–stained punch biopsy. Tender subcutaneous nodule on right upper extremity (A) showing dermal mucin deposition (B) and lobular panniculitis with a lymphoplasmacytic infiltrate (C). (Original magnifications ×40, ×100, and ×600 for A, B, and C, respectively.)

Treatment was initiated with 20 mg/d of prednisone and 100 mg/d of azathioprine, leading to mild improvement. Azathioprine treatment was discontinued 3 weeks later secondary to leukopenia. The patient had received prior myelosuppressive chemotherapy, including paclitaxel, carboplatin, cisplatin, cyclophosphamide, doxorubicin, and gemcitabine, but the most recent dose was administered at least 9 months prior to the development of leukopenia. As an alternative steroid-sparing agent, mycophenolate mofetil was started, but the patient developed a generalized morbilliform eruption on her trunk and extremities 8 days later and chose to discontinue treatment. A trial of oral methotrexate therapy was then begun at a dose of 10 mg/wk and titrated up to 15 mg/wk. She also received folic acid, 1 mg/d, in conjunction with methotrexate. All immunosuppressive agents used in the treatment of the panniculitis were discussed and selected in collaboration with the patient's oncologist.

Within one month of starting methotrexate therapy, her nodules became less painful and erythematous. Corticosteroid doses were successfully tapered from 20 mg/d to 5 mg/d. She tolerated methotrexate well, with no evidence of elevated transaminase levels and with marked improvement of panniculitis (Figure 1B). Ten months later, she continued treatment with prednisone 5 mg/d and methotrexate 15 mg/wk with no tenderness or erythema and with decreased nodularity on her upper arms and thighs. Soon thereafter, her CA-125 level increased, and ovarian adenocarcinoma was detected in a left paraaortic node on positron-emission tomography/computed tomography. The ovarian adenocarcinoma was again treated with computed tomography–guided radiofrequency ablation. She continues to be followed by dermatology, rheumatology, and medical oncology. A timeline of the course of her ovarian cancer, DM, and panniculitis is depicted in Figure 3.

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Figure 3. Timeline depicting course of patient's ovarian cancer, DM, and panniculitis. b/l Indicates bilateral; c/b, complicated by; d/c’d, discontinued; DM, dermatomyositis; IVIG, intravenous immunoglobulin; MTX, methotrexate.


Panniculitis is a rare cutaneous manifestation of DM that most commonly presents as tender, erythematous subcutaneous nodules on the thighs, arms, and buttocks1,2 and can progress to form calcifications and lipoatrophy.25 The first documented case of panniculitis in DM was published in 1924 and described a 22-year-old woman who presented with painful inflammatory nodules on the arms and thighs, muscle weakness, facial edema, and a diffuse cutaneous eruption.6 Since then, approximately 20 additional cases have been reported,2,5,720 including several cases associated with juvenile DM.4,2124 Panniculitis may present prior to, concurrent with, or subsequent to symptoms of DM. In the current literature, date of onset ranges from 14 months preceding to 5 years following DM diagnosis.25

Histologic examination of DM-associated panniculitis is nonspecific, showing a lobular panniculitis with lymphoplasmacytic infiltration. The differential diagnosis of lobular panniculitis includes infectious panniculitis, which may be from bacteria, fungi, or mycobacteria, as well as other forms of connective tissue disease–associated panniculitis, the most common of which is lupus panniculitis. Interface vacuolar change and dermal mucin are suggestive of connective tissue disease–associated panniculitis, and membranocystic changes may indicate a worse prognosis.2 A histopathologic study demonstrated that 9% of skin biopsy specimens of patients with DM showed subclinical panniculitis,26 suggesting that microscopic panniculitis might be more common than clinically recognized. An additional report documented subclinical microscopic panniculitis in patients with DM examined post mortem.27 Thus, both specific and nonspecific panniculitis may potentially occur in connective tissue disease.28 Notably, however, these studies are several decades old, and there is an absence of more recent reports in the literature validating their findings.

The pathogenesis of panniculitis in DM is unknown. It has been suggested that panniculitis and DM may be part of the same disease process because both have been reported to follow parallel clinical trajectories, improving and recurring simultaneously.2,18,25 Panniculitis may be part of the active inflammatory phase of DM, which involves multiple immunologic effector mechanisms.25

Herein, we describe a case of lobular panniculitis responsive to combination therapy with prednisone and methotrexate in a patient with DM and recurrent ovarian adenocarcinoma. The case presented is unique in that the panniculitis not only occurred in a patient with DM, but also in the setting of recurrent ovarian adenocarcinoma. The association of panniculitis with malignancy in DM is rare and of undetermined significance. To our knowledge, only 2 other reports of panniculitis in the setting of DM and malignancy exist (Table). The first is a case of panniculitis in a 51-year-old man with rhabdomyosarcoma who developed DM two months after presentation with panniculitis. This patient had a transient response to prednisolone 50 mg/d.13 A second case of panniculitis occurred in a 56-year-old woman with lymphoepithelioma-like carcinoma of the left parotid gland and DM.15 No previous cases of DM-associated panniculitis in the setting of ovarian adenocarcinoma have been reported, despite the well-established association of DM with ovarian cancer.29 Moreover, no previous cases of DM-related panniculitis in the setting of recurrent malignancy have been reported. Given the limited number of cases of panniculitis in patients with DM and malignancy, the significance of this association remains to be determined. While it has previously been suggested that panniculitis in DM may correlate with a good prognosis,20 the case presented herein documents an association with ovarian adenocarcinoma and with recurrent malignancy, suggesting that panniculitis in DM may not indicate a favorable prognosis.

Table. Characteristics of Documented Cases of Dermatomyositis-Associated Panniculitis in the Setting of Malignancy
Table. Characteristics of Documented Cases of Dermatomyositis-Associated Panniculitis in the Setting of Malignancy
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Another unresolved aspect of the clinical management of panniculitis in DM is the most appropriate choice for therapy. Early treatment is critical because spontaneous resolution does not occur, and the complications can be debilitating.1 However, due to the infrequency of the association, no randomized controlled studies have been performed. Mixed success has been reported with systemic corticosteroids, mycophenolate mofetil, azathioprine, methotrexate, and IVIG, either alone or in combination.17 Antimalarial therapy, such as hydroxychloroquine, has not been as effective in DM-related panniculitis as in lupus panniculitis.1 A recent review by Hansen and Callen1 considered combination therapy with systemic corticosteroids and steroid-sparing immunosuppressants to be the first-line treatment for panniculitis in patients with DM. Our patient was treated with prednisone and methotrexate and experienced marked improvement in her panniculitis within one month. Improvement was sustained and corticosteroids were successfully tapered throughout a 10-month follow-up period. Three additional case reports have documented the successful use of corticosteroids and methotrexate in the treatment of DM-associated panniculitis,5,18,23 and one additional report describes clinical improvement after combination therapy with prednisolone, methotrexate, and cyclosporine A.25 In contrast, therapy with methotrexate in combination with azathioprine failed to improve cutaneous disease in one case of DM-associated panniculitis.5

In conclusion, we report the first documented case of DM-related panniculitis to our knowledge occurring both in association with ovarian adenocarcinoma and with recurrent malignancy. Our case also provides further support for the efficacy of combination therapy with corticosteroids and methotrexate in the treatment of DM-associated panniculitis. Given its rarity, it is difficult to ascertain the clinical significance of panniculitis associated with malignancy and DM, and we are unable to make generalizations based on this single case. However, further study and accumulation of case data may help to decipher the potential prognostic significance as well as the most beneficial therapeutic regimens. A multidisciplinary approach to care in patients with DM, including evaluation by rheumatologists, internists, and dermatologists, is the most effective method to identify and treat cases of panniculitis in DM. Although rare, DM and its potentially associated malignancies may need to be considered in patients presenting with idiopathic lobular panniculitis.

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Article Information

Correspondence: Ruth Ann Vleugels, MD, MPH, Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Ave, Boston, MA 02115 (rvleugels@partners.org).

Accepted for Publication: January 25, 2012.

Published Online: March 19, 2012. doi:10.1001/archdermatol.2012.288

Author Contributions: Dr Vleugels had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Girouard, Penson, and Vleugels. Acquisition of data: Penson. Analysis and interpretation of data: Velez, Penson, Massarotti, and Vleugels. Drafting of the manuscript: Girouard. Critical revision of the manuscript for important intellectual content: Velez, Penson, Massarotti, and Vleugels. Administrative, technical, and material support: Girouard, Velez, Penson, Massarotti, and Vleugels. Study supervision: Vleugels.

Financial Disclosure: None reported.

Additional Contributions: George Murphy, MD, provided expertise in dermatopathology; Jamie Martin, BA, assisted in manuscript preparation; and Rei Watanabe-Hosokawa, MD, PhD, assisted in Japanese translation.

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