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Indian J Dermatol
Indian J Dermatol
IJD
Indian J Dermatol
Indian Journal of Dermatology
0019-5154
1998-3611
Wolters Kluwer - Medknow India

IJD-69-365d
10.4103/ijd.ijd_214_23
E–IJD®: Case Report
Walking a Tight Rope: Occult Lymphoma in a Case of Resistant Dermatomyositis Complicated by Tubercular Lymphadenitis and Gumma
Chhabra Namrata
Rahim Jemshi S.
Ganguly Satyaki
From the Department of Dermatology, AIIMS, Raipur, Chhattisgarh, India
Address for correspondence: Dr. Namrata Chhabra, Department of Dermatology, AIIMS, Raipur, Chhattisgarh, India. E-mail: namrata81@aiimsraipur.edu.in
Jul-Aug 2024
19 8 2024
69 4 365365
2 2023
4 2024
Copyright: © 2024 Indian Journal of Dermatology
2024
https://creativecommons.org/licenses/by-nc-sa/4.0/ This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
Dermatomyositis (DM) is an autoimmune inflammatory disease, characterized by symmetrical proximal myopathy and cutaneous manifestations. DM is associated with upto a 6-fold increased risk of cancer. Complications secondary to underlying cancer are a leading cause of mortality in DM. Here, we discuss the two year clinical course of an elderly male with recalcitrant DM. This case was complicated by tubercular lymphadenitis followed by gumma. Subsequently, he was diagnosed with lymphoma and succumbed to death. This case emphasizes the need to do extensive malignancy screening at regular intervals in DM.

KEY WORDS:

Dermatomyositis
paraneoplastic
tuberculosis
==== Body
pmcIntroduction

Dermatomyositis (DM) is an inflammatory myopathy, which usually manifests as a subacute proximal myopathy, systemic inflammatory process and a characteristic skin rash, with a 32% risk of cancer diagnosed within 3-5 years after the onset of the disorder.[1] It has been well established that dermatomyositis is strongly associated with a variety of malignancies including carcinomas of the breast, colon, ovaries, nasopharynx, melanoma and non-Hodgkin’s lymphoma, which occur primarily as a paraneoplastic syndrome. In 40% of the cases, DM presents before the development of cancer, in 26% is concurrent, and in 34% presents after any cancer manifestations.[23] Here, we discuss the clinical evolution of an elderly male with DM who subsequently developed lymphoma and the need to study occult neoplasm at regular intervals.

Case Report

A 62-year-old, diabetic man presented to our outpatient clinic in June 2018 with one-month history of rapid progression of photosensitive red rashes over the face, back of neck and knuckles along with proximal muscle weakness of the upper and lower extremities.

Examination revealed diffuse violaceous erythema involving the face predominantly the malar eminences including nasolabial folds, upper eyelids (Heliotrope rash) and forehead, associated with periorbital oedema [Figure 1]. There was diffuse erythema involving posterior aspect of the neck (Shawl sign). Multiple well-defined erythematous to violaceous papules and plaques of varying sizes were present over the metacarpophalangeal joints of bilateral hands (Gottron’s papule). Scalp showed diffuse non-scarring alopecia. Muscles across the shoulder and pelvic girdle were of power grade 4/5. Systemic examination was within normal limits.

Figure 1 Diffuse violaceous erythema involving the face predominantly the malar eminences, upper eyelids (Heliotrope rash) and Gottron’s papule over the metacarpophalangeal joints

The clinical findings were suggestive of DM, which was supported by histopathology from skin biopsy, muscle biopsy, and elevated creatinine kinase level (262 u/l). Biopsy from the left quadriceps muscle confirmed myopathic changes. ANA was positive with nuclear homogenous pattern. Muscle-specific antibodies were negative. Initial assessment for malignancy did not yield any positive results.

The patient was initiated on treatment with oral prednisolone (40 mg/day) and hydroxychloroquine (200 mg twice daily). A satisfactory response was observed in the first 4 weeks. Methotrexate was added as steroid-sparing drug, but complicated by pancytopenia and transaminitis and hence was discontinued. Subsequently, azathioprine (50 mg/day) was introduced, but it failed to maintain remission when steroid was tapered.

Meanwhile, the patient consulted an expert dermatologist from a tertiary care centre in Lucknow, where he was given intravenous immunoglobulin, and started on oral mycophenolate mofetil 500mg QID. The skin lesions improved; however, the muscle weakness did not show significant improvement. In May 2019, the patient presented with significant weight loss (7 kg) and painful swelling over the left biceps, which was suspected to be due to pyomyositis for which he was treated accordingly with IV antibiotics and he responded.

In October 2019, the patient developed painless lymphadenopathy of right axillary region. PET-CT was performed, which showed inflammatory changes in the right axillary, left supraclavicular and aortic canal abdominal lymph nodes. USG-guided biopsy from left supraclavicular lymph nodes showed ill-defined granulomas, which were AFB negative, but excision biopsy from right axillary lymph node showed necrotising granulomatous inflammation and AFB positivity, for which patient was started on antitubercular treatment (ATT). The steroids and MMF were continued in low dosage.

Till March 2020, patient`s condition got worsened in form of proximal muscle weakness and cutaneous signs and symptoms related to dermatomyositis [Figure 2]. In addition, he developed multiple fluctuant swellings over the right elbow region, back and left arm, which ruptured and revealed purulent discharge [Figure 3]. Pus from these lesions was positive for AFB. A diagnosis of tubercular gumma was made and ATT was continued. For recalcitrant DM, the patient was again started on IVIG and MMF was replaced with cyclosporine.

Figure 2 Worsening of cutaneous rash in the patient despite on steroids and mycophenolate mofetil and receiving intravenous immunoglobulin

Figure 3 Ruptured tubercular gumma over back of the patient

The gummatous lesions healed slowly with puckered scarring, In May 2020, he developed flare-up of DM in the form of cutaneous vasculitic lesions over legs [Figure 4]. Muscle power was grade 2 in the right shoulder girdle and grade 3 in the rest of the proximal muscles. Muscle enzyme levels were markedly elevated (CK-MB = 5.9, LDH = 696 and aldolase = 3.81). Despite the aggressive immunosuppressive therapy for 18 months, he continued to develop cutaneous flare-ups and progressive weakening of muscles severe enough to impair his routine activities. Cyclosporine was stopped in view of hypomagnesemia, and dose of mycophenolate mofetil was escalated to 2.5 grams in divided doses. USG abdomen showed conglomerated partially necrotic retroperitoneal lymphadenopathy suggestive of abdominal TB. ATT was extended for a total duration of 18 months.

Figure 4 Cutaneous vasculitis lesions over both legs in the patient

In view of ongoing coronavirus pandemic, in July 2020, the patient could not follow-up regularly, and IVIG was stopped. However, rest of the treatment was continued through telemedicine-dermatology OPD.

Taking into account the refractory disease course, and persistent lymphadenopathy even after ATT course, a repeat workup for paraneoplastic aetiology was performed. Blood tests revealed anaemia (Hb-6.3 g/dl) and elevated ESR (150 mm) levels. PET-CT showed multiple FDG avid hypodense lesions in the spleen with lymph nodal mass involving left supraclavicular, abdominal and retroperitoneal lymph nodes suggestive of neoplastic etiology [Figure 5]. Biopsy performed in October 2020 from an abdominal lymph node was reported as non-Hodgkin’s lymphoma (NHL). The patient was referred to an oncologist, and chemotherapy with rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP) regime was given for a total of three cycles. Unfortunately, he succumbed to death three months after the diagnosis of NHL.

Figure 5 PET-CT of the patient showing FDG avid hypodense lesions in the spleen with lymph nodal mass involving left supraclavicular, abdominal and retroperitoneal lymph nodes suggestive of neoplastic etiology

Discussion

DM is an autoimmune inflammatory disease, characterized by symmetrical proximal myopathy and cutaneous manifestations.[2] DM is associated with up to a 6-fold increased risk of cancer particularly in the first 2 years after diagnosis; its incidence is 1/100,000, with 15-30% of the cases being paraneoplastic.[45] Our patient had a negative initial malignancy evaluation and was subsequently found to have an occult malignancy two years after DM presentation.

The pathophysiologic mechanism linking systemic autoimmunity and malignancies is not well defined, but it has been postulated that antibodies produced against tumour antigens cross-react with antigens temporarily expressed during normal muscle regeneration and cause muscle inflammation.[67]

The risk factors to suspect the association between DM and cancer include advanced age, male, dysphagia, cutaneous vasculitis, skin necrosis and accelerated progression of the disease.[489] Our patient had refractory DM and developed cutaneous vasculitic lesions on treatment.

Various cancer types have been described in association with dermatomyositis, particularly breast, ovarian, lung and hematologic cancers, as well as nasopharyngeal cancer in Asian populations. Epidemiological evidence shows an association between dermatomyositis and malignancy, including non-Hodgkin’s lymphoma.[10] Immunosuppressive therapy is also known to increase the risk of developing malignancy.[11] Our patient was on immunosuppressants for two years before he was diagnosed with lymphoma.

Complications secondary to underlying cancer are a leading cause of mortality in DM, and thus, it follows that early identification of malignancy may allow for improved outcomes.[12] Data from 2 large US dermatology cohorts suggest that effective malignancy screening of DM patients often requires evaluation beyond a history, physical examination and ‘age-appropriate’ cancer screening and other modalities, such as CT scans are indicated to detect the majority of latent malignancies in the DM population.[13]

Tuberculosis (TB) reactivation has been commonly reported in DM patients after start of immunosuppressants. Our patient developed TB lymphadenitis and subsequently developed TB gumma while on ATT. This phenomenon is defined as ‘paradoxical reaction,’ which manifests as deterioration of preexisting tuberculous lesions or development of new lesions during anti-TB treatment.[1415] Paradoxical tuberculous reaction is more common in HIV-positive TB patients.[16] A similar case has been described by Gao et al.[17] in a patient with DM who developed new subcutaneous tuberculous abscesses after 2 months of anti-TB treatment. Tuberculous infection should be kept in mind when an unexplained swelling occurred in the limbs of immunocompromised patients.

Conclusion

This case describes a paraneoplastic DM as the initial presentation of a cutaneous lymphoma. When a diagnosis of DM is made, a screening for malignancy should follow because of the increased morbidity and higher risk of mortality. Recalcitrant dermatomyositis may be indicative of malignancy, even in those whose initial malignancy screening was unremarkable. Our case reaffirms the school of thought that patients with intractable disease should be subjected to more frequent and extensive malignancy screening.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
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