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4mediterranean journal
of RHEUMATOLOGY
33 3
2022
GRathore U, Nigam N,
This work is licensed under a Creative Commons Attribution 4.0 International License.
LETTER TO THE EDITOR
Panniculitis as the Transformed Cutaneous Manifestation of Refractory Dermatomyositis with Successful Management with Tofacitinib
Upendra Rathore1 , Neha Nigam2 , Amita Aggarwal1 , Latika Gupta1
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India, 2Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Mediterr J Rheumatol 2022;33(3):380-3 https://doi.org/10.31138/mjr.33.3.380
Article Submitted: 5 Mar 2021; Article Accepted: 20 Mar 2021; Available Online: 30 Sep 2022
Keywords:
A 26-year-old girl presented in 2017 with fever, proximal muscle weakness, and cutaneous rashes of one-year duration. A diagnosis of Dermatomyositis (DM) was made based on heliotrope and malar rashes, Gottron's sign, and cutaneous ulcers on the elbows, and significant proximal muscle weakness with a manual muscle testing score of 37/80 with elevated muscle enzymes (AST- 85U/L, ALT-53U/L, LDH-701U/L, CPK-303U/L). Myositis specific antibodies (MSA) were negative. Upon initiating 1 mg/kg glucocorticoids and methotrexate, a rapid improvement in muscle weakness was recorded over 8 weeks though cutaneous disease persisted. Subsequently various drugs (Hydroxychloroquine, Rituximab, Tacrolimus, Mycophenolate mofetil and Thalidomide) were unsuccessfully tried for refractory cutaneous disease over the next two years. Eventually Intravenous Immunoglobulin (IvIg) (2gm/kg 4-weekly doses) was initiated, following which rashes subsided and glucocorticoids were tapered and stopped over the next ten months.
The ongoing pandemic led to disruption of infusions for
three months due to a nationwide lockdown. The patient returned with painful subcutaneous nodules on the arms and forearm. On examination, a faint malar rash, Gottron's sign, and erythema nodosum (EN) like lesions were noted on the right arm, forearm
(Figure 1A), and thigh without overlying skin changes. Heliotrope rash, cutaneous ulcers and muscle weakness were absent. At this juncture, the EN-like lesions were believed to be due to an underlying panniculitis. A flare of DM was the prime differential, others being drug-induced, post-infectious, and granulomatous panniculitis. Although calcinosis and carcinomatous deposits presenting like this have been occasionally described in DM, these seemed unlikely. A skin biopsy confirmed lobar panniculitis with perivascular lymphocytic aggregates with vasculitis (Figure 1B-D). Tofacitinib was prescribed at 0.5 mg/kg GCs, to which she responded well with subsidence of skin lesions and normalisation of muscle enzymes 2 months later.
Our case was unique in panniculitis being the transformed cutaneous manifestation of DM in a girl with muscle involvement at the outset, which progressed to a refractory amyopathic disease many years into the illness. Panniculitis, the inflammation of subcutaneous fat is rarely reported in DM. After the first published case in 1924, nearly 60 cases have been reported till date, most being in adults with female predominance.1 In most cases the panniculitis precedes or is concurrent with myositis, while in our case it appeared after four years of the disease. Typically, panniculitis presents as thickened and firm nodules or plaques with erythematous or pigmentation overlying skin with pain and tenderness as in our case. Post-infectious (bacterial, fungal, mycobacterial) and drug-induced EN are prime differentials. The exact pathogenesis of panniculitis in myositis is unclear. On Histopathology, DM associated panniculitis shows predominantly lobular panniculitis along with lymphoplasmacytic infiltrate, and may rarely occur with
Corresponding Author:
Latika Gupta
Assistant Professor, Department of Clinical Immunology Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, India, 226014 E-mail: drlatikagupta@gmail.com Tel.: +91 0522-2495182
380 Cite this article as: Rathore U, Nigam N, Aggarwal A, Gupta L. Panniculitis as the Transformed Cutaneous Manifestation of Refractory Dermatomyositis with Successful Management with Tofacitinib. Mediterr J Rheumatol 2022;33(3):380-3.
PANNICULITIS IN IIM
Figure 1. (a) Picture of the right arm and forearm depicting erythema nodosum like lesions. (b) Infiltration of inflammatory cells within the lipocyte lobules, surrounding the adipocytes (arrow). Inset show perivascular lymphocytic infiltrate; H&E stain; 400x. (c) Dermo-epidermal inflammatory cells with focal infiltration of inflammatory cells in the epidermis (arrow); H&E stain; 400x. (d) Dermis shows mild peri-adnexal and perivascular lymphoplasmacytic infiltrate with foci of lymphocytic vasculitis (arrow).H&E stain; 400x.
vasculitis, as in our case. Depending on the aetiology, it may exhibit calcium, mucin, lipo-membranous changes, or rarely cancerous deposits.3 Panniculitis with vasculitis (as in our case) is even rarer. Previously around ten cases of DM associated panniculitis with vasculitis are reported. It may be a surrogate for more severe disease, and often requires aggressive management.2 Generally, panniculitis signify a more favourable prognosis than calcinosis.3 Complications like extreme pain, lipoatrophy and calcification may lead to high morbidity. Previous studies suggest successful management of EN with methotrexate, though panniculitis has reportedly occurred in certain cases while on methotrexate.4 MMF, CYC, and Ivlg have been occasionally used for management.5,6 Our patient was unique in being refractory to numerous immunosuppressants at the outset, requiring the usage of JAKinibs for management of panniculitis. Although interferon signatures have not been studied in DM associated panniculitis, we expect the pathway to be operative and hope JAKinibs to manage the condition successfully.
CONFLICT OF INTEREST
The authors declare no conflicts of interest.
AUTHOR CONTRIBUTIONS
UR and LG collected and analysed the clinical data. All
authors were involved in writing and reviewing the manuscript, and in manuscript ideation and preparation.
ETHICAL PUBLICATION STATEMENT
Participant provided informed consent for the photographs included in this paper. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
FUNDING
No funding required.
AVAILABILITY AND DATA SHARING
The data used during the current study is available from the corresponding author on reasonable request.
CONSENT FOR PUBLICATION
Consent for publication of pictures was given by the participant.
REFERENCES
1. Santos-Briz A, Calle A, Linos K, Semans B, Carlson A, Sangueza OP, et al. Dermatomyositis panniculitis: a clinicopathological and immunohistochemical study of 18 cases. J Eur Acad Dermatol Venereol 2018;32(8):1352-9.
2. Crowe WE, Bove KE, Levinson JE, Hilton PK. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum 1982 Feb;25(2):126-39.
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3. Chairatchaneeboon M, Kulthanan K, Manapajon A. Calcific panniculitis and nasopharyngeal cancer-associated adult-onset dermatomyositis: a case report and literature review. Springerplus 2015;4:201.
4. Girouard SD, Velez NF, Penson RT, Massarotti EM, Vleugels RA. Panniculitis associated with dermatomyositis and recurrent ovarian cancer. Arch Dermatol 2012 Jun;148(6):740-4.
5. Galimberti F, Kooistra L, Li Y, Chatterjee S, Fernandez AP. Intravenous immunoglobulin is an effective treatment for refractory cutaneous dermatomyositis. Clin Exp Dermatol 2018 Dec;43(8):906-12.
6. Moghadam-Kia S, Charlton D, Aggarwal R, Oddis CV. Management of refractory cutaneous dermatomyositis: potential role of Janus kinase inhibition with tofacitinib. Rheumatology (Oxford) 2019 01;58(6):1011-5.
PANNICULITIS INN IIM
Supplementary Table 1. Baseline investigations at presentation.
Investigation Reference value {conventional units) Value
Hemogram
Hemoglobin (gm/dl) 13-17 13.9
White blood count (per mm3) 4400-11000 14400
Platelet count (lakhs per mm3) 1.50-4.00 90000
Muscle enzymes
Creatine Phosphokinase (u/l) 20-192 303
Lactate dehydrogenase (u/l) 100-248 701
Alanine aminotransferase (u/l) 8-40 53
Aspartate aminotransferase (u/l) 8-40 85
Autoantibodies
Myositis specific antibody Negative Negative
Anti-Nuclear antibody Negative 2+ Homogenous
dsDNA antibody (lU/dl) <10 42.2
ENA Negative SS-A/Ro 60 and 52 kd
Acute phase reactants
ESR (mm/hr) 0-10 135
C reactive protein (mg/dl) 0-0.6 1.68
Others
Creatinine (mg/dl) 0.6-1.5 0.7
Alkaline phosphatase (u/l) 60-270 311
Skin Biopsy Negative Panniculitis
Abbreviations- dsDNA- double stranded deoxy ribo-nucleic acid ENA- extractable nuclear antigen ESR- erythrocyte sedimentation rate
