panniculitis

1. ULCERATED LOBULAR PANNICULITIS: AN
UNUSUAL INITIAL PRESENTATION OF ANTI-
MI-2-ALPHA POSITIVE DERMATOMYOSITIS
SRI KRISHNA PRIYA M
DEPARTMENT OF DERMATOLOGY

2. • JOURNAL: IJDVL
• AUTHOR: Ambooken B, Balakrishnan PP, Asokan N, Krishnan J.
• LOCATION: Department of Dermatology, Venereology and Leprology,
General Medicine, Government Medical College, Thrissur,
Kerala, India
• YEAR: 2022
• CITATION: 10.25259/IJDVL_848_2021

3. INTRODUCTION
• Dermatomyositis (DM) is a chronic inflammatory disorder of the skin
and muscles.
• Atypical presentations of dermatomyositis often result in delayed
diagnosis.
• Atypical presentations- panniculitis, diffuse subcutaneous edema,
erythroderma, calcinosis, ulceration, flagellate erythema, Wong-type
DM, gingival telangiectasias, and the ovoid palatal patch.

4. CASE REPORT
• 30-year-old lady with no known comorbidities presented with non-productive
cough and low-grade fever for two months ;lost six kilograms of body weight
during this period.
• Noticed a painful subcutaneous swelling on the left arm. No h/o dysphagia or loss
of appetite.
• C/E: ill-defined, tender, erythematous, indurated swelling on the left arm
• Respiratory system examination revealed decreased breath sounds in the left
lower lung field
1 month later

5. • Hemogram – normal
• ESR- 26 mm in the first hour
• Serum C-reactive protein, blood sugar levels, Hepatic, renal, and thyroid function
tests - normal.
• ANA, Serology for HIV, hepatitis B and hepatitis C viruses - negative.
• Chest X-ray, HRCT of thorax - pleural effusion.
• Mantoux test - negative.
• Pleural fluid analysis - exudative with elevated protein of 4.6 gm%, raised
adenosine deaminase of 81 U/Ml, lymphocytes

6. • Ultrasonogram, HRCT of abdomen and pelvis- normal.
• Soft tissue ultrasonogram of the tender swelling - showed diffuse subcutaneous
oedema

7. • Low-grade fever, cough, loss of weight, exudative type of pleural effusion with
predominant lymphocytes and a raised adenosine deaminase in pleural fluid;
started on anti-tuberculosis therapy
• Developed oedema of the face and extremities, painful ulcers on both hands,
forearms and at the biopsy site, fever was persisting, had difficulty in getting up
from the squatting position and climbing stairs, no history of photosensitivity.
After 6 weeks of ATT

9. • Motor system examination - grade four power thigh muscles
• Clinical possibilities of dermatomyositis, systemic lupus erythematosus or overlap
syndrome possibly exacerbated by anti-tuberculosis therapy were considered, and it was
discontinued.

10. • Hemogram - normal (except Hb -9.2 mg/dl), ESR and C-reactive protein were
normal. Peripheral smear showed no features of haemolysis. Reticulocyte count
and Direct Coombs test was negative.
• Urine routine examination and renal function tests were normal. Repeat serology
for human immunodeficiency virus, hepatitis B, and C viruses - negative
LDH Normal
Aspartate aminotransferases 196 IU/L
Alanine aminotransferases 88 IU/L
Creatine phosphokinase 548 IU/L

11. • ANA, Anti-double stranded DNA, anti-Smith, anti-histone, anti-U1RNP and anti-
PM-Scl antibodies were negative and serum levels of complements (C3 and C4)
were normal; positive for anti-Mi-2-alpha antibody
• Repeat chest X-ray, HRCT thorax and ultrasonogram of abdomen and pelvis were
normal.
• MRI of both thighs showed mild oedema and patchy contrast enhancement
involving quadriceps femoris muscles, suggestive of myositis.
• Diagnosis of PROBABLE DERMATOMYOSITIS based on the Bohan and Peter
classification.

12. • TREATMENT:
• ATT was withdrawn, treated with dexamethasone 4mg twice daily intravenously
and hydroxychloroquine 200 mg twice daily per orally
• Methyl prednisolone one gm intravenously on three consecutive days
• Dramatic response.
Fever persisted and cutaneous ulcers progressed

14. • Continued on daily prednisolone at a dose of 40 mg, tapered by 10 mg per month
till it reached 20 mg, 400 mg hydroxychloroquine, 10 mg methotrexate once
weekly added.
• On 10 mg prednisolone, she developed a relapse of panniculitis.

15. • Daily dose of prednisolone was immediately increased to 20 mg.
• 2 doses of intravenous infusion of 1g rituximab two weeks apart.
• Lesions responded well, maintained on prednisolone 10 mg daily and
methotrexate 25 mg once weekly.
NO RESPONSE

16. DISCUSSION
• UNUSUAL FEATURES OF OUR CASE
• Ulcerated lobular panniculitis
• Ulceration of heliotrope rash
• Painless palatal erosion
• Cutaneous ulcers in the context of anti-Mi-2-alpha antibody-positive
dermatomyositis.

17. • Ulcerated heliotrope rash has been recently described in a patient with
dermatomyositis associated with anti-melanoma differentiation-associated gene
5(anti-MDA5) antibody.
• Asymptomatic palatal erythema with erosion observed in our patient differed
from the ovoid palatal patch, described by Bernet et al., in transcription
intermediary factor 1-gamma associated dermatomyositis

18. ORAL LESIONS IN DERMATOMYOSITIS
OUR STUDY BERNET
Painless eroded erythematous plaque on the hard
palate
Ovoid palatal patch, with typical arcuate symmetric
erythema on the hard palate intermixed with white
macules
Anti-Mi-2 positive Anti-TIF1-g antibody positive
Low incidence of malignancy Highly associated with internal malignancy
Good prognosis Poor prognosis

19. • LOBULAR PANNICULITIS:
• Panniculitis is a rare cutaneous manifestation of DM that most commonly
presents as tender, erythematous subcutaneous nodules on the thighs, arms, and
buttocks and can progress to form calcifications and lipoatrophy.
• Arias M, Cunha LGB, Hernández MI, Kien MC, Abeldaño A. Panniculitis in a patient with dermatomyositis. An Bras
Dermatol. 2011;86(1):146-8.
• Panniculitis may precede the other symptoms of dermatomyositis by up to 14
months; therefore, in cases of idiopathic panniculitis, it would be advisable to
measure muscle enzyme levels and evaluate the patient periodically.

20. • Histology shows lobular panniculitis with a lymphoplasmacytic infiltrate, fat
necrosis and consequent fibrosis.
• Difference between dermatomyositis and lupus panniculitis is that in the former
antimalarial drugs have minimal effect
• Presence of panniculitis in a patient with dermatomyositis is believed to be
indicative of good prognosis.

21. • ATT induced Dermatomyositis:
• Dourmishev AL, Dourmishev LA. Dermatomyositis and drugs.
Adv Exp Med Biol 1999;455:187–91

22. • RITUXIMAB IN REFRACTORY DERMATOMYOSITIS:
• Rohit Aggarwal,Cutaneous improvement in refractory adult and juvenile dermatomyositis after
treatment with rituximab Division of Rheumatology and Clinical Immunology, University of
Pittsburgh School of Medicine, 3601 5th Avenue, Suite 2B, Pittsburgh, PA, USA.
• Presence of perivascular B lymphocytes than endomysial Tlymphocytes in DM
muscle
• Activation of humoral immunity; B cell antigen-driven autoimmune response
• High serum concentrations of B-cell-activating factor
supports the role of the B cell in the pathogenesis of the of DM and JDM.

23. CONCLUSION
• Lobular panniculitis can be an initial manifestation of dermatomyositis that can
precede other features by months or years.
• Ulcerative lobular panniculitis, ulcerative heliotrope rash and painless palatal
erosion are rare manifestations of anti-Mi-2-alpha antibody-positive
dermatomyositis.
• Rituximab could be a promising therapeutic option in dermatomyositis, especially
in patients with refractory panniculitis.

24. REFERENCES
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and classification. Indian J Dermatol 2012;57:366–70.
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25. • 5. Bernet LL, Lewis MA, Rieger KE, Casciola-Rosen L, Fiorentio DF. Ovoid palatal patch; a novel
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26. THANK YOU