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E-IJD® - CASE REPORT
Year : 2022  |  Volume : 67  |  Issue : 3  |  Page : 314
Migratory panniculitis with autoimmune cholangitis and pancreatitis (IgG4-Related Disease): A rare presentation


1 Consultant Dermatologist, Jaslok Hospital and Research Centre, Mumbai, India
2 Gastroenterologist, Mumbai, India
3 Research Assistant, Dr Skin Pimples Pvt. Ltd., Mumbai, India
4 Consultant Dermatologist and Epidemiologist, Mumbai, Maharashtra, India

Date of Web Publication22-Sep-2022

Correspondence Address:
Ram H Malkani
Consultant Dermatologist, Jaslok Hospital and Research Centre, Room No. 200, Dr. G Deshmukh Marg, Mumbai, Maharashtra - 400 026
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_373_20

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   Abstract 


A 30-year-old woman presented with an acute-onset high-grade fever (103°F), multiple episodes of throbbing abdominal pain in the right quadrant, and pustular red lesions on the left leg. The radiological findings were suggestive of autoimmune cholangitis and pancreatitis. The skin lesions recurred on different portions of the body, and histopathological findings showed lobular and septal lymphohistiocytic infiltrate without vasculitis. The epidermis had focal ulcerations, and the dermis showed the presence of subcutaneous fat and an infiltrate of lymphocytes, histiocytes, and neutrophils (described as lobular and septal panniculitis without vasculitis). Based on the clinical and histopathological features, a diagnosis of migratory panniculitis was made. The patient was managed with analgesics, antibiotics, and a long course of steroids; the patient responded well to these medications. To the best of our knowledge, this is one of the first case reports of migratory panniculitis associated with IgG4-related disease. Patients with migratory panniculitis should be investigated for the presence of IgG4-related autoimmune disease. However, steroids remain the drug of choice when these conditions occur together.


Keywords: IgG4 disease, Migratory panniculitis, Multiple organs, Steroids


How to cite this article:
Malkani RH, Nagral A, Karmakar S, Setia MS. Migratory panniculitis with autoimmune cholangitis and pancreatitis (IgG4-Related Disease): A rare presentation. Indian J Dermatol 2022;67:314

How to cite this URL:
Malkani RH, Nagral A, Karmakar S, Setia MS. Migratory panniculitis with autoimmune cholangitis and pancreatitis (IgG4-Related Disease): A rare presentation. Indian J Dermatol [serial online] 2022 [cited 2022 Oct 6];67:314. Available from: https://www.e-ijd.org/text.asp?2022/67/3/314/356732





   Introduction Top


Erythema nodosum is a common form of panniculitis, an uncommon condition that involves the inflammation of subcutaneous fat. The disease presents as nodules or plaques on the body.[1],[2] A clinical variant of this condition is subacute nodular migratory panniculitis; it is characterized by transient nodular lesions on the extremities and was first described by Vilanova et al. in 1956.[3] Erythema nodosum includes a spectrum of diseases: erythema nodosum migrans, subacute nodular migratory panniculitis, and chronic erythema nodosum. Though authors have different opinions on the classification of these three conditions, a review by Requena et al.[2] described these three conditions as clinical variants within the overarching diagnosis of erythema nodosum. We present a rare case of migratory panniculitis with underlying autoimmune cholangitis and pancreatitis.


   Case Report Top


A 30-year-old woman presented with an acute-onset high-grade fever (103°F), multiple episodes of throbbing, non-colicky, non-radiating abdominal pain in the right quadrant, and pustular red lesions on the left leg [Figure 1]. She reported similar pustular lesions on other parts of the body in the past. The discharge from these cutaneous lesions grew Staphylococcus aureus sensitive to levofloxacin and clindamycin. The laboratory findings were raised total leucocyte counts (21450 per cubic mm) and altered liver enzymes (serum glutamic pyruvic transaminase (SGPT) – 62.7 U/L, alkaline phosphatase – 182 U/l, and gamma glutamyl transferase – 659 U/L). The ultrasonography of the liver showed parenchymal disease, splenomegaly, and an enlarged lymph node. The magnetic resonance cholangiopancreatography was normal. The liver biopsy (a report was available of the biopsy done elsewhere) showed lymphoplasmacytic inflammation with occasional portal microgranuloma, features suggestive of autoimmune cholangitis. A provisional diagnosis of pyoderma gangrenosum with autoimmune cholangitis was made. She responded well to antibiotics, analgesics, and steroids; the pain subsided, and the cutaneous lesions healed.
Figure 1: (a) Single, erythematous, crusted, indurated tender plaque on the right leg suggestive of erythema nodosum migrans. (b) Red pustular lesion on the other body parts in the past

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A year later, she had another episode of severe pain in the left hypochondrium with multiple pustular lesions on the medial aspect of the right knee and lateral aspect of the leg. She also reported multiple episodes of jaundice. A computerized tomography scan of the abdomen showed hepatosplenomegaly with grade IV severity and acute pancreatitis [Figure 2]a,[Figure 2]b,[Figure 2]c. The Bedside Index of Severity in Acute Pancreatitis score was 1, and the biochemical parameters were altered. A skin biopsy from the leg lesion showed lobular and septal lymphohistiocytic infiltrate without vasculitis. The epidermis had focal ulcerations, and the dermis showed presence of subcutaneous fat, and an infiltrate of lymphocytes, histiocytes, and neutrophils; described as lobular and septal panniculitis without vasculitis. These features were suggestive of panniculitis [Figure 3]a,[Figure 3]b, and [Figure 4]. Based on these features, a diagnosis of migratory panniculitis was made. The patient responded to analgesics, antibiotics, and a long course of steroids and was discharged.
Figure 2: (a) CT scan image showing enhancement along biliary tree cholangitis. (b) CT scan image showing bulky edematous tail of pancreas with surrounding inflammatory changes. (c) CT scan showing hepatomegaly and dilated portal vein

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Figure 3: (a and b) Histopathological image shows that the overlying dermis has perivascular lymphohistiocytic infiltrate (a) and lobular and septal panniculitis without vasculitis (b) (Hematoxylin and Eosin 4X)

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Figure 4: Histopathological image shows the presence of subcutaneous fat with infiltrate consisting of lymphocytes, histiocytes, and few neutrophils, suggestive of panniculitis (Hematoxylin and Eosin 10X)

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The patient was readmitted with abdominal pain and pustular lesions on the knees. The ultrasound of the abdomen and pelvis liver showed parenchymal disease, slightly atrophied pancreas with prominent main dilated pancreatic duct, and tiny hypoechoic cystic areas in the body of the pancreas. Thus, the complete diagnosis was autoimmune cholangitis with pancreatitis (potentially IgG4 type disease) along with migratory panniculitis. Although she was started on analgesics and antibiotics, she always responded well to steroids.

The last we heard from the relatives was that the patient had succumbed to the disease after being admitted for recurrent pain and new lesions.


   Discussion Top


We describe a rare presentation of panniculitis, migratory panniculitis, associated with systemic manifestations of IgG4-related autoimmune involvement of multiple organs. Though any organ may be involved in this condition, it is commonly seen in the liver, pancreas, kidneys, and adnexal structures, and the diagnostic feature is the presence of lymphohistiocytic infiltration along with IgG4-rich plasma cells.[4] The etiology of IgG4-related disease first described by Hamano et al. in 2001 is still unknown; however, there is a probable role of CD4+ cytotoxic T-cells and B-cells in the pathogenesis of this condition.[5],[6] It has also been postulated that infiltration of fibrocytes in the tissues may cause fibrosis.[5] The comprehensive clinical criteria for diagnosis of IgG4-related disease are clinical features showing the characteristic features (such as diffuse/localized swelling) in organs, elevated IgG4 (>135 mg/dL), and histopathological features of infiltration and fibrosis.[7] The probable criteria for the disease are 1 and 3. Furthermore, the team has also suggested that individual organ diagnostic criteria (such as cholangitis, kidney) may be used if the comprehensive criteria are not used for diagnosis.[7] The diagnostic criteria for IgG4-related sclerosing cholangitis include features of diffuse or segmental narrowing in the biliary tract (imaging features), hematological examination (elevated IgG4), coexisting autoimmune pancreatitis, and histopathological features (such as lymphocytic and plasmacytic infiltration) with an added optional criterion of steroid therapy.[8] The authors have suggested criteria 1 and 3 as definitive criteria for diagnosis of this condition (which were seen in our patient). We did not have IgG levels; these are a part of the comprehensive criteria but not a part of the definitive criteria for IgG4-related sclerosing cholangitis. However, a later study found that elevated serum IgG4 levels have poor specificity and predictive values in IgG4-related diseases.[9] Additionally, other antibodies such as anti-ENA and dsDNA may be negative, though ANAs may be present in low titers.[5] These are not included in the diagnostic criteria for IgG-related diseases. Steroids are the drugs of choice and rituximab is useful in those cases that are steroid-resistant.[5] Our case responded to steroids.

It has been reported that autoimmune pancreatitis with intra or extra-hepatic biliary tract strictures is mostly related to IgG4 disease.[10] Though it is common in older men, our case was a 30-year-old female. IgG4-related diseases may mimic malignancies and may also be associated with tumors.[11] Thus, these patients should be investigated for the presence of tumors, particularly in the gastrointestinal tract. The condition responds to corticosteroids; the effect is pronounced in the inflammatory phase.[4]

Migratory panniculitis may be associated with multiple factors such as infections, malignancies, or other systemic diseases.[1] The histopathological findings of septal infiltration without vasculitis are considered an important diagnostic feature of this disease.[1],[2] Unlike IgG4 disease, migratory panniculitis is common in females and occurs in the third decade of life; however, both these conditions respond to steroids.

We had diagnosed the initial lesions as pyoderma gangrenosum on clinical assessment. However, after the lesions recurred and a detailed biopsy report, we diagnosed it as a case of migratory panniculitis. We did a thorough review of the literature and found cases of panniculitis associated with pancreatic diseases, and subcutaneous nodules and itchy plaques in IgG4-related disease including a reference to IgG4-related skin diseases.[12],[13],[14],[15] However, to the best of our knowledge, this is one of the first case reports of migratory panniculitis associated with IgG4-related disease. We would like to present this case for its rare presentation. Furthermore, as a dermatologist, one should be aware of the underlying systemic associations of migratory panniculitis. These patients should also be investigated for the presence of IgG-4-related autoimmune disease. However, steroids remain the drug of choice when these conditions occur together.

Acknowledgements

We would like to acknowledge the Department of Radiology, Jaslok Hospital for all the radiological investigations for this case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Blake T, Manahan M, Rodins K. Erythema nodosum - A review of an uncommon panniculitis. Dermatol Online J 2014;20:22376.  Back to cited text no. 1
    
2.
Requena L, Yus ES. Panniculitis. Part I. Mostly septal panniculitis. J Am Acad Dermatol 2001;45:163-83; quiz 184-6.  Back to cited text no. 2
    
3.
Aguade JP, Pinol J, Vilanova X. [Subacute migrating nodular hypodermitis]. Ann Dermatol Syphiligr (Paris) 1956;83:369-404.  Back to cited text no. 3
    
4.
Nambiar S, Oliver TI. IgG4 Related Disease (IgG4 RD) [Updated 2019 Jun 04]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; May 10, 2022. Available from: https://www. ncbi.nlm.nih.gov/books/NBK499825/.  Back to cited text no. 4
    
5.
Maritati F, Peyronel F, Vaglio A. IgG4-related disease: A clinical perspective. Rheumatology 2020;59(Suppl 3):iii123-31.  Back to cited text no. 5
    
6.
Okazaki K, Umehara H. Current concept of IgG4-related disease. Curr Top Microbiol Immunol 2017;401:1-17.  Back to cited text no. 6
    
7.
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:21-30.  Back to cited text no. 7
    
8.
Ohara H, Okazaki K, Tsubouchi H, Inui K, Kawa S, Kamisawa T, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012;19:536-42.  Back to cited text no. 8
    
9.
Carruthers MN, Khosroshahi A, Augustin T, Deshpande V, Stone JH. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 2015;74:14-8.  Back to cited text no. 9
    
10.
Whitcomb DC. Autoimmune pancreatitis: Clinical manifestations and diagnosis, Friedman LS. (Section Editor) Grover S. (Deputy Editor) 2020. Available from: https://www.uptodate.com/contents/autoimmune-pancreatitis. [Last accessed on 2020 Apr 03].  Back to cited text no. 10
    
11.
Notohara K, Kamisawa T, Uchida K, Zen Y, Kawano M, Kasashima S, et al. Gastrointestinal manifestation of immunoglobulin G4-related disease: Clarification through a multicenter survey. J Gastroenterol 2018;53:845-53.  Back to cited text no. 11
    
12.
Garcia-Romero D, Vanaclocha F. Pancreatic panniculitis. Dermatol Clin 2008;26:465-70, vi.  Back to cited text no. 12
    
13.
Lopez A, Garcia-Estan J, Marras C, Castaño M, Rojas MJ, Garre C, et al. Pancreatitis associated with pleural-mediastinal pseudocyst, panniculitis and polyarthritis. Clin Rheumatol 1998;17:335-9.  Back to cited text no. 13
    
14.
Sato Y, Takeuchi M, Takata K, Ohno K, Iwaki N, Orita Y, et al. Clinicopathologic analysis of IgG4-related skin disease. Mod Pathol 2013;26:523-32.  Back to cited text no. 14
    
15.
Satou A, Notohara K, Zen Y, Nakamura S, Yoshino T, Okazaki K, et al. Clinicopathological differential diagnosis of IgG4-related disease: A historical overview and a proposal of the criteria for excluding mimickers of IgG4-related disease. Pathol Int 2020;70:391-402.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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