|LETTER TO THE EDITOR
|Year : 2022 | Volume
| Issue : 3 | Page : 112-114
Pancreatic involvement in a case of linear immunoglobulin a disease with ulcerative colitis: True or a coincidental association?
Preema Sinha1, Afreen Ayub1, Priyank Dhiman2, Saikat Bhattacharjee3, Deep Kumar Raman4, Kumar Alok1
1 Department of Dermatology, Base Hospital, Lucknow, Uttar Pradesh, India
2 Department of Gastroenterology, Command Hospital, Lucknow, Uttar Pradesh, India
3 Department of Radiodiagnosis and Imaging, Command Hospital, Lucknow, Uttar Pradesh, India
4 Department of Pathology, Command Hospital, Lucknow, Uttar Pradesh, India
|Date of Submission||28-Apr-2022|
|Date of Decision||24-May-2022|
|Date of Acceptance||31-May-2022|
|Date of Web Publication||05-Jul-2022|
Department of Dermatology, Base Hospital, Lucknow - 226 002, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sinha P, Ayub A, Dhiman P, Bhattacharjee S, Raman DK, Alok K. Pancreatic involvement in a case of linear immunoglobulin a disease with ulcerative colitis: True or a coincidental association?. Gastroenterol Hepatol Endosc Pract 2022;2:112-4
|How to cite this URL:|
Sinha P, Ayub A, Dhiman P, Bhattacharjee S, Raman DK, Alok K. Pancreatic involvement in a case of linear immunoglobulin a disease with ulcerative colitis: True or a coincidental association?. Gastroenterol Hepatol Endosc Pract [serial online] 2022 [cited 2022 Sep 30];2:112-4. Available from: http://www.ghepjournal.com/text.asp?2022/2/3/112/349946
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) or linear IgA disease is a rare autoimmune subepidermal bullous disease with a characteristic linear pattern of IgA on direct immunofluorescence (DIF). It has a bimodal distribution with a peak incidence at 5 years of age and in adulthood, which can occur either after puberty or around 60–65 years of age. Mostly, LABD is idiopathic; however, it can be associated with certain drugs, malignancy, systemic autoimmune diseases such as systemic lupus erythematosus, Crohn's disease, and ulcerative colitis (UC). There are numerous reports of inflammatory bowel disease (IBD), in particular, UC associated with this autoimmune blistering disease. However, the involvement of pancreas in a case of LABD is rare. We hereby report a case of a young male with this rare subepidermal blistering disease with an uncommon organ involvement.
A 31-year-old male with no known comorbidities presented with recurrent episodes of multiple itchy tense blisters on both extremities and trunk for the past 6 months with exacerbation of symptoms for the past 10 days. These blisters would rupture within a week to leave behind raw areas which healed with dark pigmentation and scar. He also gave 1-year history of abdominal pain and loose stools after food intake which was sometimes associated with blood in stools. There was no history of any offending drug or alcohol intake.
Dermatological examination revealed multiple polysized crusted plaques with vesicles along margins (cluster of jewel appearance) along with few clear fluid-filled as well as hemorrhagic vesicles and bullae predominantly on the extremities, equally involving both flexure and extensor aspects [Figure 1]. Multiple polysized erosions and postinflammatory hyperpigmented macules were also seen. There was no involvement of palms and soles, oral mucosa, and conjunctiva.
|Figure 1: Multiple polymorphic lesions, crusted plaques in annular and polycyclic arrangement, hemorrhagic crust, erosions, and multiple polysized scars on the extremities and buttocks|
Click here to view
Laboratory evaluations revealed raised white blood cell count- 12,500/L and an elevated erythrocyte sedimentation rate of 64 mm/h. Rest all investigations were within normal limit. Antinuclear antibody screening was negative.
Histopathological examination (HPE) from a fresh vesicle revealed a split at dermoepidermal junction with abundant polymorphs and occasional eosinophils within the blister cavity. Polymorphs could be seen infiltrating beneath epidermis with keratinocyte necrosis. Area around the blister showed marked spongiosis within the epidermis and a mixed perivascular infiltrate of polymorphs, lymphocytes, and histiocytes [Figure 2]a and [Figure 2]b. DIF from perilesional skin showed linear positivity (3+) along the basement membrane for antibody against IgA while being negative for IgG, IgM, and C3 [Figure 2]c. The above findings confirmed the diagnosis of LABD.
|Figure 2: (a and b) A subepidermal cleft with accumulation of neutrophils (a, H and E stain, ×100; b, H and E stain, ×200). (c) Direct immunofluorescence from perilesional skin showing linear positivity (3+) along the basement membrane for antibody against IgA while being negative for IgG, IgM, and C3. Ig: Immunoglobulin|
Click here to view
Ultrasonography abdomen revealed features of calcific pancreatitis while contrast-enhanced computed tomography of the abdomen revealed multiple large calcifications in pancreatic parenchyma with multiple intraductal calculi in the main pancreatic duct [Figure 3]. Colonoscopy revealed pseudopolyps in the transverse colon and descending colon, grade 3–4 colitis till cecum with backwash ileitis. HPE revealed chronic active ileocolitis with inflammatory pseudopolyp favoring UC. Based on the clinical picture and above findings, he was diagnosed by a gastroenterologist as moderate severe UC.
|Figure 3: Contrast-enhanced computed tomography abdomen revealing multiple large calcifications in pancreatic parenchyma with multiple intraductal calculi in main pancreatic duct|
Click here to view
The patient was started on oral prednisolone (0.5 mg/kg) and the dose decreased gradually; concomitantly, dapsone was started at the dose of 100 mg/day. For his IBD, he was started on azathioprine and mesalazine tablets. The patient showed improvement within days and is presently on follow-up.
LABD is a rare, acquired, autoimmune, subepithelial vesiculobullous disease, which is primarily caused by IgA autoantibodies directed against heterogeneous antigens in the basement membrane (BM) of the skin and mucosae., These heterogeneous antigens of the BM include LABD97, LAD-1, BPAg2, laminin-γ1 chain, BPAg1, LAD285, and collagen VII.
There is no dearth in case reports documenting the association between IBD, a chronic recurrent disease characterized by abdominal pain, diarrhea, and tenesmus with LABD; however, conclusive evidence is still lacking. The onset of UC is generally before LABD, likely triggering this rare blistering disease as seen in our case; however, no association is found between the activity of UC and LABD.
Apart from IBD, LABD is known to be associated with other disorders of autoimmune or other origin, which includes pancreatic lipase deficiency, chronic hepatitis, celiac disease (gluten-sensitive enteropathy), and both lymphoid and nonlymphoid malignant diseases.,,,,
There is enough evidence to show that autoimmune diseases can coexist in the same patient, either sequentially or concurrently, and multiple factors including immunologic, genetic, endocrine, and environmental contribute to this coexistence. Several reports have made it increasingly clear that autoimmune bullous disorders may represent the cutaneous manifestation of a multiorgan disease because of potential common pathogenic mechanisms.
Pancreatitis is an inflammatory injury which leads to the self-digestion of pancreatic tissue and can be both acute and chronic, and pancreatic calcification is a diagnostic feature of chronic pancreatitis even in the absence of the clinical signs and symptoms. In our case, the patient had chronic calcific pancreatitis. There is no reported case of pancreatitis in LABD; however, inflammation in this mixed gland is not uncommon in a case of IBD. Pancreatitis in patients with IBD is generally either due to drugs or sclerosing cholangitis., Largely, pancreatic calcifications, as in chronic calcific pancreatitis, have been associated with alcohol abuse, but less common causes are developmental and neoplasm.,
In case no etiological factors can be determined, as in our case, the term idiopathic pancreatitis is used, which if occurring in association with IBD has been classified as an extraintestinal manifestation of IBD. There are some explanations which have been given by various authors to find a link between pancreatitis and IBD. Genetic susceptibility loci, immune dysregulation, and alteration of microflora of immune response disorders play an important role in pancreatitis and IBD.
It is difficult to ascertain the exact cause of pancreatitis in our patient, although literature search does suggest some association between IBD and pancreatitis, however the role of LABD in involvement of pancreas needs further research but the coexistence of the three conditions in one patient is unique hence reported by us.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Fortuna G, Marinkovich MP. Linear immunoglobulin A bullous dermatosis. Clin Dermatol 2012;30:38-50.
Chen S, Mattei P, Fischer M, Gay JD, Milner SM, Price LA. Linear IgA bullous dermatosis. Eplasty 2013;13:ic49.
Shipman AR, Reddy H, Wojnarowska F. Association between the subepidermal autoimmune blistering diseases linear IgA disease and the pemphigoid group and inflammatory bowel disease: Two case reports and literature review. Clin Exp Dermatol 2012;37:461-8.
Antiga E, Caproni M, Fabbri P. Linear immunoglobulin a bullous dermatosis: Need for an agreement on diagnostic criteria. Dermatology 2013;226:329-32.
Vassileva S, Drenovska K, Manuelyan K. Autoimmune blistering dermatoses as systemic diseases. Clin Dermatol 2014;32:364-75.
Jones MR, Hall OM, Kaye AM, Kaye AD. Drug-induced acute pancreatitis: A review. Ochsner J 2015;15:45-51.
Lesniak RJ, Hohenwalter MD, Taylor AJ. Spectrum of causes of pancreatic calcifications. AJR Am J Roentgenol 2002;178:79-86.
Barthet M, Hastier P, Bernard JP, Bordes G, Frederick J, Allio S, et al.
Chronic pancreatitis and inflammatory bowel disease: True or coincidental association? Am J Gastroenterol 1999;94:2141-8.
[Figure 1], [Figure 2], [Figure 3]