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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 763-765 |
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| Bullous lupus: An atypical initial presentation of systemic lupus erythematosus post-delivery with dramatic response to dapsone |
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Aditi Ajay Shende, Siddhi B Chikhalkar, Ketki Bhoite, Vidya D Kharkar
Seth G S Medical College and KEM Hospital, Mumbai, India
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Siddhi B Chikhalkar
Seth G S Medical College and KEM Hospital, Mumbai
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_495_21
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How to cite this article:
Shende AA, Chikhalkar SB, Bhoite K, Kharkar VD. Bullous lupus: An atypical initial presentation of systemic lupus erythematosus post-delivery with dramatic response to dapsone. Indian J Dermatol 2022;67:763-5 |
How to cite this URL:
Shende AA, Chikhalkar SB, Bhoite K, Kharkar VD. Bullous lupus: An atypical initial presentation of systemic lupus erythematosus post-delivery with dramatic response to dapsone. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:763-5. Available from: https://www.e-ijd.org/text.asp?2022/67/6/763/370306 |
Sir,
A 21-year-old female, post-delivery developed pruritic clear fluid-filled lesions over face, neck, trunk and extremities for two months and painful oral ulcers for 2 weeks associated with fever, easy fatigability, hair-fall, polyarthralgia, dyspnoea on exertion and dryness of the mouth. She had spontaneous abortion 2 years ago. Clinical examination revealed a cachexic patient with multiple tense bullae on normal looking skin over the face, neck, trunk and extremities [Figure 1]. Multiple ulcers on the hard palate and buccal mucosa. Direct and marginal Nikolsky's sign and bulla spread sign were negative. Systemic examination was normal. A provisional diagnosis of Bullous systemic lupus erythematosus (BSLE) was kept with differentials of bullous pemphigoid, linear IgA disease and bullous drug eruption.
Routine laboratory evaluation showed anaemia (Hb- 6.2 g/dL), leukopenia (2900 cells/mm3) and platelet 4 lakh cells/mm3. Biopsy from the vesicle showed sub-epidermal bulla with sparse neutrophil-dominant inflammatory infiltrate at the dermo-epidermal junction (DEJ) and perivascular lymphocytic infiltrate [Figure 2]. Direct immunofluorescence (DIF) showed linear staining of BMZ with IgG and C3 with epidermal anti-nuclear antibody (ANA) staining [Figure 3]. Indirect immunofluorescence (IIF) revealed epidermal ANA staining with IgG. Renal function was preserved but 24-hour urine protein was markedly raised (1428 mg/day). Complement levels were low (C3 = 24.8 mg/dL; C4 = 4.8 mg/dL); ANA was positive (1:320) and anti-double-stranded DNA was positive. ANA blot was positive for anti-Sm, anti-ribonucleoprotein, anti-nucleosome, anti- ribosomalP and anti-histone antibodies. Anti-phospholipid profile was negative. Renal biopsy revealed membranous lupus nephritis (Class V). Based on the above, the final diagnosis of BSLE (EULAR/ACR criteria, total score 37) with class V lupus nephritis was made. The patient was started on intravenous methylprednisolone pulse therapy (1 gm for 5 days) and hydroxychloroquine 300 mg once a day. After 5 days of pulse therapy tablet of prednisolone 40 mg once a day was added. For lupus nephritis tablet mycophenolate mofetil 500 mg twice a day was added after nephrology reference. Along with this, she was advised strict photoprotection and topical moderately potent corticosteroids. However, even after continuing this treatment, the patient kept on developing new bullae though her older lesions started to heal. Hence, she was planned for dapsone and a glucose-6-phosphate dehydrogenase assay was sent which was normal. For anemia, she was transfused 2 units of PCV after which her hemoglobin increased to 8.4 g/dl. She was then shifted to intravenous iron sucrose to bring up the hemoglobin to 10.2 g/dl and then tablet dapsone 50 mg per day was added to her treatment. She stopped developing new lesions the next day with the healing of existing lesions. After monitoring hemoglobin for 10 more days, the dose of dapsone was increased to 100 mg per day. Complete clearance of lesions with post-inflammatory hypopigmentation was noticed at end of 10 days [Figure 4]. Due to persistent proteinuria, tablet mycophenolate mofetil and tablet prednisolone 40 mg for 4 weeks (tapered to 30 mg) were continued. The patient is in remission for 9 months with the resolution of proteinuria in 3 months. | Figure 2: Subepidermal blister with neutrophil dominant infiltrate at DEJ and perivascular infiltrate (H and E, x40)
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 | Figure 3: DIF suggestive of linear staining of basement membrane with IgG and C3
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BSLE is a rare blistering disease with distinctive clinical, histologic and immunopathologic features.[1] Vesicles in Systemic lupus erythematosus (SLE) are formed due to: (1) interface vacuolar dermatitis; (2) association with other autoimmune blistering diseases; (3) autoimmune blistering disease related to anti-collagenVII (BSLE).[2] Histology shows subepidermal blister and neutrophil predominant perivascular infiltrate but SLE features are absent.[1] Criteria are used to diagnose BSLE [Table 1].[1] BSLE may or may not be associated with SLE flare.[3] A multi-centre study of 128 cases described lupus nephritis in 50% of cases, mainly class III or IV.[4] (seen in our case) Dramatic response to dapsone, with cessation of new lesions in 1-2 days is known.[1],[2],[3] Same was seen in our case. Dapsone is efficacious even at a low dose (25-50 mg) and should be started early.[1] Improvement in systemic symptoms does not match the cutaneous response with dapsone, thus requiring systemic corticosteroids and immunosuppressants (as in our case). The patient showed only partial improvement of skin lesions with immunosuppressants and complete response was seen only after the addition of dapsone. Recurrences are common on rapid withdrawal of dapsone; hence continued for 1 year.[5]
There are no case reports of SLE with bullous variant as initial presentation after delivery. To conclude, with BSLE as the initial presentation of SLE clinicians should bear in mind, that it is a diagnostic possibility while evaluating cases of vesiculobullous eruption and do in-depth investigations to rule out any underlying severe systemic involvement (especially renal).
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.
 References | |  |
| 1. | Grover C, Khurana A, Sharma S, Singal A. Bullous systemic lupus erythematosus. Indian J Dermatol 2013;58:492.  [ PUBMED] [Full text] |
| 2. | Padrão EMH, Teixeira LF, Maruta CW, Aoki V, Felipe da Silva AS, Kim EIM, et al. Bullous systemic lupus erythematosus - A case report. Autops Case Rep 2019;9:e2018069. |
| 3. | Hall RP, Lawley TJ, Smith HR, Katz SI. Bullous eruption of systemic lupus erythematosus. Ann Intern Med 1982;97:165-70. |
| 4. | de Risi-Pugliese T, Cohen Aubart F, Haroche J, Moguelet P, Grootenboer-Mignot S, Mathian A, et al. Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases. Semin Arthritis Rheum 2018;48:83-9. |
| 5. | Alahlafi AM, Wordsworth P, Wojnarowska F. The lupus band: Do the autoantibodies target collagen VII? Br J Dermatol 2004;150:504-10. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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