|Year : 2021 | Volume
| Issue : 2 | Page : 138-141
Atypical cutaneous manifestations in lupus erythematosus: A report of three cases
Soumya Mary Koshy1, Remya Raj Rajamohanan1, Satyaki Ganguly2, Anita Ramdas3, Sheela Kuruvila1
1 Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Puducherry, India
2 Department of Dermatology, AIIMS, Raipur, Chhattisgarh, India
3 Department of Pathology, Pondicherry Institute of Medical Sciences, Puducherry, India
|Date of Submission||07-May-2021|
|Date of Decision||24-Jul-2021|
|Date of Acceptance||26-Jul-2021|
|Date of Web Publication||30-Dec-2021|
Remya Raj Rajamohanan
Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) can present with a wide morphological spectrum of cutaneous manifestations. In addition to its classical cutaneous presentations such as malar rash, discoid rash, annular, or papulosquamous lesions, there can also be a few unusual manifestations. We present a report of three patients evaluated in a tertiary care center satisfying the Systemic Lupus International Collaborating Clinics criteria for SLE and presenting with unusual manifestations such as bullous lesions, toxic epidermal necrolysis like picture, and coexisting with pemphigus foliaceous.
Keywords: Bullous systemic lupus erythematosus, cutaneous lupus erythematous, pemphigus erythematosus, systemic lupus erythematosus, toxic epidermal necrolysis-like lupus erythematosus
|How to cite this article:|
Koshy SM, Rajamohanan RR, Ganguly S, Ramdas A, Kuruvila S. Atypical cutaneous manifestations in lupus erythematosus: A report of three cases. J Curr Res Sci Med 2021;7:138-41
|How to cite this URL:|
Koshy SM, Rajamohanan RR, Ganguly S, Ramdas A, Kuruvila S. Atypical cutaneous manifestations in lupus erythematosus: A report of three cases. J Curr Res Sci Med [serial online] 2021 [cited 2022 Jun 29];7:138-41. Available from: https://www.jcrsmed.org/text.asp?2021/7/2/138/334457
| Introduction|| |
Skin is the second most common organ to be involved in lupus erythematosus (LE). Accurate knowledge and prompt diagnosis of cutaneous lesions in LE are important as they provide an idea about the extent of systemic involvement and disease activity. Patients with LE can have various atypical cutaneous manifestations in addition to common lesions such as malar rash and discoid rash. Uncommon and atypical manifestations such as bullous LE and toxic epidermal necrolysis (TEN) like presentation of LE can be life-threatening.
| Case Reports|| |
A 25-year-old female presented with 3 months history of erythematous non-itchy lesions over the face, trunk, and forearms for 3 months. She complained of development of blisters over the face and upper back for prior 2 weeks. She also had photosensitivity but no history of wheals before the onset of the blisters. On physical examination, multiple tense vesicles and bullae were seen with a predilection for the sun-exposed areas [Figure 1]a and [Figure 1]b. Few lesions on the back had coalesced to give a “string of pearls” appearance [Figure 1]c. An intact bulla was seen on the hard palate [Figure 1]d. Provisional diagnosis of linear IgA disease, bullous pemphigoid, and bullous SLE were considered.
|Figure 1: (a) Multiple vesicles over the face (b) Vesicles over the back showing predilection for photoexposed areas (c) Vesicles with “string of pearls” appearance over the upper back (d) Single vesicle on the soft palate|
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Relevant laboratory investigations showed anemia, leukopenia, elevated antinuclear antibody (ANA), and anti-dsDNA levels. Skin biopsy taken from a vesicle over the arm showed a subepidermal bulla with infiltration of neutrophils and eosinophils [Figure 2]a, while direct immunofluorescence (DIF) from unexposed skin showed deposition of IgG along the basement membrane zone [Figure 2]b. Twenty-four hour urine protein levels were high, and renal biopsy was suggestive of Grade II lupus nephritis.
|Figure 2: (a) Subepidermal bulla with inflammatory infiltrate (H and E, ×10) (b) Direct immunofluorescence showing linear deposition of IgG along the basement membrane zone|
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The features of the patient satisfied the American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria for the diagnosis of bullous SLE. She was started on oral prednisolone 1 mg/kg/day and hydroxychloroquine. Since she continued to develop new bullae, dapsone 50 mg OD was later added, and the patient showed excellent response after that.
A 33-year-old female who was a known case of auto-immune hemolytic anemia (AIHA) and hypothyroidism for 8 years, developed erythematous lesions over the face, arms, back with fever, and oral erosions for 1 week. She also had redness and discharge from the eyes for 2 days. She was on a maintenance therapy of oral prednisolone 10 mg/day and azathioprine 100 mg/day for AIHA for almost 8 years, which she had discontinued 2 months before the presentation. Examination revealed purpuric macules with epidermal detachment over the face and crusting over the lips and eyelids [Figure 3]a. Targetoid lesions were seen over the back, forearms, and thighs [Figure 3]b.
|Figure 3: (a) Coalescing purpuric macules and epidermal detachment over the face and neck with hemorrhagic crusting of lips (b) Purpuric macules and atypical target lesions over the back|
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On investigation, she had elevated anti-dsDNA antibodies levels. Skin biopsy showed subepidermal split with overlying epidermis showing necrosis and formation of apoptotic bodies [Figure 4]. A diagnosis of TEN type of acute cutaneous LE (ACLE) was made. Prednisolone 60 mg/day and cyclosporine 150 mg/day were started along with supportive treatment. The lesions started resolving within 10 days.
|Figure 4: Subepidermal bulla with necrosis and apoptotic keratinocytes in the epidermis (H and E, ×40)|
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A 45-year-old female presented with blisters, painful erosions, and scaling over the scalp, face, trunk, and upper limbs for 5 months. The lesions were associated with burning sensation over the face for 10 days. Clinical findings included widespread erythematous plaques over the face, upper trunk, back, and arms with scaling and crusted erosions [Figure 5]a and [Figure 5]b. Nikolsky's sign was positive. Skin biopsy revealed subcorneal cleft [Figure 6]a, while DIF showed intercellular epidermal staining of IgG and C3 [Figure 6]b. Pemphigus foliaceous was diagnosed, and she was started on dexamethasone and cyclophosphamide pulse therapy. On further evaluation, ANA and anti dsDNA were found to be high, thus satisfying ACR criteria for SLE. Based on these findings, SLE co-existent with pemphigus foliaceous was diagnosed and tablet hydroxychloroquine 200 mg twice daily was also added.
|Figure 5: (a) Erosions with crusting over the face and chest (b) Extensive erosions with flake-like scales over the back|
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|Figure 6: (a) Subcorneal cleft (H and E, ×40) (b) Direct immunofluorescence-intercellular epidermal staining of IgG and C3|
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| Discussion|| |
Cutaneous involvement occurs in 70%–85% of all patients with SLE. The skin manifestations in SLE can have a varied presentation and have been broadly classified as LE-specific and nonspecific skin lesions based on morphology and histologic features. LE-specific lesions such as DLE and lupus panniculitis show features such as interface changes, basement membrane thickening, perivascular and periadnexal mononuclear cell infiltrate, and dermal mucin deposition. These LE-specific lesions have been further subdivided into ACLE, sub ACLE, and chronic cutaneous LE. LE-nonspecific skin lesions refer to lesions occurring due to SLE, but the aforementioned histologic features are absent on biopsy. They include nonscarring alopecia, cutaneous vasculitis, livedo reticularis, and Raynaud's phenomenon. It is important to identify these nonspecific lesions because their presence may indicate systemic involvement as well as high disease activity.
Bullous lesions are LE nonspecific lesions which can occur in <1% of SLE patients and they may also be the first manifestation of ACLE in some patients. The lesions occur predominantly over sun-exposed areas and are seen more often in young adult females. Here, antibodies target collagen VII (noncollagenous domain 1) on the lamina densa. It is histologically characterized by subepidermal bullae containing predominantly neutrophils with immunoglobulin IgG, IgA, IgM, and C3 deposited at the basement membrane zone. The indirect immunofluorescence using salt split skin can help differentiate bullous SLE from other subepidermal blistering conditions as here the immunoreactants bind to the floor of the induced blister. In 1983, Camisa and Sharma proposed the following diagnostic criteria for bullous systemic LE: (1) Fulfilment of diagnosis of SLE based on ACR criteria, (2) vesicles and bullae on both sun-exposed and covered skin, (3) histopathology showing subepidermal blister with neutrophilic infiltrate in dermis, (4) DIF showing IgG and/or IgM and often IgA at BMZ, and (5) Indirect immunofluorescence revealing the presence of antibodies to Type VII collagen. Bullous LE is known to respond very well to dapsone therapy.
In ACLE with SJS-TEN like presentation, it is important to rule out classical drug-induced TEN. It is challenging to differentiate both as clinically both conditions present with atypical target lesions and epidermal detachment with involvement of two or more mucosae. Furthermore, TEN is known to occur more frequently in association with connective tissue disorder and such patients are also on multiple medications which increase their risk for a drug rash. The onset of skin lesions in ACLE associated SLE-TEN is insidious but responds to oral steroids much faster than drug-induced TEN. In the present study, the patient was already a known case of SLE, but the increasing levels of anti-dsDNA indicated increasing disease activity with the acute flare.
Pemphigus erythematosus (PE), which is an LE nonspecific skin lesion, is a differential diagnosis for patients with pemphigus foliaceous with SLE like our case. This term is controversial and Senear and Usher had classified PE as a mild localized form of pemphigus foliaceous mostly over the malar region with ANA positivity. However, in our case, the lesions were extensive; therefore, coexistent pemphigus foliaceous with SLE will be a more apt diagnosis.
| Conclusion|| |
The present case series indicates the importance of having a high index of suspicion to diagnose uncommon SLE presentations. Predilection for young females and photodistributed lesions should prompt for an evaluation of ANA, anti dsDNA titers, and complement levels. Skin biopsy and immunofluorescence tests are also useful, although not a part of ACR and SLICC criteria for the diagnosis of SLE. Prompt treatment with steroids and other steroid sparing immunosuppressants can help control disease activity. Patients should be advised on strict photoprotection, compliance to long-term medications, and regular follow-up.
The authors declare that signed consent forms have been obtained from patients for the publication of photographs.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given consent for their images and other clinical information to be reported in the journal. The patients understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]