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Case Report 


Sara Gari et al, 2019;3(7):628–631.

International Journal of Medicine in Developing Countries.

An atypical presentation of discoid lupus erythematosus

Sara Gari1*, Ghaidaa Sindi2, Rahaf Alturkistani2, Mayar Almehmadi2

Correspondence to: Sara Gari

*Department of Dermatology, King Abdul-Aziz University Hospital, Jeddah, Saudi Arabia.

Email: gari_sa [at] yahoo.com

Full list of author information is available at the end of the article.

Received: 16 January 2019 | Accepted: 31 January 2019


ABSTRACT

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus. Typical skin lesions of DLE present as red or purple macules, papules, or small plaques that rapidly develop a hyperkeratotic surface. Lesions in untreated patients will be complicated by skin atrophy and scarring alopecia. Differential diagnosis includes a wide spectrum of medical diseases and may be induced by some drugs. We present a case of atypical DLE discoid skin ulcers that were confirmed histopathologically to be DLE with negative systemic lupus erythematosus markers.


Keywords:

Lupus erythematosus, pneumonia, discoid lupus, lupus panniculitis.


Introduction

Discoid skin lesions are seen in patients with a wide spectrum of medical diseases, such as cutaneous lupus erythematosus (CLE), cutaneous sarcoidosis, erythema multiforme, generalized granuloma annulare, Sweet’s syndrome, and fungal infections [1]. Moreover, discoid lesions may be induced by some drugs, mainly fluorouracil agents or tumor necrosis factor-α antagonists. Among these conditions, discoid lupus erythematosus (DLE) is the most common [2].

DLE is a type of chronic CLE that presents in skin and mucosa, with no systemic involvement. The typical cutaneous lesions of DLE appear as erythematous, edematous, and scaling papules with size ranges from a few millimeters to a few centimeters. The papules then progress and spread centrifugally, coalescing into plaques. Lifting of the scales reveals a carpet-tack appearance with dilated pilosebaceous orifices occupied by horny plugs. Healing of the lesions occurs in the center, resulting in atrophy, scarring, telangiectasia, pigmentations, and scarring alopecia [3,4].

We present a case of multiple atypical ulcerated skin plaques that were confirmed histopathologically to be DLE with negative systemic lupus erythematosus (SLE) markers.


Case Report

A 42-years-old Saudi female, known to suffer from type 2 diabetes, hypertension, and had a past gastric sleeve 10 years ago, presented to the dermatology clinic in King Abdul-Aziz University Hospital in July 2017 with painful itchy erythematous ulcerated plaques that appeared 2 weeks earlier.

On examination, these plaques were located mainly on the extensor surface of both forearms and the right thigh. In the forearms, the plaques were 5–6 with a size that ranged from 3 to 4 cm in diameter (Figure 1). There were two to four plaques on the right thigh that measured 2–3 cm in diameter. In addition, there was erythema and edema of the right-hands’ middle and index fingers, which were slightly tender and flexed (Figure 2).

A bacterial swab was taken from the plaques where there was crusting and oozing and a topical Fucidin antibiotic and Augmentin 625 mg tablet Q8 hours were prescribed for 1 week, waiting for improvement. The patient came back a week later with no improvement. Therefore, a skin biopsy was done and the histopathological examination revealed superficial and deep perivascular and periadnexal infiltrate of lymphocytes. There was marked interface vacuolar degeneration at the dermo-epidermal junction with necrotic keratinocytes and underlying dermal melanophages. Overlying hyperkeratosis was evident and a hint of dermal mucin was identified. The ulcerated area showed ulceration with necroinflammatory exudate. A full autoimmune screening was carried out and it showed that ANA, C-ANCA, and P-ANCA were all negative; complement 3 level was 1.37 g/l and complement 4 was 0.52 g/l, both being in the normal range; C-reactive protein was high (it was 41.51 mg/l while the reference range is 0–3 mg/l).

Figure 1. Cutaneous ulcerated plaques on the extensor surface of left (A) and right (B) arms and elbows.

Figure 2. Right hand (A) shows erythema and swelling mainly of the index and middle fingers; left hand (B) shows mild erythema and swelling of the thumb and index fingers.

The patient was referred to the rheumatology clinic for her tender swollen joints and lethargy, as well as for further systemic evaluation. The rheumatology agreed on our management and starting her on prednisolone 0.5 mg/kg with a weekly tapering dose of 5 mg and hydroxychloroquine 200 mg twice a day with gradual modest improvement. Ulcerated plaques were healed and the itching and pain were decreased with mild erythema and post-inflammatory hyperpigmentation.


Discussion

We report here a case of atypical presentation of DLE associated with fever and arthralgia. The differential diagnosis of discoid skin rash includes a variety of conditions, such as DLE, cutaneous sarcoidosis, erythema multiforme, generalized granuloma annulare, and Sweet’s syndrome, and many other conditions [1].

DLE may occur in association with SLE or usually occur in the absence of systemic disease. Incidence of CLE is 4.2 cases/100,000 and the DLE accounts for 50%–75% of CLE. Cutaneous lupus is subdivided into three categories: acute CLE (ACLE), subacute CLE (SCLE), and chronic CLE (CCLE) [5].

Almost all patients with ACLE develop SLE during the course of their disease [6]. Patients are in the third decade of life [7] and have signs and symptoms of SLE, as well as positive serologic findings of SLE [8]. The disease may present as localized form such as the classic malar or butterfly rash seen in SLE. A generalized form presents as photosensitive lupus dermatitis or a maculopapular lupus rash [9].

The SCLE is the more photosensitive subset of CLE. It usually presents in the third or fourth decade of life [7], with more tendency to affect women than men [10]. It presents as a photodistributed, nonscarring papulosquamous or annular, polycyclic eruption that can be isolated or can involve mild extracutaneous manifestations [11]. Lesions are photoprovoked and can persist for long periods of time. Lesions tend to heal without scar formation [12].

The CCLE is thought to be two to three times more frequent than SLE, with the common age of onset being 20–40 years of age [13]. CLE is considered to have a less severe course and a better prognosis than SLE; it comprises many subtypes, such as DLE; hypertrophic DLE; lupus panniculitis; mucosal DLE; lupus tumidus; and chilblains LE. The patient in our study did not have a history suggestive of autoimmune disease. Histopathological examination showed characteristics of interface vascular dermatitis consistent with DLE.

The patient displayed ulcerated inflamed plaques of large size around 5–6 cm long and different shapes with sharp margin associated with systemic manifestations, i.e., fever, arthralgia located on both arms and the right thigh, which was atypical for classic DLE.

As regards to systemic manifestations that may be seen in patients with CLE, arthralgia was reported more commonly in varieties of CLE [14,15] but systemic involvement is minimal in cases of DLE [3].

For patients with widespread lesions and risk of (or actual) systemic involvement, antimalarials, such as hydroxychloroquine or chloroquine, are the first-line systemic treatment, irrespective of the subtype of the disease. Systemic corticosteroids can be used additionally in patients with highly acute and severe skin lesions but should be used for a limited time due to the side-effects. Further second-line treatment options include methotrexate, retinoids, and dapsone; mycophenolate mofetil and mycophenolate acid are third-line treatment options. Biological agents, such as belimumab or sirukumab, are promising new therapeutic options but their efficacy and safety in the treatment of patients with CLE still need to be evaluated in clinical trials [16]. Our patient received a treatment that was comprised of prednisolone (systemic steroid) and hydroxychloroquine (antimalarial drug).

Patients with CLE should be followed up. For those with unstable, active disease, follow-up should be assigned at biweekly to monthly intervals. For patients with stable disease, follow-up can be assigned every 3–6 months.

The manifestations of the patient pointed at first encounter to CLE. However, the complications that followed later may indicate a transition to SLE or the condition may have been associate with SLE from the start and the patient was among the minority that had falsely negative serology work-up.


Conclusion

DLE can present in many unusual, atypical forms. CLE may progress into SLE, so follow-up is recommended at regular intervals of 6 months for patients that are diagnosed to have one type of CLE.


Conflict of interest

The authors declare that there is no conflict of interest regarding the publication of this article.


Funding

None.


Consent for publication

Informed consent was obtained from the patient to publish this case in a medical journal.


Ethical approval

Ethical approval is not required at our institution to publish an anonymous case report.


Author details

Sara Gari1, Ghaidaa Sindi2, Rahaf Alturkistani2, Mayar Almehmadi2

  1. Dermatology Consultant, Department of Dermatology, King Abdul-Aziz University Hospital, Jeddah, Saudi Arabia
  2. House Officers, Department of Dermatology, King Abdul-Aziz University Hospital, Jeddah, Saudi Arabia

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How to Cite this Article
Pubmed Style

Gari SA, Sindi G, Alturkistani RF, Almehmadi M. An atypical presentation of discoid lupus erythematosus: a case report. IJMDC. 2019; 3(7): 628-631. doi:10.24911/IJMDC.51-1547586961


Web Style

Gari SA, Sindi G, Alturkistani RF, Almehmadi M. An atypical presentation of discoid lupus erythematosus: a case report. http://www.ijmdc.com/?mno=26391 [Access: August 20, 2019]. doi:10.24911/IJMDC.51-1547586961


AMA (American Medical Association) Style

Gari SA, Sindi G, Alturkistani RF, Almehmadi M. An atypical presentation of discoid lupus erythematosus: a case report. IJMDC. 2019; 3(7): 628-631. doi:10.24911/IJMDC.51-1547586961



Vancouver/ICMJE Style

Gari SA, Sindi G, Alturkistani RF, Almehmadi M. An atypical presentation of discoid lupus erythematosus: a case report. IJMDC. (2019), [cited August 20, 2019]; 3(7): 628-631. doi:10.24911/IJMDC.51-1547586961



Harvard Style

Gari, S. A., Sindi, . G., Alturkistani, . R. F. & Almehmadi, . M. (2019) An atypical presentation of discoid lupus erythematosus: a case report. IJMDC, 3 (7), 628-631. doi:10.24911/IJMDC.51-1547586961



Turabian Style

Gari, Sara Abdulhadi, Ghaidaa Sindi, Rahaf Fadil Alturkistani, and Mayar Almehmadi. 2019. An atypical presentation of discoid lupus erythematosus: a case report. International Journal of Medicine in Developing Countries, 3 (7), 628-631. doi:10.24911/IJMDC.51-1547586961



Chicago Style

Gari, Sara Abdulhadi, Ghaidaa Sindi, Rahaf Fadil Alturkistani, and Mayar Almehmadi. "An atypical presentation of discoid lupus erythematosus: a case report." International Journal of Medicine in Developing Countries 3 (2019), 628-631. doi:10.24911/IJMDC.51-1547586961



MLA (The Modern Language Association) Style

Gari, Sara Abdulhadi, Ghaidaa Sindi, Rahaf Fadil Alturkistani, and Mayar Almehmadi. "An atypical presentation of discoid lupus erythematosus: a case report." International Journal of Medicine in Developing Countries 3.7 (2019), 628-631. Print. doi:10.24911/IJMDC.51-1547586961



APA (American Psychological Association) Style

Gari, S. A., Sindi, . G., Alturkistani, . R. F. & Almehmadi, . M. (2019) An atypical presentation of discoid lupus erythematosus: a case report. International Journal of Medicine in Developing Countries, 3 (7), 628-631. doi:10.24911/IJMDC.51-1547586961