Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
Users online: 3526  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page

Table of Contents 
Year : 2017  |  Volume : 62  |  Issue : 4  |  Page : 440
Severely crusted cheilitis as an initial presentation of systemic lupus erythematosus

1 National Skin Centre, Department of Dermatology, Singapore
2 Singapore General Hospital, Singapore

Date of Web Publication10-Jul-2017

Correspondence Address:
Wai Man Mandy Chan
National Skin Centre, 1 Mandalay Road, 308205
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_559_16

Rights and Permissions


Lupus erythematosus (LE) is an autoimmune disease which may initially present solely with lip lesions. Due to a wide spectrum of presentation, these features may initially be misdiagnosed as other oral diseases such as lichen planus, erythema multiforme (EM), and actinic cheilitis, leading to a delay in diagnosis and treatment. We discuss a case of severely crusted cheilitis which was initially diagnosed as EM, with subsequent development of subacute cutaneous LE, and progression to systemic LE. We will discuss the clinical and histological features of lupus cheilitis.

Keywords: Cheilitis, crusted, systemic lupus erythematosus

How to cite this article:
Mandy Chan WM, Pang SM, Ng SK. Severely crusted cheilitis as an initial presentation of systemic lupus erythematosus. Indian J Dermatol 2017;62:440

How to cite this URL:
Mandy Chan WM, Pang SM, Ng SK. Severely crusted cheilitis as an initial presentation of systemic lupus erythematosus. Indian J Dermatol [serial online] 2017 [cited 2020 Sep 23];62:440. Available from:

What was known?

  • Oral manifestation of lupus can have a wide spectrum of clinical presentation
  • This patient presented with lower lip involvement suggesting that this was a photoaggravated or induced condition

   Introduction Top

A 54-year-old Chinese woman with a past medical history of hypertension on lisinopril for more than 5 years presented to a tertiary dermatology center with a 2-month history of abrupt onset, painless severe crusting of the lower lip [Figure 1]a. Apart from her antihypertensive, she was not on any other medications or supplements. Examination revealed extensive hyperkeratotic crust over the lower lip, sparing the upper lip. The rest of the oral cavity and skin was normal. She had no photosensitivity, oral ulcers, joint pains, dry eyes, and absence of family history of autoimmune disease. Based on clinical examination, an initial diagnosis of erythema multiforme (EM) was entertained with differentials including actinic cheilitis, lichen planus (LP), lupus cheilitis, and paraneoplastic pemphigus. She was referred to a tertiary dental center for a biopsy for which she declined and was subsequently given prednisolone 30 mg daily for 2 weeks for the treatment of presumed EM and topical clobetasol ointment.
Figure 1: (a) Severely crusted cheilitis of the lower lip; (b) near-complete resolution after treatment with oral prednisolone 30 mg daily for 2 weeks

Click here to view

On review 2 weeks later, the patient's severe crusted cheilitis improved [Figure 1]b and [Figure 2]. We encouraged a biopsy, and further laboratory investigations revealed a normal full blood count and high antinuclear antibodies (ANAs) of >1/800, nucleolar and speckled pattern. However, the patient subsequently defaulted further follow-up.

Six months later, the patient presented with widespread photodistributed erythematous scaly patches and plaques over the face, chest, and extensors of the forearms [Figure 3]a and [Figure 3]b. A skin biopsy from the left forearm revealed interface vacuolar dermatitis, lymphocytic inflammation, lower epidermal apoptosis, hyperkeratosis, and hypergranulosis. There was no follicular plugging or vasculitis. Periodic acid–Schiff (PAS) stain demonstrated basement membrane thickening [Figure 4]a. Direct immunofluorescence (DIF) showed IgM and C3 immunodeposits along the basement membrane zone [Figure 4]b.
Figure 3: (a and b) Photodistributed erythematous scaly patches and plaques over the face and neck, chest, and extensors of forearms

Click here to view
Figure 4: (a) Biopsy from the left forearm showing interface dermatitis and thickening of basement membrane (PAS, ×10); (b) Direct immunofluorescence showing granular C3 along basement membrane zone

Click here to view

Based on clinicopathological correlation, a diagnosis of subacute cutaneous lupus erythematosus (SCLE) was made, and the patient was started on hydroxychloroquine 300 mg daily, and subsequently increased to 400 mg daily, topical corticosteroids, and photoprotection. She was reviewed by a rheumatologist, and investigations did not fulfill the American College of Rheumatology (ACR) criteria for a diagnosis of systemic lupus erythematosus (SLE). Two months later, her rashes progressed despite treatment. Further investigations revealed urine proteinuria (urine PCR, 0.28 mg/mmol), autoimmune hemolytic anemia (elevated lactate dehydrogenase, 529 U/L; low haptoglobin, 0.35 G/L; positive direct anti-human globulin test 1+), and positive autoantibodies to extractable nuclear antigens, including ribonucleoprotein, and Ro (SSA) antibody were detected. Rheumatological evaluation fulfilled the ACR criteria for SLE. Prednisolone 40 mg daily was added with control of disease activity.

   Discussion Top

The frequency of which SLE presents with oral lesions ranges from 9% to 45%.[1] Mucocutaneous manifestations of lupus erythematosus (LE) can present in a variety of ways, which can cause difficulties in early diagnosis as demonstrated in our case.[2]

Lupus cheilitis has various clinical presentations ranging from atrophic plaques to white/keratotic, purpuric, bullous, and verrucous lesions. Concomitant oral and cutaneous discoid lesions with high titers of circulating ANA may be at particular risk of developing systemic disease and warrant careful monitoring.[3] The most common areas for lesions are buccal mucosa followed by hard palate and lower lip.[4] Histopathological features of oral LE lesions are mainly lichenoid mucositis, with perivascular inflammatory infiltrate, atrophy of rete pegs, liquefactive degeneration of basal epithelial cells, and patchy PAS-positive subepithelial deposits.[2] Histopathological diagnosis of LE should be confirmed with DIF to rule out other lesions such as LP; DIF in oral LE lesions is frequently positive, and the most common immunoreactants identified are IgM and C3.[5] There are no large or controlled studies in literature on the management of oral LE. General measures include smoking cessation and topical and oral corticosteroids. Antimalarials, azathioprine,[6] methotrexate,[7] dapsone,[8] and gold [9] have been described in isolated and small case series. Our patient's severe cheilitis responded well to oral prednisolone with no recurrence of lesions. She was subsequently treated with oral hydroxychloroquine and prednisolone for progression from SCLE to LE with good response.

Oral manifestations of EM can range from tender superficial erythematous and hyperkeratotic plaques to painful hemorrhagic bullae and erosions.[10] Most patients have chronic or recurrent oral lesions, which can occur with typical and atypical target lesions. The exact pathogenesis is unknown but has been suggested to be an immunologically mediated reaction to infectious agent (such as herpes simplex virus) or drugs. Our patient's severe crusting was painless, with no bullae or erosions. Her episodes were not recurrent and targetoid lesions were absent.

This case demonstrates an unusual presentation of severe crusting cheilitis as a presenting feature of SLE. Our patient presented with lower lip involvement only, suggesting that this was a photoaggravated or induced condition. High ANA titers should have prompted a high suspicion for lupus cheilitis instead of EM. Close follow-up is needed, and repeat biopsies and laboratory investigations should be done as patients can progress to SLE.


The authors would like to acknowledge and thank Dr. Chan Mei Fung Michelle and Dr. Al Jajeh Issam for their histopathological input.

   Declaration of Patient Consent Top

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


Conflicts of interest

There are no conflicts of interest.

   References Top

Burge SM, Frith PA, Juniper RP, Wojnarowska F. Mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Br J Dermatol 1989;121:727-41.  Back to cited text no. 1
Nico MM, Bologna SB, Lourenço SV. The lip in lupus erythematosus. Clin Exp Dermatol 2014;39:563-9.  Back to cited text no. 2
Schiødt M. Oral discoid lupus erythematosus. II. Skin lesions and systemic lupus erythematosus in sixty-six patients with 6-year follow-up. Oral Surg Oral Med Oral Pathol 1984;57:177-80.  Back to cited text no. 3
Khatibi M, Shakoorpour AH, Jahromi ZM, Ahmadzadeh A. The prevalence of oral mucosal lesions and related factors in 188 patients with systemic lupus erythematosus. Lupus 2012;21:1312-5.  Back to cited text no. 4
Lourenço SV, Nacagami Sotto M, Constantino Vilela MA, Rodrigues Gonçalves de Carvalho F, Rivitti EA, Menta Simonsen Nico M. Lupus erythematosus: Clinical and histopathological study of oral manifestations and immunohistochemical profile of epithelial maturation. J Cutan Pathol 2006;33:657-62.  Back to cited text no. 5
Orteu CH, Buchanan JA, Hutchison I, Leigh IM, Bull RH. Systemic lupus erythematosus presenting with oral mucosal lesions: Easily missed? Br J Dermatol 2001;144:1219-23.  Back to cited text no. 6
Bottomley WW, Goodfield M. Methotrexate for the treatment of severe mucocutaneous lupus erythematosus. Br J Dermatol 1995;133:311-4.  Back to cited text no. 7
Ruzicka T, Goerz G. Dapsone in the treatment of lupus erythematosus. Br J Dermatol 1981;104:53-6.  Back to cited text no. 8
Edwards MB, Gayford JJ. Oral lupus erythematosus. Three cases demonstrating three variants. Oral Surg Oral Med Oral Pathol 1971;31:332-42.  Back to cited text no. 9
Kohli PS, Kaur J. Erythema multiforme-oral variant: Case report and review of literature. Indian J Otolaryngol Head Neck Surg 2011;63 Suppl 1:9-12.  Back to cited text no. 10

What is new?

  • This case demonstrates an unusual presentation of severely crusted cheilitis as a presenting feature of systemic lupus erythematosus
  • We point out important features to distinguish it from differentials of other causes of crusted cheilitis and stress re-evaluation of patients particularly if ANA titers are high


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


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (852 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Declaration of P...
    Article Figures

 Article Access Statistics
    PDF Downloaded58    
    Comments [Add]    

Recommend this journal