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  Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 7  |  Issue : 1  |  Page : 20-23  

Cutaneous lupus erythematosus of elbows: A distinct entity?


1 Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Web Publication18-Jan-2016

Correspondence Address:
Dr. Laxmisha Chandrashekar
Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.174306

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   Abstract 

The elbow is not recognized as common site for cutaneous lupus erythematosus (CLE) lesions. Twelve cases of CLE over the elbows were evaluated for systemic involvement and Cutaneous Lupus Disease Area and Severity Index activity and damage scores and Systemic Lupus Erythematosus Disease Activity Index scoring was done. Histopathological examination of the affected skin was performed in doubtful cases. Most of the patients were women (10, 83.3%) with mean age of 28.75 years. Three patients had only elbow lesions and the remaining nine patients had CLE lesions at sites other than the elbows, of which five had elbow lesions preceding skin lesions elsewhere over the body and three patients were not aware of whether elbow lesions preceded or succeeded CLE lesions at other sites, and one patient had noticed malar rash 9 months prior to elbow lesions. All the patients antinuclear antibody positivity, systemic involvement, and fulfilled criteria for systemic lupus erythematosus. This peculiar localization of CLE to the elbows may be associated with a greater risk of systemic involvement and may be an predictor of flare of LE.

Keywords: Cutaneous lupus erythematosus, elbow, systemic lupus erythematosus


How to cite this article:
Singh N, Chandrashekar L, Kumar N, Kar R, Sylvia MT, Thappa DM. Cutaneous lupus erythematosus of elbows: A distinct entity?. Indian Dermatol Online J 2016;7:20-3

How to cite this URL:
Singh N, Chandrashekar L, Kumar N, Kar R, Sylvia MT, Thappa DM. Cutaneous lupus erythematosus of elbows: A distinct entity?. Indian Dermatol Online J [serial online] 2016 [cited 2018 May 22];7:20-3. Available from: http://www.idoj.in/text.asp?2016/7/1/20/174306


   Introduction Top


Cutaneous lupus erythematosus (CLE) usually presents on photoexposed areas such as face, neck, back, extensor aspects of upper limb, and dorsa of hands. The elbow is not recognized as a common site for CLE except for a few reports.[1],[2] We describe a series of 12 patients with CLE over the elbow who had systemic involvement.


   Case Reports Top


All cases attending dermatology clinic from September 2012 to May 2013 for clinical features suggestive of lupus erythematosus were examined for CLE lesions over the elbows [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f,[Figure 1]g,[Figure 1]h,[Figure 1]i,[Figure 1]j,[Figure 1]k,[Figure 1]l. All cases of CLE had lesions on the elbows were evaluated for systemic involvement and severity of CLE lesions was assessed by Cutaneous Lupus Disease Area and Severity Index (CLASI) activity and damage scores [3]; and systemic involvement was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scoring.[4] Histopathological examination of the affected skin was performed in doubtful cases.
Figure 1: (a-l) Cutaneous lupus erythematosus lesions over bilateral elbows except (b) case with unilateral cutaneous lupus erythematosus lesions (right elbow)

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Most of the patients were women (10, 83.3%) with mean age of 28.75 years (range: 15–50 years old). Out of twelve patients, three patients had only elbow lesions and among them, one patient (Case 1) had not noticed any skin lesion and had presented for premature graying of hair and on examination was found to have CLE lesions over the elbows [Figure 2]a. The remaining nine patients had CLE lesions at sites other than the elbow, of which five had elbow lesions preceding skin lesions elsewhere in body and three patients were not aware of whether elbow lesions preceded or succeeded CLE lesions at other sites, and one patient had noticed malar rash 9 months prior to the elbow lesions. The five patients who had elbow lesions preceding other skin lesions, the lesions preceded by 4 days, 1 week, 3 weeks, 4 weeks, and 5 months. All patients except one had itching associated with the skin lesions over the elbow. They presented with erythematous papules and plaques in all cases. Scaling was seen in 11 cases and atrophy in eight cases at the time of our assessment. Seven cases had associated oral mucosal erosions, seven patients had diffuse alopecia and thinning and coarseness of hair, and one patient had premature graying of hair. All the patients were antinuclear antibody positive, had systemic involvement [Table 1] and fulfilled criteria for systemic lupus erythematosus (SLE).
Figure 2:(a) Erythematous papules and plaques with mild scaly surface and minimal atrophy seen over bilateral elbows. (b) Epidermis is thinned out with prominent basal layer vacuolization (H and E; ×100). (c) Prominent periadnexal mucin deposition and collagen homogenization (H and E; ×100). (d) Leukocytoclasis with inset showing fibrin deposition with leukocytoclasis in the vessel wall (H and E; ×200)

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Table 1: Systemic manifestations of cases with CLE over elbows

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Four cases were found to have autoimmune hemolytic anemia (AIHA), of which two were previously diagnosed and were on treatment and had no hematological abnormality at the time of our assessment. Five cases had lupus nephritis. The mean (SD) CLASI activity and damage scores were 8.25 (7.29) and 5.83 (4.32), respectively. The mean CLASI activity scores were of moderate severity, whereas the mean CLASI damage scores were of mild severity. The mean SLEDAI score was 14 (6.82) at the time of patient's examination.

Skin biopsy (elbow) was taken for histopathological examination in seven out of 12 patients. Histopathological findings [Figure 2]b,[Figure 2]c,[Figure 2]d are summarized in [Table 2]. Mild to moderate perivascular and periadnexal infiltrate was observed in almost all cases. Periadnexal mucin deposition [Figure 2]c was seen in all cases, whereas in two of the seven cases, mucin was also present interstitially. Collagen homogenization was seen in all cases and so was scanty interstitial histiocytic infiltrate. Leukocytoclasis [Figure 2]d was seen in four out of seven cases. Epidermal changes were seen in all but one case.{Figure 2}
Table 2: Histopathological changes in CLE of elbows

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   Discussion Top


Cutaneous lupus erythematous (CLE) lesions over the elbow are unique and may mimic polymorphic light eruption due to its waxing and waning nature. Usually, CLE of elbow is considered to be rare as we came across only two such instances where CLE over elbow has been described in the literature.[1],[2] The first description was of a lady with recurrent eruption of erythematous papules and plaques over the elbow in the summer season for 10 years and the clinical and histopathological findings were compatible with lupus erythematosus tumidus (LET). The other description of CLE of the elbow consisted of seven patients with pruriginous erythematous papules and plaques with mild scaling present symmetrically over the elbows, all of whom had a previous or concomitant diagnosis of LE. All but one of our cases had CLE lesions over both elbows as was reported by Bielsa et al.[2] These CLE lesions over the elbows were not polymorphic light eruption (PMLE) even though a similar peculiar localization has been reported recently as PMLE.[5] Close monitoring and follow up is required in cases of CLE as there is a high probability of an additional diagnosis of SLE.[6] The presence of autoimmune hemolytic anemia in four of our cases and lupus nephritis in another five cases may suggest a severe systemic involvement in patients with CLE over the elbows. It is not clear if CLE lesions over the elbows are an indicator of flare of LE since in five cases elbow lesions preceded ACLE rash. We did not compare our data with cases of CLE without elbow lesions and the frequency of systemic involvement, which is a major limitation of our case series apart from the small number of patients.


   Conclusion Top


We believe that this peculiar localization of CLE to the elbow is not restricted to the subset of LET; it may be associated with a greater risk of systemic involvement and may also be an predictor of flare of LE.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Di Cesare A, Fargnoli MC, Lozzi GP, Peris K. Symmetric distribution of lupus erythematosus tumidus on the elbows. Eur J Dermatol 2006;16:450-1.  Back to cited text no. 1
    
2.
Bielsa I, Guinovart RM, Fernández-Figueras MT, Rodríguez C, Ferrándiz C. Cutaneous lupus erythematosus on the elbows: A peculiar localization. Lupus 2012;21:84-8.  Back to cited text no. 2
    
3.
Albrecht J, Taylor L, Berlin JA, Dulay S, Ang G, Fakharzadeh S, et al. The CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index): An outcome instrument for cutaneous lupus erythematosus. J Invest Dermatol 2005;125:889-94.  Back to cited text no. 3
    
4.
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 1992;35:630-40.  Back to cited text no. 4
    
5.
Molina-Ruiz AM, Sanmartín O, Santonja C, Kutzner H, Requena L. Spring and summer eruption of the elbows: A peculiar localized variant of polymorphous light eruption. J Am Acad Dermatol 2013;68:306-12.  Back to cited text no. 5
    
6.
Grönhagen CM, Fored CM, Granath F, Nyberg F. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: A population-based cohort of 1088 patients in Sweden. Br J Dermatol 2011;164:1335-41.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

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