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Cutaneous manifestations of lupus erythematosus from a tertiary care centre in coastal Kerala.

BACKGROUND

Lupus erythematosus (LE) is a heterogeneous disease with a wide clinical spectrum and course that can vary considerably ranging from disease limited to skin to serious manifestations that can be found in systemic LE (SLE). Skin is the second most common organ affected; second only to musculoskeletal system.

Cutaneous lesions, even though rarely life threatening may persist for many years causing alopecia, scarring, pigmentary abnormalities and disfigurement. Thus, these are potentially disabling, limiting quality of life with several patients experiencing some form of vocational handicap.

Dermatological manifestations of LE are myriad and often possess diagnostic and therapeutic challenge. Skin lesions in LE can act as diagnostic clue as well as predictor of severity of systemic involvement. Since the subsets in cutaneous LE differ considerably in relation to their clinical course and systemic involvement, identification and classification of cutaneous lesions is important prognostically also. Though there are several studies on LE, most investigators have focused on patients with SLE alone. The present study attempts to describe the broad clinical spectrum of skin disease in LE including those patients without SLE.

MATERIALS AND METHODS

This descriptive study was conducted from January 2014 to June 2015 (18 months) including all clinically diagnosed patients with acute, subacute and chronic cutaneous LE or patients satisfying 1982 Revised Criteria for Classification of Systemic Lupus Erythematosus and its update in 1997 by American College of Rheumatology (ACR) [1,2] among patients attending outpatient and inpatient departments of Dermatology, Venereology and Leprosy, at a tertiary care hospital in Alappuzha, India. Patients with LE-unrelated skin lesion alone, were excluded.

Forty patients satisfied the study criteria. Informed written consent was taken prior to detailed history, systemic and dermatological examinations. Cutaneous manifestations of LE were charted out as LE specific and LE nonspecific according to Gilliam's classification. [3] Punch biopsy of skin for histopathological and direct immunofluorescence study was done in clinically doubtful cases of cutaneous LE. Investigations including haemoglobin, total WBC count, differential count, ESR, platelet count, urine- albumin & microscopy, ANA, Anti-ds-DNA were done in all patients. Data was recorded in pre-structured proforma and analysed using SPSS 16.0 software. Qualitative data was analysed using percentages and quantitative data was analysed using mean. Permission to conduct the study was obtained from Human Ethical Committee and Institutional Research Committee.

RESULTS

Out of the 40 patients included in the study, 26 were female (Male: Female = 1:1.86). Ages of patients ranged from 13 years to 75 years (mean age = 39.8 years). LE specific lesions were seen in 38 patients (95%) and LE nonspecific lesions in 16 patients. Twenty-four patients had LE specific lesions alone (60%), 2 had LE nonspecific lesions alone (5%) and 14 patients (35%) had both LE specific and LE nonspecific lesions.

Of the 40 patients, features suggestive of systemic involvement were as follows: photosensitivity was seen in 18 patients, oral ulcers in 12 patients, arthralgia in 14 patients, neurological disorder in 2 patients, renal disorder in 4 patients, haematological disorder 16 patients, immunological abnormalities in 18 patients. Serositis in the form of cardiopulmonary disease was not observed in the study. Eighteen patients (45%) in the study satisfied the ACR criteria for diagnosis of SLE. Ratio of patients with isolated cutaneous LE to SLE was 1.22:1.

Among LE specific lesions, the most common type was Chronic Cutaneous LE (CCLE) seen in 34 patients followed by Acute Cutaneous LE (ACLE) in 8 patients and Subacute Cutaneous LE (SCLE) in 2 patients. Out of 8 patients with ACLE, 5 had malar rash alone, 1 patient had maculopapular lupus rash alone and 2 had both malar rash and maculopapular lupus rash. Only psoriasiform type of lesions were observed in SCLE.

Out of the 34 patients who had chronic cutaneous LE lesions, the most common type was classic Discoid LE (DLE) seen in 30 patients followed by mucosal DLE in 5 patients and hypertrophic LE in 3 patients. Other variants like lupus profundus, lupus tumidus, chilblain LE and lichenoid DLE were not observed in the study.

Photosensitivity was most commonly observed in ACLE patients (100%) in the study and least in CCLE patients (32.35%). Oral ulcers were also most commonly found in ACLE patients followed by SCLE (50%) and CCLE (23.52%). Arthralgia was present in all patients (100%) with SCLE and 62.5% patients with ACLE. But only 23.52% of CCLE patients had arthralgia. None of the patients had cardiopulmonary involvement. Neuropsychiatric disorder in the form of psychosis was seen in 12.5% of ACLE patients and 2.94 % of CCLE patients. Haematological disorders were present in all patients (100%) with ACLE and SCLE lesion and 29.4% of CCLE patients. Renal disorder was present mostly in ACLE patients (37.5%) followed by CCLE (8.8%). Immunological abnormalities were found in all patients (100%) with ACLE and SCLE, and 35.3% of CCLE patients.

Out of the 40 patients included in the study, 16 (40%) had LE nonspecific lesions, all of whom had features of SLE as well. Eighty nine percent of the SLE patients (n=14) had LE nonspecific lesions. The most common LE nonspecific lesion observed was non-scarring alopecia. Lupus hair (3 patients), telogen effluvium (13 patients) and alopecia areata (2 patients) were the types of non-scarring alopecia. Other LE nonspecific lesions were cutaneous vascular disease (palpable purpura-1 patient, periungual telangiectasia-3 patients), leg ulcers (2 patients) and erythema multiforme (1 patient). Patient with erythema multiforme also had SCLE, positive ANA, Anti-Sm antibody, Anti-Ro antibody and satisfied the ACR criteria for diagnosis of SLE.

Features suggestive of systemic involvement were more commonly found in patients with LE nonspecific skin lesions.

DISCUSSION

Lupus erythematosus comprises of a wide spectrum of clinical manifestations. Cutaneous manifestations of LE have been evaluated by multiple authors highlighting various clinical presentations of the disease. Malaviya [4] et al, Das [5] et al, Kole and Ghosh [6] have extensively described the cutaneous manifestations of LE in Indian patients. This study evaluated the dermatologic manifestations of LE in patients from central Kerala. The study provides a preliminary data for comparing cutaneous manifestations of LE to other populations in India and the world.

Female gender was more frequently associated with LE and clinical findings in this study, similar to the observations reported by Biazar [7] from Europe (3.3:1), Moghadam-Kia [8] et al from US (3.2:1) and Das [5] et al (4:1) from Kolkata. The mean age of patients in this study was 39.8 (range 13-75 years). Similar findings were reported by Tebbe and Orfanos [9] in their study on 97 LE patients, with commonest age of presentation between 21 and 50 years.

A significant number of patients with cutaneous disease also have systemic involvement. Forty five percent of the patients in this study had features satisfying ACR criteria for SLE. Similar findings were reported by Biazar [7] et al (40.7%) and Meuth [10] et al (44%). Among the clinical features suggestive of systemic involvement, the most common signs/symptoms in the study population were photosensitivity (45%) and immunological abnormalities (45%). The prevalence of photosensitivity varies among different populations. Foering [11] et al reported 68% prevalence of photosensitivity in US patients and Sanders [12] et al found it to be 92%.

The most common LE-specific lesion in this study was CCLE (85%). Cardinali [13] et al also had a similar observation with 72.5% patients of CCLE, 15% patients of ACLE and 8% patients of SCLE. Majority (87.5%) of ACLE patients had their lesions limited to the malar area. Cardinali [13] et al (96%) and Meuth et al [10] (100%) reported similar distribution of ACLE. Most studies [8,13,14,15] report psoriasiform lesions as the commonest type of SCLE lesion. Both the SCLE patients in this study had psoriasiform lesions. The most common type of CCLE lesion was classic DLE (88.2%) similar to Moghadam-Kia et al [8]. Systemic involvements were common in ACLE and rare in CCLE. Photosensitivity, immuno-haematologic and renal disorders were the commonest findings in ACLE patients. Compared to other reports, incidence of systemic involvement in SCLE patients was higher in the study group. [11,14,15] Similar observations in ACLE and CCLE were made by others. [7,10,16]

LE-nonspecific skin lesions were seen exclusively in patients with SLE. The most common LE nonspecific lesion was non-scarring alopecia with predominant telogen effluvium. Similar findings have been reported from India by Cole [6] and Ghosh [6] and Biazar et al [7] from Germany. The incidence of cutaneous vascular disease was lower compared to other reports. [13,17] Association of LE with EM and positive Anti Ro in one patient with SCLE could suggest a diagnosis of Rowell's syndrome in this patient. Rowell's syndrome is reported in less than 100 patients worldwide. [18] Photosensitivity, oral ulcers, arthralgia, renal, haematological and immunological disorders, and ANA positivity were significantly associated with LE nonspecific skin disease. This is consistent with the observation by Cardinali [13] et al that presence of nonspecific skin disease implies systemic disease.

CONCLUSION

Cutaneous manifestations of Lupus erythematosus are most commonly seen in adult females. CCLE is the commonest cutaneous manifestation of LE. Presence of LE nonspecific lesions can be a predictor of systemic involvement. A proper understanding of dermatological manifestations of lupus can act as a valuable aid in making diagnosis (LE specific lesions) as well as predicting prognosis (Presence of LE nonspecific lesions indicating systemic involvement).

REFERENCES

[1] Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25(11):1271-7.

[2] Hochberg MC. Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40(9):1725.

[3] Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus 1997;6(2):84-95.

[4] Malaviya AN, Singh RR, Singh YN, et al. Prevalence of systemic lupus erythematosus in India. Lupus 1993;2(2):115-8.

[5] Das NK, Dutta RN, Sengupta SR. Skin lesions in lupus erythematosus: a marker of systemic involvement. Indian J Dermatol 2011;56(5):537-40.

[6] Kole AK, Ghosh A. Cutaneous manifestations of systemic lupus erythematosus in a tertiary referral center. Indian J Dermatol 2009;54(2):132-6.

[7] Biazar C, Sigges J, Patsinakidis N, et al. Cutaneous lupus erythematosus: first multicenter database analysis of 1002 patients from the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Autoimmun Rev 2013;12(3):444-54.

[8] Moghadam-Kia S, Chilek K, Gaines E, et al. Cross-sectional analysis of a collaborative web-based database for lupus erythematosus associated skin lesions: 114 prospectively enrolled patients. Arch Dermatol 2009;145(3):255-60.

[9] Tebbe B, Orfanos CE. Epidemiology and socioeconomic impact of skin disease in lupus erythematosus. Lupus 1997;6(2):96-104.

[10] Meuth AM, Amler S, Haust M, et al. A database analysis of cutaneous lupus erythematosus with the EUSCLE core set questionnaire. Acta Derm Venereol 2010;90(4):386-94.

[11] Foering K, Goreshi R, Klein R, et al. Prevalence of self-report photosensitivity in cutaneous lupus erythematosus. J Am Acad Dermatol 2012;66(2):2208.

[12] Sanders CJ, Weelden VH, Kazzaz GA, et al. Photosensitivity in patients with lupus erythematosus: a clinical and photobiological study of 100 patients using a prolonged phototest protocol. Br J Dermatol 2003;149(1):131-7.

[13] Cardinali C, Caproni M, Bernacchi E, et al. The spectrum of cutaneous manifestations in lupus erythematosus--the Italian experience. Lupus 2000;9(6):417-23.

[14] Callen JP, Klein J. Subacute cutaneous lupus erythematosus. Clinical, serologic, immunogenetic, and therapeutic considerations. Arthritis Rheumatism 1988;31(8):1007-13.

[15] Black DR, Hornung CA, Schneider PD, et al. Frequency and severity of systemic disease in patients with subacute cutaneous lupus erythematosus. Arch Dermatol 2002;138(9):1175-8.

[16] Callen JP, Fowler JF, Kulick KB. Serologic and clinical features of patients with discoid lupus erythematosus: relationship of antibodies to single-stranded deoxyribonucleic acid and of other antinuclear antibody subsets to clinical manifestations. J Am Acad Dermatol 1985;13(5 Pt 1):748-55.

[17] Vitali C, Bencivelli W, Isenberg DA, et al. Disease activity in systemic lupus erythematosus: report of the consensus study group of the European workshop for rheumatology research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European consensus study group for disease activity in SLE. Clin Exp Rheumatol 1992;10(5):541-7.

[18] Antiga E, Caproni M, Bonciani D, et al. The last word on the so-called Rowell's syndrome? Lupus 2012;21(6):577-85.

Joice James (1), Sandhya Somasekharan Nair (2)

(1) Junior Consultant, Department of Dermatology, District Hospital, Kozhencherry, Pathanamthitta, Kerala.

(2) Assistant Professor, Department of Dermatology, Venereology and Leprosy, Government T. D. Medical College, Alappuzha, Kerala.

Financial or Other, Competing Interest: None. Th

Submission 25-04-2017, Peer Review 19-05-2017, Ju

Acceptance 25-05-2017, Published 01-06-2017. pa

Corresponding Author:

Dr. Joice James, Junior Consultant, Sy Department of Dermatology, Am District Hospital, Kozhencherry, at Pathanamthitta, Kerala.

E-mail: joicejames2@gmail.com

DOI: 10.14260/jemds/2017/740

Caption: Figure 1. Distribution of LE Specific and LE Nonspecific Skin Lesions

Caption: Figure 2. Subacute Cutaneous LE- Psoriasiform Lesions on Forearms.

Caption: Figure 4. Mucosal DLE Lesion on Hard Palate (arrow).

Caption: Figure 5. Erythema Multiforme
Table 1. Distribution of LE Specific Skin Lesions

                ACLE    SCLE   CCLE

Number of         8      2      34
patients
Percentage of   20.00   5.00   85.00
  patients

Table 2. Distribution of LE Nonspecific Skin Lesions

                Non-scarring   Cutaneous    Leg     Erythema
                  Alopecia     Vascular    Ulcers   Multiforme
                                Disease

Number of            13            4         2          1
  patients
Percentage          32.5          10         5         2.5
  of patients

Figure 3. Distribution of Variants
of CCLE in Percentage

Classic DLE        88.23
Hypertrophic DLE    8.82
Lupus profundus      0
Mucosal DLE        14.71
Lupus tumidus       0
Chilblain LE         0
Lichenoid DEL        0

Note: Table made from bar graph.
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Article Details
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Title Annotation:Original Research Article
Author:James, Joice; Nair, Sandhya Somasekharan
Publication:Journal of Evolution of Medical and Dental Sciences
Article Type:Report
Geographic Code:9INDI
Date:Jun 1, 2017
Words:2306
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