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CASE REPORT
Year : 2022  |  Volume : 23  |  Issue : 2  |  Page : 133-135

Juvenile systemic lupus erythematosus presenting only as nonscarring alopecia


1 Department of Dermatology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

Date of Submission05-Oct-2021
Date of Decision17-Nov-2021
Date of Acceptance19-Nov-2021
Date of Web Publication30-Mar-2022

Correspondence Address:
Dr. Reena Rai
Department of Dermatology, PSG Institute of Medical Sciences and Research, Coimbatore - 641 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.ijpd_141_21

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  Abstract 


Juvenile onset systemic lupus erythematosus (JSLE) is a rare multisystem, autoimmune disorder. Alopecia in SLE generally follows a pre-established disease with systemic symptoms. A 13-year-old girl presented with sudden hair loss over the vertex of scalp of 1-month duration. No other cutaneous or systemic symptoms were noted. Trichoscopy, histopathology, positive ANA, and low complement levels helped in diagnosing JSLE. Patchy nonscarring alopecia, as the sole presenting feature without systemic involvement, is rare.

Keywords: Juvenile onset systemic lupus erythematosus, nonscarring alopecia, patchy


How to cite this article:
Vinitha N M, Rai R, Maheswari G U. Juvenile systemic lupus erythematosus presenting only as nonscarring alopecia. Indian J Paediatr Dermatol 2022;23:133-5

How to cite this URL:
Vinitha N M, Rai R, Maheswari G U. Juvenile systemic lupus erythematosus presenting only as nonscarring alopecia. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 May 17];23:133-5. Available from: https://www.ijpd.in/text.asp?2022/23/2/133/341460




  Introduction Top


Juvenile onset systemic lupus erythematosus (JSLE) is an autoimmune, inflammatory, multisystem disease which occurs before 16 years of age. It has similar clinical and immunological characteristics as in adults except for the severity in disease course.[1]

Nonscarring alopecia is one of the common cutaneous manifestation of both adult and JSLE. It occurs with systemic involvement or mucocutaneous manifestations and usually does not precede the onset of the disease. Alopecia in SLE indicates an active disease and can present in different forms such as diffuse type, patchy type, or lupus hair.[2]


  Case Report Top


A 13-year-old girl presented with hair loss over vertex of scalp for 1 month. There was no history of photosensitivity. There were no changes in weight gain and appetite. No significant past history was present. Cutaneous examination revealed patchy areas of nonscarring alopecia in a butterfly pattern, measuring 7 cm × 5 cm and 10 cm × 5 cm with erythema, over the vertex region of scalp [Figure 1]. Hair over rest of the scalp was normal. Her skin, nails, and oral cavity were not involved. Systemic examination was found to be normal.
Figure 1: Patchy area of nonscarring alopecia measuring 7 cm × 5 cm and 10 cm × 5 cm, over the vertex region of scalp

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A clinical diagnosis of alopecia areata (AA) was made. Trichoscopic examination was done which showed focal atrichia, decreased hair shaft pigmentation, hair shaft thinning, and short regrowing hairs [Figure 2].
Figure 2: Focal atrichia (blue star), decreased hair shaft pigmentation (green arrow), hair shaft thinning (red arrow), and short regrowing hairs (yellow triangle) (contact polarized light dermoscopy, Dermlite DL4; 10x)

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Histopathology revealed basal vacuolar damage, focal basement membrane thickening, marked telogenisation of most hair follicles, increase in dermal mucin, perifollicular inflammation, and peribulbar fibrosis in few hair bulbs suggestive of biphasic alopecia of lupus erythematosus (LE) [Figure 3]a, [Figure 3]b, [Figure 3]c. Since histopathological findings were suggestive of SLE, routine blood investigations along with ANA and complement levels were done. ANA Immunofluorescence (IF) showed smooth nuclear membrane pattern 3+ and her profile was positive for nRNP/Sm, Sm, SS- A, Ro 52, and nucleosomes. Complement levels (C3, C4) were found to be low and TSH was elevated (13.64).
Figure 3: (a) Longitudinal section shows focal vacuolar damage with mild lymphocytic exocytosis, peri and intrafollicular lymphohistiocytic infiltrates (H and E, 4x). (b) Transverse section shows marked telogenisation, perifollicular fibrosis, peri and intrafollicular lymphohistiocytic infiltration (H and E, 4x. (c) Alcian Blue PAS stain highlighting dermal mucin

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Complete blood picture, erythrocyte sedimentation rate (ESR), renal function tests, liver function tests, urine analysis, and spot urinary protein creatinine ratio were all within normal limits.

The patient was treated with systemic steroids and mycophenolate mofetil 1000 mg. She had decreased hair fall and regrowth of hair over the affected areas after 3 months of follow-up. She did not develop any systemic symptoms thereafter.


  Discussion Top


Nonscarring alopecia is a manifestation of SLE. It can be misdiagnosed as AA, telogen effluvium, or anagen effluvium.

Alopecia is one of the cutaneous manifestations of SLE and it commonly indicate an active, well-established disease. They have different subtypes, comprising LE-specific and LE-nonspecific changes on histology. The LE-specific alopecias are usually reversible with treatment of SLE. Udompanich et al.[3] has classified different types of alopecia which can occur in SLE [Table 1].[3]
Table 1: Different types of alopecia in systemic lupus erythematosus

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Patchy nonscarring alopecia without active disease and as a sole presenting feature of JSLE is rare. Since AA also is a feature of SLE, it has to be differentiated from patchy nonscarring alopecia due to SLE and trichoscopy and histopathology can help in diagnosis.

In SLE, patients present with incomplete patchy hair loss associated with erythema and complete patchy hair loss, devoid of erythema is seen in AA. Trichoscopy of SLE demonstrates hair shaft hypopigmentation and thinning with or without vessel changes and the characteristic findings in AA such as black dots, yellow dots, and exclamation hairs were not seen in our patient.[4]

The histopathologic features were also in favor of SLE, and the diagnosis was further confirmed by positive ANA and low complement levels.

According to revised Systemic Lupus International Collaborating Clinic criteria,[5] four of the diagnostic criteria should be fulfilled including one clinical and one immunological criteria, for the diagnosis of JSLE. Our patient had nonscarring alopecia, positive ANA, anti-Sm antibody, and low complement levels, thus fulfilling the criteria.

The presentation of nonscarring alopecia in JSLE, with no mucocutaneous or systemic involvement, as seen in our case, is rare.[6] Hence, it is important to also consider the differential diagnosis of connective tissue disease in patients with nonscarring alopecia.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hiraki LT, Benseler SM, Tyrrell PN, Harvey E, Hebert D, Silverman ED. Ethnic differences in pediatric systemic lupus erythematosus. J Rheumatol 2009;36:2539-46.  Back to cited text no. 1
    
2.
Chanprapaph K, Udompanich S, Visessiri Y, Ngamjanyaporn P, Suchonwanit P. Nonscarring alopecia in systemic lupus erythematosus: A cross-sectional study with trichoscopic, histopathologic, and immunopathologic analyses. J Am Acad Dermatol 2019;81:1319-29.  Back to cited text no. 2
    
3.
Udompanich S, Chanprapaph K, Suchonwanit P. Hair and scalp changes in cutaneous and systemic lupus erythematosus. Am J Clin Dermatol 2018;19:679-94.  Back to cited text no. 3
    
4.
Concha JS, Werth VP. Alopecias in lupus erythematosus. Lupus Sci Med 2018;5:e000291.  Back to cited text no. 4
    
5.
Petri M. SLICC revision of the ACR classification criteria for SLE. Arthritis Rheum 2009;60:895.  Back to cited text no. 5
    
6.
El-Hamd MA, Aboeldahab S. Diffuse nonscarring hair loss of the scalp is an early sign of juvenile systemic lupus erythematosus: A case report. Egypt J Dermatol Venereol 2020;40:118-20.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

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