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CASE REPORT
Year : 2020  |  Volume : 40  |  Issue : 2  |  Page : 118-120

Diffuse nonscarring hair loss of the scalp is an early sign of juvenile systemic lupus erythematosus: a case report


Department of Dermatology, Venereology and Andrology, Faculty of Medicine, Sohag University, Sohag, Egypt

Date of Submission24-Aug-2019
Date of Acceptance02-Jan-2020
Date of Web Publication09-Jun-2020

Correspondence Address:
Mohammed Abu El-Hamd
Department of Dermatology, Venereology and Andrology, Faculty of Medicine, Sohag University. PO Box 82524, Sohag
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejdv.ejdv_44_19

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  Abstract 


Juvenile systemic lupus erythematosus is a rare multisystemic autoimmune disorder characterized by several clinical and immunological features. In the present case, a 13-year-old female presented with diffuse nonscarring hair loss at the frontal and vertex of the scalp. The hairs were dry, fragile, and easily detached. She had patches of erythema on the palmar aspects of both hands. She had no other cutaneous, nail, or mucous membrane manifestations. Erythrocyte sedimentation rates were elevated. The titer of antinuclear antibody was positive, and anti-double strand DNA antibody was positive. Otherwise, all laboratory and radiological investigations were normal.

Keywords: anti-double strand DNA antibody, juvenile systemic lupus erythematosus, systemic lupus erythematosus


How to cite this article:
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 Venerol 2020;40:118-20

How to cite this URL:
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 Venerol [serial online] 2020 [cited 2020 Aug 12];40:118-20. Available from: http://www.ejdv.eg.net/text.asp?2020/40/2/118/286287




  Introduction Top


Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder characterized by several clinical and immunological features. Approximately 10–20% of patients with SLE have a disease onset before the age of 16 years, which is called juvenile systemic lupus erythematosus (JSLE). Incidence, severity, system affections, and prognosis of disease differ among ethnic groups [1]. JSLE is a rare disease with an incidence of 0.3–0.9/100 000 children-years and a prevalence of 1.89–25.7/100 000 children [2].

Several presentations are defined by the 11 American College of Rheumatology (ACR) classification criteria for SLE, four of which need to be fulfilled for a patient to be diagnosed as having SLE [3]. The diagnosis of JSLE is often delayed because it manifests with several nonspecific symptoms and signs. It is a serious illness causing significant morbidity, delay of normal growth, and increased mortality rate. Early diagnosis and treatment lead to better outcomes [4].


  Case presentation Top


A 13-year-old female was presented with diffuse hair falling of the scalp of 6-month duration with insidious onset and progressive course. There was no history of fever or constitutional manifestations. She had no a history suggestive of other body systems affection, with no a family history of autoimmune disorders. In addition, she had no history of received any medications. There was no a positive history of consanguinity between parents.

On admission, physical examination revealed that she was average body built with the following findings: temperature, 37.0°C; heart rate, 85 beats per minute; respiratory rate, 27 breaths per minute; blood pressure, 110/70 mmHg; and body weight, 40 kg.

She was presented with diffuse nonscarring hair loss at the frontal and vertex of the scalp. The hair was dry, fragile, and easily detached. Moreover, she had patches of erythema on the palmar aspects of both hands. She had no other cutaneous, nail, or mucous membrane manifestations.

Regarding laboratory investigations, erythrocyte sedimentation rates (ESR) were 70 mm in the first hour and 100 mm in the second hour. The titer of antinuclear antibody (ANA) (ELISA) was positive (6.01), and anti-double strand DNA antibody (anti-dsDNA) was positive (86.83).

Complete blood picture, serum ferritin, C-reactive protein, urine analysis, kidney function tests, liver function tests, and thyroid function tests showed normal values.

Regarding radiological investigations, chest radiograph and abdominal ultrasonography were normal.

Moreover, trichoscopic examination was done and revealed single hair unit, peripilar sign in frontal area, and in closer view, scattered telangiectasia without any other abnormalities ([Figure 1],[Figure 2],[Figure 3],[Figure 4]).
Figure 1 A 13-year-old female presented with diffuse hair falling of the scalp, which was an early sign of JSLE. JSLE, juvenile systemic lupus erythematosus.

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Figure 2 A 13-year-old female presented with patches of erythema on the palmar aspects of both hands.

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Figure 3 Trichoscopic examination revealed single hair unit, with telangiectasia in frontal area of the scalp.

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Figure 4 Trichoscopic examination revealed single hair unit with peripilar sign in frontal area of the scalp.

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  Discussion Top


Mucocutaneous manifestations are very common in JSLE (60–85%). When considering the ACR diagnostic criteria, cutaneous manifestations occur second to involvement of the hematological (50–100%) and musculoskeletal systems (60–90%) [3]. Wananukul et al. [5] reported that cutaneous changes in JSLE were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (19%).

Cutaneous vasculitis usually affects small blood vessels. The lesions are characterized as petechiae or palpable purpura and may occasionally have a blister. They are commonly found on the face, palms, and soles of the feet. The lesions are induced by the formation of immune complexes and neutrophilic infiltration, and the presence of vasculitic lesions strongly relates to systemic disease activity [6].

Alopecia often presents with generalized hair loss without signs of inflammation on the scalp. Diffuse, nonscarring alopecia in JSLE and adult patients with SLE usually suggests an active disease. However, it may occur three months after a severe lupus flare, for example, telogen effluvium, which is a nonspecific finding and can also occur after any significant systemic disturbance [7].

However, in this case report, the first clinical manifestations were not in agreement with the previous studies. First, chronic diffuse nonscarring hair loss presented at the frontal and vertex areas of scalp for the first time. Second, cutaneous vasculitis was presented at the palmer aspects of both hands. Finally, and most importantly, there were no other dermatological or constitutional manifestations or any signs suggestive of other system affections. Therefore, the diagnosis of this case was a challenge with respect to the age of the patient without any specific symptoms or signs of JSLE.

As manifestations in the patient were characterized by an initial presentation of gradually aggravated diffuse hair loss of 6-month duration and cutaneous vasculitis without any symptoms or signs suggesting other systems affections, so, the authors suspected many clinical differential diagnoses such as connective tissue disease, thyroid disorders, or drug reactions. As complete blood picture, reticulocytic count, ferritin level, and thyroid function test did not indicate any abnormalities, so ESR, ANA, and anti-dsDNA were performed, which led to the identification of JSLE.

Apart from the skin lesions (diffuse nonscarring alopecia and cutaneous vasculitis), no abnormalities of multiple organs were seen. In addition, urine analysis, renal and liver function tests, chest radiograph, and abdominal ultrasound were normal. Raised ESR and positive ANA and anti-dsDNA test results were detected in this patient, all of which suggested JSLE. According to ACR [3], this patient only had diffuse nonscaring alopecia and positive ANA and anti-dsDNA test results; the diagnosis was not straightforward as the manifestations did not satisfy 4 of the 11 diagnostic criteria.A new revision by the Systemic Lupus International Collaborating Clinic (SLICC) group [8] appears to be more applicable for diagnosis of JSLE in this patient, as she had nonscarring alopecia and positive ANA and anti-dsDNA test results, according to the SLICC, which are sufficient for the diagnosis of SLE, even if the renal pathology is unknown. However, the SLICC revision also recommends that a patient can be classified as having SLE if the patient has biopsy-proven lupus nephritis with ANA or anti-dsDNA. Therefore, a renal biopsy is equally important in the diagnosis of SLE, but this patient refused to perform a renal biopsy.

This patient was referred to Pediatric Rheumatology Department for further management.

This case report study highlights an unusual manifestation of JSLE in a 13-year-old female patient. Diffuse nonscarring hair loss of the scalp and patches erythema of the palmar aspects of both hands are early signs of JSLE. ANA and anti-dsDNA are essential investigations to obtain early recognition and diagnosis of JSLE.

Acknowledgements

The authors are grateful to all the faculty and postgraduates in our scientific departments for their invaluable help in conducting this study

An informed consent form was signed by the father of the patient to publish this case report.

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; 36:2539–2546.  Back to cited text no. 1
    
2.
Kamphuis S, Silverman ED. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 2010; 6:53846.  Back to cited text no. 2
    
3.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40:1725.  Back to cited text no. 3
    
4.
Watson L, Leone V, Pilkington C, Tullus K, Rangaraj S, McDonagh JE et al. Disease activity, severity, and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort. Arthritis Rheum 2012; 64:2356–2365.  Back to cited text no. 4
    
5.
Wananukul S, Watana D, Pongprasit P. Cutaneous manifestations of childhood systemic lupus erythematosus. Pediatr Dermatol 1998; 15:342–346.  Back to cited text no. 5
    
6.
Bouaziz JD, Barete S, Le Pelletier F, Amoura Z, Piette JC, Francès C. Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases. Lupus 2007; 16:163–167.  Back to cited text no. 6
    
7.
Millikan L. Hirsutism, postpartum telogen effluvium, and male pattern alopecia. J Cosmet Dermatol 2006; 5:81–86.  Back to cited text no. 7
    
8.
Petri M. SLICC revision of the ACR classification criteria for SLE. Arthritis Rheum 2009; 60:895.  Back to cited text no. 8
    


    Figures

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



 

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