Acute Cutaneous and Generalized Discoid Lupus Erythematosusin

Systemic Lupus Erythematosus with Neuropsychiatric

Complications: A Multidisciplinary Approach

Fadhli Aulia Mughni

, Randy Satria Nugraha

, Eyleny Meisyah Fitri

, Tjhin Wiguna

,Windy

Keumala Budianti

Department of Dermatology and Venereology Faculty of Medicine Universitas Indonesia/Dr. CiptoMangunkusumo

National Central General Hospital, Indonesia

Department of Psychiatry Faculty of Medicine Universitas Indonesia/

Dr. CiptoMangunkusumo National Central General Hospital, Indonesia

Keywords: Acute cutaneous lupus erythematosus, discoid lupus erythematosus, multidisciplinary, neuropsychiatric

Abstract: Acute cutaneous lupus erythematosus (ACLE) is a type of cutaneous lupus erythematosus (CLE) which

presents as the characteristic malar rash and has high association with the systemic lupus erythematosus

(SLE). Discoid lupus erythematosus (DLE) is a type of chronic CLE (CCLE) typically present as discoid

atrophic scars with central hypopigmentation and is usually localized to skin lesions. SLE can present with a

wide spectrum of complications affecting multiple organs, including neuropsychiatric involvement. We

report a case of a 15-year-oldfemalepresented with the characteristic malar rash which is aggravated by sun

exposure, multiple atrophic scars on the scalp and legs, and scaring alopecia. Clinical history and complete

physical examination confirmed the diagnosis of ACLE and generalized DLE. She has been treated

routinely for SLE since two years ago with oral methylprednisolone and mycophenolate mofetil. She also

experienced psychiatric disorders, mainly depression and anxiety, and was consulted to the psychiatry clinic

for neuropsychiatric complications.We focus to describe our management plan in compiling detailed

regimen of topical sunscreen and corticosteroid usage which is tailored to the patient’s routine activity and

limitations. We also provided continuous support and established collaborative management plan with other

associated departments for evaluation of other complications. This personalized and multidisciplinary

approach proves to increaseher compliance andresulted in significant improvement of the disease and her

quality of life.

1 INTRODUCTION

Lupus erythematosus (LE) is a term used to describe

varieties of autoimmune disorders characterized by

autoimmunity towards the nucleosome and

ribonucleoprotein components. This disease ranges

from the mildest form, with only localized cutaneous

lesions, to the severe form with multiple organs

involvement and high mortality rate. Based on

clinical and histopathology,CLE-specific skin

diseaseis divided intoACLE, subacute CLE (SCLE),

and CCLE. (Costner et al., 2012)

Acute CLE is observed in 20 to 60% of LE

patients, found more often in females, and it is the

CLE most associated with SLE. (Costner et al.,

2012). Clinically ACLE is categorized into the

localized form, with erythema and edema on malar

region, and the generalized form, as large

morbilliform or exanthematous eruption focused on

the extensor aspects of arms and hands. Patients with

SCLE shows characteristic form of erythematous

macules, papules, or plaques arranged annularly or

polycyclic on sun-exposed body areas, with 50%

systemic involvement.CCLE usually manifests only

as skin lesions, most commonly DLE. (Costner et

al., 2012;Oke V et al., 2013)

It is important to evaluate the possibility of

systemic disease association in diagnosing CLE,

including neuropsychiatric manifestation. Diagnosis

can be established clinically, while laboratory and

histopathology examination may help to confirm

diagnosis and differentiate CLE types/ The

management of CLE generally include protection

320

Mughni, F., Nugraha, R., Fitri, E., Wiguna, T. and Budianti, W.

Acute Cutaneous and Generalized Discoid Lupus Erythematosusin Systemic Lupus Erythematosus with Neuropsychiatric Complications: A Multidisciplinary Approach.

DOI: 10.5220/0009987803200324

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 320-324

ISBN: 978-989-758-469-5

from ultraviolet (UV) radiation, avoidance of

photosensitizing agents, and topical therapy.

Administration of systemic immunosuppressive

agent is usually reserved for CLE in generalized

form or who have failed to benefit adequately.

(Costner et al., 2012;Hejazi et al., 2016)

Promptdiagnosis and thorough evaluation of

multiple organs involvement in ACLE and other

types of CLE greatly impact the patient’s prognosis

and quality of life. Neuropsychiatric complications

in LE patients are not commonly found and are still

poorly understood. It requires holistic management

and collaboration with other specialties according to

the systems involved. We report a case of ACLE and

generalized DLE in SLE with neuropsychiatric

complications. This case report emphasizes the need

to assess systemic complications and the importance

of multidisciplinary approach in CLE treatment.

2 CASE

A 15-year-old female was referred to the

Dermatology and Venereology clinic, Dr.

CiptoMangunkusumo National Central General

Hospital with the symptom of erythematous rash on

both cheeks. One month ago, slightly painful

erythematous patches appeared on patient’s cheeks

which are exacerbated by heat and sun exposure,

with no pruritus. Approximately in two weeks, the

lesions expanded and spread to the neck and chest

areas, with multiple small erosions observed on both

cheeks. There was no symptom of oral ulcer.

The patient has a history of SLE compatible

with SLICC (Systemic Lupus International

Collaborating Clinics) criteria since 2016. (Yu C et

al, 2014). She routinely visits the Allergy-

Immunology clinic of the Pediatric department since

diagnosed and was being treated with

methylprednisolone once daily (8 mg) and

mycophenolate mofetil twice daily (540 mg in the

morning and 360 mg in the evening) until present.

She was also given hydroxychloroquine once daily

(200 mg) but was stopped only two weeks after

retinopathy developed. Warfarin was prescribed due

to her antiphospholipid syndrome. She experienced

multiple episodes of ACLE and DLE lesions since

two years ago, and was treated with topical

corticosteroid and sunscreen. The lesions sometimes

were reduced, howeverone year later the lesions

started to spread further accompanied by progressive

hair loss on certain areas of the scalp.

Since May 2017, she started to experience

psychiatric disorders, mainly depression and anxiety,

and was consulted to the Psychiatry clinic. She was

diagnosed with neuropsychiatric systemic lupus

erythematosus (NPSLE) and was treated with

sertraline once daily (25 mg) and aripiprazole once

daily (7.5 mg), however she did not visit the clinic

routinely and did not take the medications as

instructed. She is currently on the 10

grade and

lives with her parents. From our interview with her

and her father, we found that since diagnosed with

SLE, she became less talkative and often seclude

herself from her peers. She often felt less confident

and has trouble concentrating while studying at

school. She described that she did not apply

sunscreen routinely because she was not convinced

that it helps.

Physical examination revealed the

characteristic malar or butterfly rash on her face, as

well as multiple discoid atrophic scars on both

supraorbital areas and multiple small erosions with

telangiectasia (Figure 1). Moon face appearance was

observed. There are also multiple erythematous

nummular patches on neck and chest, multiple

atrophic scars on the lower legs and scalp, with

alopecia and hair thinning on the surrounding areas

(Figure 2). Assessment of lesions using the Revised

Cutaneous Lupus Erythematosus Disease Area and

Severity Index (RCLASI) showed total activity

score and total damage score of 13 and 11,

respectively. Laboratory test showed increased anti

ds-DNA (268.6 IU/mL), increased ACA IgM (34

MPL), and decreased C3 and C4 levels (37.2 and

7.66 mg/dL, respectively).No histopathological or

direct immunofluorescence examination were

performed.

Figure 1.Classic ACLE and DLE lesions.Typical early

erythematous plaque demonstrating hyperkeratosis and

accentuation of follicle orifices and multiple sharply

demarcated, round-to-ovoid slightly indurated

erythematous plaque on both cheeks and eyebrows.

Acute Cutaneous and Generalized Discoid Lupus Erythematosusin Systemic Lupus Erythematosus with Neuropsychiatric Complications: A

Multidisciplinary Approach

321

Figure 2.(A) Multiple atrophic scars and alopecia on the

occipital areas of the scalp; (B) Multiple atrophic scars on

the anterior lower legs.

She was diagnosed with ACLE and generalized

DLE in SLE with neuropsychiatric complications.

She was treated with a strict regimen of sunscreen

application (every two to three hours on sun-exposed

areas) on sun-exposed areas, which was adjusted to

her school schedule of indoor and outdoor activities

to prevent overuse and increase compliance. Topical

corticosteroid was also added while the systemic

therapy was supervised by the Pediatry clinic. We

also advised the patient and her father about the

importance and potential benefits of routine visit to

the Psychiatry clinic. After six weeks of treatment,

the erythematous plaques on cheeks subsided, while

the DLE lesions and alopecia remain the same.

Several new erosions and excoriations emerged.

Previous wounds expanded, some covered with dark

crusts and produced yellowish pus. Gram stain

examination was performed and showed bacterial

infection. After managing the infection with wet

dressing and systemic antibiotic the skin lesions

showed marked improvement, confirmed by the

decrease of RCLASI total activity score from 13 to

9. Routine visit to the Psychiatry clinic greatly

improved the patient’s compliance and quality of

life. This shows the importance of recognizing

systemic complications and multidisciplinary

management in CLE patients.

3 DISCUSSION

The patient showed characteristic clinical

appearance of localized ACLE. However, other

possible differential diagnosis that have similar

features should be excluded, such as acne rosacea,

dermatomyositis, polymorphous light eruption

(PMLE), or photoallergic contact dermatitis.

rosacea we usually find characteristic erythematous

papules or pustules, while cutaneous lesions such as

Gottron papules, pruritus and erythematous patches

distributed on the extensor aspects and muscle

weakness are commonly found in dermatomyositis,

thus both are excluded. (Costner et al., 2012; Okon

et al., 2013). She was not diagnosed as PMLE

because there were no pinkish or erythematous

papules, and other sun-exposed area besides the

face, neck, and chest were not involved.(Lehmann et

al., 2011). Photoallergic contact dermatitis could be

considered, due to the history of regular topical

sunscreen usage since 2016 which contains photo-

allergenic substances, especially butylmethoxy-

dibenzoylmethane, octylmethoxycinnamate, and

methylbenzylidene camphor. (Goncalo et al., 2013)

However, her lesions were transient and only

appeared on certain areas, which makes this

diagnosis unlikely.

Physical examination also revealed multiple

discoid atrophic scars with peripheral

hyperpigmentation and central hypopigmentation on

scalp and lower legs, which are characteristic of

classic DLE lesions. DLE lesions on hairy areas

usually show follicular keratotic plug and may cause

scarring alopecia, also found in this patients.

(Cortner et al., 2012). She experienced alopecia

from one year ago and did not show any

improvement until recently, which support the

assessment that it is scarring alopecia caused by

DLE. (wang et al., 2011). This can highly impact the

patient’s quality of life, especially in female

teenager, highlighting the importance of specialized

evaluation and treatment in this case.

Evaluation of CLE lesions can be very

difficult, due to the chronic nature of the disease and

low response towards therapy. The extent and

severity of CLE lesions in this patient were not

evaluated thoroughly, which may contribute to

herlong-lasting lesions.In 2005, there was a tool

developed to evaluate the activity and damage of the

disease in CLE patients, termed CLASI (cutaneous

lupus erythematosus disease area and severity

index).

However in 2010 this tool is revised and

termed Revised CLASI (RCLASI).This revised tool

allow for a more complete and thorough evaluation

of all lesions as it consider different form of CLE

lesions, such as superficial or adherent scaling, and

the type of dyspigmentation. It also assesses the

presence of mucous membrane lesions and alopecia

(Kuhn et al., 2010).

Since May 2017, she was diagnosed with

neuropsychiatric SLE, which is a form of

neurological involvement in SLE. Clinical

manifestations of NPSLE could appear as

neurological symptoms, such as epileptic seizure,

headache and cerebrovascular disease, or cognitive

and psychiatric disorders. (Souirti et al., 2013).The

A

B

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prevalence of depression in SLE patients was found

to be six times higher than healthy subjects, which

was correlated with serum IL-10 concentrations,

relationship assessment, and fatigue

severity.(Figueiredo-Braga et al., 2018)

Psychiatric

disorder could also be triggered by corticosteroid

usage in 10% of patients that manifest as mood

disorder in 93% of those patients.(Bertsias et al.,

2010). In this patient, NPSLE manifested more

clearly as psychiatric disorder, which are mood

disorder, depression, and anxiety, prompting her

referral to the Psychiatry clinic. She was treated with

antidepressant (sertraline) and antipsychotic

(aripiprazole), complemented by supportive

psychotherapy for depressive symptom. This is

consistent with the European League Against

Rheumatism (EULAR) recommendation for the

management of SLE with neuropsychiatric

manifestations in 2014. .(Bertsias et al., 2010).

Treatment of CLE is still a major challenge

because there is still no specific therapy approved

for CLE. The purposes of CLE management are

educating the patient about the disease and

prevention by avoiding trigger factors.(Hejazi et al.,

2016) These can be achieved by minimizing

ultraviolet (UV) exposure, physical protection, and

application of water-resistant broad-spectrum

sunscreen that contain sun protection factor (SPF) ≥

30 dan UVA-blocking agents.

This patient has

already used SPF≥ 30 sunscreen since 2016 and was

also given vitamin D3(cholecalciferol)

supplementation 1 tablet per day. However, she

admitted that she only applied the sunscreen two to

three times per day. This frequency could not

provide adequate photoprotective effect, considering

the sunburn dosage percentage after 30 minutes of

sun exposure, and the sunscreen actual usage

concentration of 0.5–0.8 mg/cm

, to achieve

adequate photoprotection the sunscreen must be

reapplied every 1.25–2 hours for SPF 30, and every

2.5–4 hours for SPF 45. The vitamin D3 dosage

given was also not enough, as one tablet of the

patient’s supplement only contain 133 IU of

cholecalciferol. It is recommended to give vitamin

D3 supplementation of at least 400 IU per day for all

CLE patients.(Hejazi et al., 2016)

For a more comprehensive treatment of this

patient, we need to pay attention to the risk of the

development of other diseases associated with SLE

and CLE. Cerebrovascular disorders affect 2–15%

SLE patients, most commonly acute ischemic stroke.

Moreover, this patient has a history of

antiphospholipid syndrome which increase the risk

of thrombosis, another risk factor of cardiovascular

disease. (Souirti et al., 2013). The risk of

cardiovascular disorder is apparently also higher in

CLE patients compared to the general

population.(Hesselvig et al., 2017). Considering

those conditions, thorough cardiologic evaluation

should be one of the priorities in the management

plan of this patient.

4 CONCLUSION

Management of CLE and SLE is quite challenging.

Diagnosis can be made by clinical basis, however

the assessment of affected areas and disease severity

must be performed thoroughly. The RCLASI is a

reliable tool for that purpose and should be used

anytime possible to allow efficient and accurate

treatment plan. Protection from sun exposure using

topical sunscreen is still the most important aspect of

CLE management. Clinicians should allocate

adequate time to educate the patient and caretaker

regarding the correct method of sunscreen

application, as well as other methods of sun

protection. Neuropsychiatric complication is a rare

manifestation of systemic involvement in SLE

patient,which may result from both the physical

effect of autoimmunity on the nervous system and

the suffering due to pain and disability. (Figueiredo-

Braga et al., 2018)

Therefore, it is crucial to be

aware of any early signs and symptoms of systemic

involvement and consult with the corresponding

department. Multidiscipline approach might result in

less severe complications, better quality of life and

higher therapy success rate in CLE and SLE

patients.

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