New Onset Plaques in an Exchange Student in Malaysia

Presented by Henry Foong
Ipoh, Malaysia

Abstract: 26 yo man with 2 month history of plaques face and chest

HPI:  The patient is a 26-yr-old healthy Libyan student who presented with a two month history of erythematous plaques on the face and chest.  He first noticed the reddish plaques on the chest and subsequently spread to the face.  Presently, it also involves the elbows and knees. It is asymptomatic and does not appear to be transient.  It does not seem to be aggravated by sunlight, heat, cold or physical activity.  He is otherwise well and is not on any long term medications/herbs/OTC.

O/E:  Shows few erythematous raised annular plaques 1-2 cm on the anterior chest wall, forehead, cheek, extensor surfaces of the elbows and knees. They do not blanch with pressure.  There are a few patches of alopecia with underlying erythematous skin noted on the occipital scalp. 

Clinical Images:

 

 

 

Lab: Blood counts and biochemistry were normal.  VDRL was negative. Anti-nuclear antibody serology was 1:320 titre.  

Path: Skin biopsy results: Section shows skin composed of epidermis and dermis. Hyperkeratosis and atrophied epidermis are seen. There is basal layer degeneration. Pigment laden macrophages are seen in the upper dermis. Perivascular lymphocytic infiltrates are seen in the upper and mid dermis. No granulomas are seen.

Diagnosis:  Lupus erythematosus

Plan: The immediate plan is to institute oral prednisolone 30mg daily and hydroxychloroquine 400mg daily with advise on sunblocks. However, on examination by ophthalmologist, he found maculopathy in this patient and raised the question of suitability of hydroxychloroquine in this patient.

Questions
Which type of LE would this patent fit into.  Subacute LE?
Which steroid sparing agent would you use? cellcept or imuran?

Comment by Richard Sontheimer, M.D.

The clinical and histopathologic features are highly suggestive of lupus-specific skin disease. From the photographs I cannot tell if this is subacute cutaneous LE or generalized discoid LE (induration of the lesions would be more consistent with discoid LE). Lesions in the scalp would argue more for discoid LE. However, the positive ANA would be more typical of SCLE. If possible I would check this patient's Ro/SS-A and La/SS-B autoantibody status. It is possible at times to see overlapping features of subacute SCLE and discoid LE concurrently. I would also establish a baseline for possible development of clinically significant systemic LE in the future with the following laboratory screening: Complete blood count with differential, serum chemistry screen, erythrocyte sedimentation rate and urinalysis.

Maculopathy in such a young male is quite unusual. Were there any associated visual field deficits with this maculopathy? If not, I would not exclude the possibility of an ongoing trial of oral hydroxychloroquine or chloroquine with careful ophthalmology followup. In addition, antimalarial therapy with oral quinacrine could be considered as this antimalarial drug does not add risk for retinal toxicity. Thalidomide at could also be considered in such case presuming it was available to the patient and appropriate followup for neurologic toxicity could be assured.

With respect to immunosuppressive steroid-sparing effect I would first consider methotrexate. After that I would consider CellCept as Imuran is relatively more toxic. In addition aggressive topical corticosteroid therapy would also be advised.