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.
