Nigerian Journal of Paediatrics

Neonatal lupus erythematosus in a Nigerian infant

Niger J Paediatr 2017; 44 (2):84 – 86

CASE REPORT

Ezeudu CE

Ezejiofor OI

Neonatal lupus erythematosus

Igwe WC

in a Nigerian infant

Ezechukwu CC

Nwankwo HM

Ezejiofor IF

DOI:http://dx.doi.org/10.4314/njp.v44i2.8

Accepted: 15th June 2017

Abstract : Systemic lupus erythe-

of this very rare condition in

matosus is a chronic inflammatory

infants. A 6-week-old female in-

Ezeudu CE (

)

autoimmune disease that affects

fant presented at the paediatric unit

Igwe WC, Ezechukwu CC

all organ systems and follows a

of our facility with a history of a

Department of Paediatrics,

relapsing and remitting course

pre-auricular skin eruptions of five

with presentation ranging from

weeks and poor suckling of three

Ezejiofor OI

indolent to fulminant. It is an un-

weeks duration. The skin lesions

Department of Medicine

common condition in children and

persisted unchanged after 14 days

Nnamdi Azikiwe University

usually manifests as the cutaneous

of antibiotics. A skin snip was

Awka, Anambra state, Nigeria

form in the paediatric age group.

taken for histology and a diagnosis

Email: ezeuducj@yahoo.com

We report a case of neonatal lupus

of neonatal lupus erythematosus

erythematosus in a six-week-old

was made.

Nwankwo HM

Nigerian infant seen at NAUTH,

Department of Medicine

Nnewi. The objective of this re-

Key words: neonatal lupsus

port is to highlight the existence

erythematosus, infant, Nnewi.

Ezejiofor IF

Department of Histopathology,

Nnamdi Azikiwe University

Teaching Hospital, Nnewi,

Anambra state, Nigeria

Introduction

face, neck, and scalp.⁴ These lesions appear typically as

erythematous plaques that may persists as alopecia over

Systemic lupus erythematosus is a chronic inflammatory

a period of time. We report a case of neonatal lupus ery-

autoimmune disease that affects all organ systems and

thematosis in a six- week-old female infant seen at the

follows a relapsing and remitting course with presenta-

paediatric unit of our facility.

tion ranging from indolent to fulminant. It is an uncom-

mon condition in children and usually manifests as the

cutaneous form in paediatric age group. The exact

Case Report

pathogenesis of neonatal lupus erythematosus (NLE) is

not well understood, but it has been attributed to a lot of

OM is a six-week-old female infant who presented at the

factors, mainly genetic and environmental. Some drug

paediatric unit of Nnamdi Azikiwe University Teaching

induced cases have been reported. Immunoglobulin G

2.3

Hospital, Nnewi with a rash of five weeks and poor

(IgG) against R o (SSA), La(SSB) and/or U1- ribonucleo-

suckling of three weeks duration. She was well until the

protein produced by the mother are passively transferred

second week of life when she developed skin eruptions

transplacentally, predisposing the infant to the develop-

around the left ear but later spread to involve the left

ment of neonatal lupus erythematosus. Inspite of this,

parietal area. The lesions started as small vesicles con-

only about 2% of babies with positive maternal transpla-

taining clear fluid, ruptured spontaneously, and then

cental antibodies develop neonatal lupus erythematosus.

ulcerated. (Figure 1).Similar lesions also developed on

the occipital region of the scalp. Two weeks into the

The major clinical manifestations of neonatal lupus ery-

illness she was noticed to be suckling poorly and to be

thematosus are dermatologic, cardiac and hepatic. Some

losing weight. She was given some drugs purchased

infants may also manifestcentral nervous system, and

from a patent medicine dealer and also received treat-

haematologic abnormalities. Conduction cardiac abnor-

ment from a private hospital with no significant im-

malities, hepatomagaly, seizures, anaemia and thrombo-

provement. She was then brought to our facility for fur-

cytopenia can all occur. Cutaneous manifestations of

ther management.

NLE occur in about 70% of patients with neonatal lupus

She was the first of a set for twins delivered at term to a

erythematosus and lesions are seen predominantly in the

thirty-one-year old primiparous woman. Her pregnancy

and delivery were normal and there was no significant

Fig 2: Photomicrograph of Neonatal lupus erythematosus

illness in the early neonatal period. She weighed 3kg at

showing hyperkeratosis (white arrow) and moderate mononu-

birth.

clear inflammatory infiltrate in the upper dermis associated

Physical examination revealed a weak infant with a nor-

with dermal oedema (an arrow). There is also focal basal cell

vacuolization and melanin pigment incontinence (double ar-

mal body temperature, mildly pale, anicteric with no

rows).

sign of dehydration. She weighed 2.5kg with a head

circumference of36.5cm. She was semiconscious and

globally hypertonic. There were erythematous, ulcera-

tive lesions around the left ear extending to the parietal

area with a significant hair loss on the affected areas.

The occipital area also has similar lesion but with less

ulceration. Abdomen was full and soft and moved with

respiration. Liver was palpable 2cm below the right

coastal margin in the mid-clavicular line. Other findings

on systemic examinations were essentially normal.

Complete blood count showed marked neutrophil leuko-

cytocysis with toxic granulations. Erythrocyte sedimen-

tation rate was 85mm/hr.

Diagnosis of failure to thrive due to poor nutritional

intake and sepsis were made. She was placed on stan-

Discussion

dard treatment for sepsis and nutritional rehabilitation.

Two weeks into admission the lesionspersisted with

Neonatal Lupus erythromatosus is usually seen in in-

some scarring leaving some areas of hypopigmentation/

fants born to mothers with auto- antibodies against R o

hyperpigmentation and alopecia. The dermatologist was

(SSA), La(SSB) and/or U1- ribonucleoprotein. These

invited after alopecia had persisted for several weeks.

auto- antibodies gain access to the fetal circulation

The dermatological review found multiple erythema-

through the placenta. The circulating auto-antibodies

tous, hypopigmented/hyperpigmented atrophic plaques

produce signs and symptoms in the infant that are simi-

on the left ear, left parietal and occipital areas, and

lar to adult Lupus erythematosus. The mothers of these

raised a suspicion of lupus erythematosis.

infants with NLE are usually diagnosed of systemic lu-

Biopsy and histology of the lesion showed hyperkera-

pus erythematosus (SLE) or other autoimmune condi-

totosis with follicular plugging. There was dermoepider-

tions such as Rheumathoid arthritis or Sjogren’s syn-

mal mononuclear inflammatory cell infiltration extend-

drome. The commonest abnormalities seen in the new-

ing to the adnexal structures and subcutaneous tissues.

born babies with NLE are dermatological, cardiac, and

There was basal layer vacuolation with melanin inconti-

occasionally other organs such as the liver may be in-

nence and few colloidal bodies. (Fig 2). The overall

volved. The disease is rare, affecting only approximately

picture was consistent with a diagnosis of neonatal

1 in 20,000 live born infants.

lupus erythematosus.

The patient showed marked improvement and was dis-

Such dermatological abnormalities include the cutane-

charged home after three months of conservative man-

ous manifestations of lupus erythematosus seen in adults

agement. She was however, subsequently lost to follow-

such as; Chronic Discoid Lupus, Subacute Lupus, and

up.

Acute Lupus rashes. Moretti et al reported a two month

old infant with such dermatological lesions on the left

Fig 1: Ulceration involving the left pinna and the parietal scalp

with loss of hair, marked erythema, patches of hypopigmenta-

cheek, scalp, retroauricular area and abdominal skin.

tion and hyperpigmentation

These rashes were present in the index patient in the

head and neck region which are usually common sites

because of their photosensitive nature. The formation of

multiple atrophic scars and dyspigmentation on the scalp

with scarring alopecia was very much in keeping with

the clinical appearance of chronic discoid lupus erythe-

matosus. These characteristic skin lesions with other

clinical features such as poor suckling, failure to gain

weight and generalized hypertonia strengthened the

clinical suspicion of Neonatal lupus erythematosus.

The elevated Erythrocyte sedimentation rate (ESR) of

85mm/hr and skin biopsy histology findings consistent

with chronic discoid lupus erythematosus were all sup-

portive of the diagnosis of NLE. The absence of clinical

features suggestive of any auto-immune condition in the

mother does not exclude the diagnosis. In all such

cases, it will be very informative to screen the mother

for evidence of circulating auto-antibodies. This was

Conclusion

however not possible in the index case because of finan-

cial constraints and unavailability of the auto-antibodies

Lupus erythematosus is rare in paediatric age group but

screening tests in our centre.

should be suspected in infants with persistent skin

The exact pathogenesis of the condition is not very clear

lesions and alopecia around the head and neck region.

but the transplacental passage of auto-antibodies such as

anti-Rho (SSA) antibody which is present in 95% of

cases of Neonatal lupus is fundamental. The signifi-

8,9

Conflict of interest: None

cant improvement made before discharge on conserva-

Funding: None

tive management is likely because of the disappearance

of the circulating antibodies within several months of

management.

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