Childhood onset systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Subacute cutaneous lupus erythematosus (SCLE) is a rare subtype of juvenile onset SLE. Renal involvement is seen in 50–70% of cases of SLE in children and could be the initial presenting clinical feature in many cases. Here we present a child with lupus nephritis who later developed skin lesions suggestive of SCLE.

1. Subacute cutaneous lupus erythematosus associated with
lupus nephritis

2. Case Report
Subacute cutaneous lupus erythematosus
associated with lupus nephritis
Alkarani T. Patil a,*
, Sahana M. Srinivas b
, H.V. Shubha c
, K.S. Sanjay d
a
Associate Professor of Pediatrics and Incharge Pediatric Nephrology, Indira Gandhi Institute of Child Health,
Dharmaram College Post, Bangalore, India
b
Consultant Pediatric Dermatology, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health,
India
c
Post Graduate Student Pediatrics, Indira Gandhi Institute of Child Health, India
d
Professor of Pediatrics, Indira Gandhi Institute of Child Health, India
a r t i c l e i n f o
Article history:
Received 19 February 2015
Accepted 4 March 2015
Available online xxx
Keywords:
Subacute cutaneous lupus
Lupus nephritis
Methyl prednisolone
a b s t r a c t
Childhood onset systemic lupus erythematosus (SLE) is a multisystem autoimmune dis-
ease. Subacute cutaneous lupus erythematosus (SCLE) is a rare subtype of juvenile onset
SLE. Renal involvement is seen in 50e70% of cases of SLE in children and could be the initial
presenting clinical feature in many cases. Here we present a child with lupus nephritis who
later developed skin lesions suggestive of SCLE.
Copyright © 2015, Indraprastha Medical Corporation Ltd. All rights reserved.
1. Introduction
SLE is the most common autoimmune connective tissue dis-
order in children. The disease has varied clinical presentation
and requires a long term follow up. 15e20% of SLE patients
develop signs and symptoms during childhood.1
SCLE is a
variant of cutaneous lupus and is rarely seen in children with
few cases reported in literature. Here we report a case of 13
year old female child with lupus nephritis who developed
cutaneous lesions suggestive of SCLE in our pediatric
nephrology unit.
2. Case report
We are presenting a13 year old female child diagnosed to have
class 2 mesangioproliferative glomerulonephritis, and
immunofloresence showed mesangial deposits of IgG, IgA,
IgM, C3c, Kappa and lambda. Granular deposits were also seen
along the blood vessels, including peritubular capillaries
(Fig. 1a and b) 6 months back. The child presented with itchy
and painful skin lesions on face, trunk, neck and extremities
and painless oral ulcers from past 1 month. Lesions were
associated with photosensitivity. In view of lupus nephritis
* Corresponding author.
E-mail address: alkaranipatilurs@gmail.com (A.T. Patil).
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Please cite this article in press as: Patil AT, et al., Subacute cutaneous lupus erythematosus associated with lupus nephritis,
Apollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.03.003

3. the child was started on oral steroids but she had stopped the
treatment abruptly.
Cutaneous examination showed multiple erythematous
necrotic papules with annular scaly plaques on the periocular
region, neck, chest and back. Multiple hyperpigmented scaly
plaques were present on extensor aspect of upper and lower
leg. Bilateral periocular odema was present (Fig. 2a and b). Oral
mucosa showed multiple pin point hemorrhages on palate
and buccal mucosa. Investigations revealed anaemia, throm-
bocytopenia and increased ESR. Urine examination showed
proteinuria and hematuria. Antinuclear antibodies (ANA) and
Anti Ro/SSA were positive. Anti dsDNA was negative. Anti-
phospholipid type 3 antibody was positive. Skin biopsy from
the back lesions showed focal basal vacuolar changes with
perivascular lymphocyte infiltrate and pigmented histiocytes
at the upper dermis. Many foci of mucin deposition were seen
in the dermis (Fig. 1c). Based on the above clinical findings a
diagnosis of SCLE was considered.
The child was started on intravenous methlyprednisolone
pulse therapy and topical sunscreen and there was 80%
improvement with post inflammatory hyperpigmentation
after 5 days of treatment. Patient was continued on oral ste-
roids in tapering doses to keep her in remission.
3. Discussion
SCLE is a rare variant of connective tissue disorder charac-
terized by non scarring, non atrophic photosensitive
dermatosis. It is extremely rare in children, but more
Fig. 1 e a: Renal biopsy done for the diagnosis showed class II mesangioproliferative glomerulonephritis. b:
Immunofloresence picture showing mesangial deposits of IgG, IgA, IgM, C3c, Kappa and lambda. In addition also seen are
granular deposits of IgG, IgM and C3c are seen in the blood vessels. c: Skin biopsy showing focal basal vacuolar changes
with perivascular lymphocytic infiltrate and mucin deposition in the dermis. [H and E x10]
Fig. 2 e a: Multiple erythematous necrotic papules with annular scaly plaques on the periocular region, face with bilateral
periocular edema. b: Multiple erythematous necrotic papules on the back.
a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1 e32
Please cite this article in press as: Patil AT, et al., Subacute cutaneous lupus erythematosus associated with lupus nephritis,
Apollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.03.003

4. common in adults. Females are more affected. Worldwide
SCLE prevalence ranges from 17e48 cases per 100,000 pop-
ulations.2
About 15e20% of SCLE patients also have other
types of CLE lesions, and about 50% of SCLE patients fulfil the
American College of Rheumatology (ACR) criteria for SLE but
seldom develop systemic disease. In our case it was inter-
esting to note the renal involvement in the form of lupus
nephritis.3
Etiopathogenesis of SLE is multi factorial. Genetic predis-
position is a major risk factor for the development of SCLE.
SCLE is associated with human leucocyte antigen (HLA)-B8,
HLA-DR3, HLA-DRw52, and HLA-DQ1.4
SCLE has been associ-
ated with complement C2 and C4. SCLE usually manifests
following light exposure, but other triggers or inciting factors
may also be involved as not all patients develop disease
following photoexposure. SCLE is the most common photo-
sensitive variant and both Ultraviolet A and B are potentially
pathogenic.5
The eruption of SCLE initially presents as erythematous
papules or small plaques which eventually develops to form
hyperkeratotic papulosquamous or annular or polycyclic le-
sions. They occur on exposed sites that are neck, followed by
face, extensor aspects of hands, arms, lower limb and scalp.
80% of patients develop lesions on trunk and upper extrem-
ities while only 20% have lesions on face or scalp.6
The course
of the disease is often marked by exacerbations and re-
missions. They usually heal without scarring, leaving behind
post inflammatory hyperpigmentation.
SCLE is unusually less severe than acute variant but rarely
can they develop severe symptoms with multiorgan involve-
ment. 50% of patients with SCLE meet criteria for having SLE,
and approximately 10% of SLE patients have SCLE lesions.
Individuals with papulosquamous type of SCLE are more likely
to develop renal disease.7
50% of SCLE patients are associated
with joint involvement.
Lupus nephritis is quite common in childhood onset SLE.
Approximately 50e70% of SLE patients presents with renal
involvement and could be the initial presenting symptom.
Cutaneous lesions may develop initially along with systemic
symptoms or may present later. In our case the child was
diagnosed with lupus nephritis and managed but subse-
quently after few months developed cutaneous lesions sug-
gestive of SCLE.
Majority of SCLE shows positive ANA and positive Anti Ro/
SSA and Anti La/SSB autoantibodies. Anti dsDNA is usually
positive in systemic lupus erythematosus but may be positive
in 12% patients with SCLE.8
Skin biopsy shows licheniod
degeneration of basal cell layer with perivascular and peri-
appendageal inflammatory infiltrate with fibrin deposition in
dermis.
Management should be individualized and adjusted ac-
cording to disease activity. Measures include topical sun-
screens and systemic treatment includes oral steroids and
immunosuppressants like hydroxychloroquine, metho-
trexate, mycophenolate mofetil, azathioprine, dapsone,
intravenous immunoglobulin, cyclosporine and
cyclophosphamide.9
Since this child has extensive erosive
skin lesions, was treated with intravenous methlypredniso-
lone for 3 days and continued on oral steroids. Her lesions
subsided within 5 days and she showed excellent response.10
In conclusion, any child with mucocutaneous lesion asso-
ciated with systemic involvement in SLE needs to be regularly
assessed and monitored. Sun protection is vital and should be
encouraged. In children, mucocutaneous lesions associated
with systemic disease require treatment with systemic
immunosuppressive drugs in order to achieve adequate dis-
ease control.
Conflicts of interest
All authors have none to declare.
Acknowledgement
We would like to thank Anand diagnostics laboratory for
providing the renal biopsy pictures.
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Please cite this article in press as: Patil AT, et al., Subacute cutaneous lupus erythematosus associated with lupus nephritis,
Apollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.03.003

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