subacute cutaneous lupus erythematosus associated with lupus nephritis

5
Subacute cutaneous lupus erythematosus associated with lupus nephritis

Upload: apollo-hospitals

Post on 15-Jul-2015

99 views

Category:

Health & Medicine


0 download

TRANSCRIPT

Page 2: Subacute cutaneous lupus erythematosus associated with lupus nephritis

ww.sciencedirect.com

a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1e3

Available online at w

ScienceDirect

journal homepage: www.elsevier .com/locate/apme

Case Report

Subacute cutaneous lupus erythematosusassociated 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, Indiab Consultant Pediatric Dermatology, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health,

Indiac Post Graduate Student Pediatrics, Indira Gandhi Institute of Child Health, Indiad 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

* Corresponding author.E-mail address: [email protected]

http://dx.doi.org/10.1016/j.apme.2015.03.0030976-0016/Copyright © 2015, Indraprastha M

Please cite this article in press as: Patil AApollo Medicine (2015), http://dx.doi.org/

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.

om (A.T. Patil).

edical Corporation Ltd. A

T, et al., Subacute cuta10.1016/j.apme.2015.03.

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

ll rights reserved.

neous lupus erythematosus associated with lupus nephritis,003

Page 3: Subacute cutaneous lupus erythematosus associated with lupus nephritis

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]

a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1e32

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 odemawas 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

Fig. 2 e a: Multiple erythematous necrotic papules with annula

periocular edema. b: Multiple erythematous necrotic papules on

Please cite this article in press as: Patil AT, et al., Subacute cutaApollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.03.

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

r scaly plaques on the periocular region, face with bilateral

the back.

neous lupus erythematosus associated with lupus nephritis,003

Page 4: Subacute cutaneous lupus erythematosus associated with lupus nephritis

a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) 1e3 3

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.

Individualswith papulosquamous type of SCLE aremore 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

Please cite this article in press as: Patil AT, et al., Subacute cutaApollo Medicine (2015), http://dx.doi.org/10.1016/j.apme.2015.03.

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 andmonitored. 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.

r e f e r e n c e s

1. Jimenez S, Cervera R, Font J, Ingelmo M. The epidemiology ofsystemic lupus erythematosus. Clin Rev Allergy Immunol.2003;25:3e12.

2. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence ofcutaneous lupus erythematosus, 1965-2005: a populationbased study. Arch Dermatol. 2009;145:249e253.

3. Callen JP. Drug-induced subacute cutaneous lupuserythematosus. Lupus. 2010;19:1107e1111.

4. Lin JH, Dutz JP, Sontheimer RD, Werth VP. Pathophysiology ofcutaneous lupus erythematosus. Clin Rev Allergy Immunol.2007;33:85e106.

5. Klein LR, Elmets CA, Callen JP. Photoexacerbation ofcutaneous lupus erythematosus due to ultraviolet Aemissions from a photocopier. Arthritis Rheum.1995;38:1152e1156.

6. Tebbe B. Clinical course and prognosis of cutaneous lupuserythematosus. Clin Dermatol. 2004;22:121e124.

7. Callen JP, Kulick KB, Stelzer G, Fowler JF. Subacute cutaneouslupus erythematosus. Clinical, serologic, and immunogeneticstudies of forty-nine patients seen in a nonreferral setting. JAm Acad Dermatol. 1986;15:1227e1237.

8. Chiewchengchol D, Murphy R, Edwards SW, Beresford MW.Mucocutaneous manifestations in juvenile-onset systemiclupus erythematosus: a review of literature. Pediatr Rheumatol.2015;13:1e9.

9. Pai VV, Naveen K, Athanikar S, Dinesh U, Reshme P,Divyashree R. Subacute cutaneous lupus erythematosuspresenting as erythroderma. Indian J Dermatol. 2014;59:634.

10. Cardinali C, Melani L, Giomi B, Caproni M, Fabbri P. Systemiclupus erythematosus with unusual maculopapular anderosive cutaneous lesions. Skin Med. 2004;3:292e293.

neous lupus erythematosus associated with lupus nephritis,003

Page 5: Subacute cutaneous lupus erythematosus associated with lupus nephritis

Apollo hospitals: http://www.apollohospitals.com/Twitter: https://twitter.com/HospitalsApolloYoutube: http://www.youtube.com/apollohospitalsindiaFacebook: http://www.facebook.com/TheApolloHospitalsSlideshare: http://www.slideshare.net/Apollo_HospitalsLinkedin: http://www.linkedin.com/company/apollo-hospitalsBlog:Blog: http://www.letstalkhealth.in/