Research Article

A Rare Manifestation of Bullous Systemic Lupus Erythematosus in Children: A 10-year Retrospective Study in a Tertiary Care Hospital

Table 1

Description of clinical characteristic, laboratory findings, treatments, and outcomes of pediatric BSLE patients in the study population.

CategoryNo. 1No. 2No. 3No. 4No. 5

Age at diagnosis (years)1011131215
GenderFMFFF
Constitutional symptoms/weight lossYesYesYesYesYes

Cutaneous lesion
(i) Vesiculobullous patternGeneralized tense bullae with varying sizes on the face, trunk, back, and extremitiesGeneralized tense bullae with varying sizes on the face, lips, trunk, back, and genitaliaMultiple tense bullae on the lips, perioral, and genital areaMultiple tense bullae on the lips, mucosa, and genital areaLarge and multiple tense bullae on the lips and perioral area
(ii) Oral ulcerYesYesYesYesYes
(iii) Mucosal lesionsYesYesYesYesYes
(iv) Other cutaneous manifestationNoCutaneous vasculitis, palmar erythemaMalar rashDiscoid rash on the scalp with scarring alopeciaNo

Histopathological tissue compatible with SLE
(i) SkinYesYesNoNoYes
(ii) KidneyNoNoYesYesNo
(iii) Other tissueNoNoNoPericardiumNo

Systemic involvement
 Neurological involvementNoYes (Seizure)Yes (Seizure)NoNo
 PolyarthritisNoNoNoYesYes
 SerositisYes (pleural effusion)Yes (pleural effusion)Yes (pleural and pericardial effusion)Yes (pericardial effusion with cardiac tamponade)Yes (pleural effusion)
 Hematological abnormalitiesYesYesYesYesYes
(i) AIHAYesYesYesYesYes
(ii) LymphopeniaYesYesYesYesYes
(iii) ThrombocytopeniaNoYesYesYesYes
(iv) MAS/HLHNoYesNoNoNo
(v) DCTNegativePositivePositivePositivePositive
 Renal involvementYesYesYes (LN class IV)Yes (LN class IV)Yes
(i) HypertensionYesYesYesYesYes
(ii) BUN/Creatinine72.7/2.735.3/1.7333/1.426.9/1.7723/1.07
(iii) ProteinuriaYes (protein 4+)Yes (protein 1+)Yes (protein 3+)Yes (protein 2+)Yes (protein 3+)
(iv) GlomerulonephritisYes (RBC 10–20)Yes (RBC 5–10)Yes (RBC 20–30)Yes (RBC 30–50)Yes (RBC 10–20)
(v) UPCR (mg/mg)35.77.53.72
(Normal range <0.2)

Complement levels
(i) C3 (normal range 90–180)Low (52.9)Low (9.8)Low (12.3)Low (22)Low (18)
(ii) C4 (normal range 10–40)Low (10.7)Low (0.6)Low (6)Low (7)Low (5)
Vitamin D level (normal range >20)Low (8.24)Low (23DeficiencyN/AN/A
Serum albumin (g/dl)3.22.12.43.33.0

ANAPositivePositivePositivePositivePositive
Coarse speckle typeCoarse speckle typeHomogenous typeHomogenous typeCoarse speckle type
1 : 25601 : 25601 : 12801 : 51201 : 2560

Anti-dsDNAPositivePositivePositivePositivePositive
720 IU/ml104.8 IU/ml

Anti-smithPositiveNegativeN/AN/AN/A
116 IU/ml

TreatmentsDapsoneDapsonePrednisoloneDapsoneDapsone
PrednisoloneIVIGMMFPrednisolonePrednisolone
MMFPrednisoloneCPMMFHCQ
HCQMMFHCQHCQ
HCQ

Outcomes
(i) Cutaneous clearance (days)14567510
(ii) Long-term cutaneous lesionsDiscolouration hypopigmentation on the involved areaDiscolouration on the involved areaHypopigmentation on the involved areaComplete recoveryComplete recovery

(iii) Renal impairmentYesYesYesYesYes
LNLNLNLNLN

(iv) Hematological abnormalitiesImproveImproveRecoveryRecoveryRecovery

AIHA, autoimmune induced hemolytic anemia; ANA, antinuclear antibody; Anti-dsDNA, anti-double stranded DNA; BUN, blood urea nitrogen; CP, cyclophosphamide; DCT, direct Coombs’ test; HCQ, hydroxychloroquine; HLH, hemophagocytic lymphohistiocytosis; IVIG, intravenous immunoglobulin; LN, lupus nephritis; MAS, macrophage activation syndrome; MMF, mycophenolate mofetil; N/A, not available; UPCR, urine protein/creatinine ratio.