Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

Table 1 Cutaneous manifestations, demographic data, disease activity, outcome, skin biopsy and treatment in six childhood-onset systemic lupus erythematosus (cSLE) patients with lupus erythematosus panniculitis (LEP)

Patient	Interval between LEP and cSLE diagnosis, years	SLEDAI-2 K	Painful Plaques / painful subcutaneous nodules	Cutaneous hyperpigmentation / hypopigmentation / atrophy	Skin biopsy findings; direct immunofluorescence staining (DIF)	Treatments
1	−2.5	3	- / +	+ / - / +	LP; mild GCD of IgM at DEJ)	PD, AM, NSAID, AZA
2	11.4	13	+ / +	+ / - / +	LP with vasculitis; moderate GCD of IgM at DEJ)	PD, MP, AM, AZA, MTX, IVIG
3	5.5	13	+ / +	+ / + / +	LP; intense GCD of IgG, IgG, IgA and C3 at DEJ	PD, MP, CY, MTX
4	0	25	- / +	- / - / -	LP, vasculopathy obliterans; absence of IgG, IgM, IgA, C3 and C4 at DEJ	No treatment^a
5	0	0	+ / +	+ / + / +	LP; absence of IgG, IgM, IgA, C3 and C4 at DEJ	PD, AM, NSAID, MMF, rituximab
6	9.91	5	+ / +	+ / - / +	LP; ND	PD, MP, AM, MTX, MMF, IVCYC

^adeceased before starting any treatment, + presence, − absence, LP lobular panniculitis, APS antiphospholipid syndrome PD prednisone, MP methylprednisolone, NSAID nonsteroidal anti-inflammatory, AM antimalarial, AZA azathioprine, IVCYC intravenous cyclophosphamide, CY cyclosporine, MTX methotrexate, MMF mycopheneolate mofetil, IVIG intravenous immunoglobulin, GCD granular and continuous deposits, DEJ dermo-epidermal junction, ND not done, SLEDAI-2 K Systemic Lupus Erythematosus Disease Activity Index 2000

ISSN: 2523-3106