Linear lupus erythematosus profundus in a child

Area of Science:

• Dermatology
• Pathology
• Rheumatology

Background:

• Lupus erythematosus profundus (LEP) is a rare, deep form of cutaneous lupus.
• It typically presents as subcutaneous nodules or plaques, often associated with systemic lupus erythematosus.
• Linear presentations of LEP are exceptionally uncommon in pediatric cases.

Observation:

• A 9-year-old Japanese male presented with a 6-year history of a linear skin eruption on his left leg.
• The eruption followed a distinct linear pattern, suggesting a specific etiological or developmental process.
• Clinical examination noted persistent inflammatory changes along the affected limb.

Findings:

• Histopathological examination revealed intense lymphocytic panniculitis, indicating deep dermal inflammation.
• Perivascular and periappendageal infiltrates of lymphocytes were prominent, characteristic of inflammatory dermatoses.
• Vacuolization of the basal cell layer suggested interface dermatitis, a common feature in lupus erythematosus.

Implications:

• This case expands the known clinical spectrum of lupus erythematosus profundus.
• It highlights the importance of considering LEP in pediatric patients with unusual linear inflammatory skin conditions.
• The findings may prompt further research into the pathogenesis and diagnostic criteria for linear LEP.