Lupus profundus in a three years old boy with a favorable response to hydroxychloroquine and steroids: Case report & a comprehensive case-based review

Abstract

Background

To ensure patients receive timely dermatologic care, dermatologists and rheumatologists must be fully aware of the full range of cutaneous diseases in systemic lupus erythematosus (SLE).

Aim of the work

This report aims to highlight a rare presentation of lupus profundus in a pre-school aged boy to highlight diagnostic challenges, management, and outcome. Similar juvenile cases worldwide are also presented for a comparison via a comprehensive review of existing literature.

Case presentation

A three-year-old Saudi boy of non-consanguineous parents and with six healthy siblings, presented at two years of age with multiple 1–2 cm firm, erythematous, indurated and mildly tender subcutaneous nodules and plaques on the right upper-arm and lower-extremities. Some lesions were ulcerative with suppurative discharge. There were no systemic symptoms of fever, arthritis, or photosensitivity, making the diagnosis of SLE unlikely. Anti-nuclear antibody (ANA) was negative and complements normal. Skin biopsy revealed perivascular lymphohistiocytic infiltrate mixed with plasma cells, follicular plugging, superficial telangiectasia, dermal fibrosis, lobular and septal panniculitis and hyaline fat necrosis. The patient received potent topical steroid twice daily, and hydroxychloroquine (HCQ) 5 mg/kg, and prednisolone 5 mg oral daily. At 8-weeks, there was complete clinical resolution, with residual mild lipoatrophy. A 1-year follow-up found no recurrence or progression to SLE.

Conclusion

The diagnosis of lupus profundus in children is confirmed by biopsy and early intervention using HCQ, local and systemic steroid therapy can noticeably lead to rapid complete remission and to minimize cosmetic and psychosocial sequelae. Long-term follow-up is recommended to monitor for recurrence or SLE progression in the pediatric population.