Drug1st line

Hydroxychloroquine

Antimalarial; modulates immune response, inhibits TLR signalling

Response rate
~57% response rate (UV data)
Onset
Weeks–months
Route
Oral 200-400 mg daily
Line
1st
IgM effect
Partial complement improvement
Evidence level
amber

Evidence summary

One of the first drugs shown effective in HUVS (Lopez et al. 1984). First-line for mild-to-moderate cutaneous disease with arthralgias. Part of the colchicine-HCQ-dapsone backbone used in the French nationwide study. Reported helpful in approximately 57% of UV patients.

Approved indications

Conditions for which Hydroxychloroquine has regulatory approval (not specific to rare diseases covered here):

Systemic Lupus ErythematosusRheumatoid ArthritisMalaria

Drug identifiers

DrugBankDB01611
ATC CodeP01BA02