Traditionally, HV is treated conservatively, since classic HV usually undergoes spontaneous remission before adulthood. The therapeutic strategies consist of broad-spectrum sunscreens with a high sun protection factor and avoidance of sunlight exposure [
7,
28,
40]. In patients who do not respond to conservative treatment or have a prolonged refractory course, dermatologists should consider the possibility of HVLPD, particularly atypical/severe HV or HVLL. Currently, there are no guidelines to treat patients with HVLPD, mostly owing to the rarity of the disease. Several regimens, including phototherapy [
41], immunomodulatory agents (antiviral agents, interferon, thalidomide, hydroxychloroquine, intravenous immunoglobulin, etc.) [
17,
19,
49,
50,
51,
52], systemic corticosteroids [
17,
19], chemotherapy [
19,
53], and hematopoietic stem cell transplantation [
19,
53] have been used with variable rates of success. Regarding phototherapy, narrowband UVB (NBUVB) phototherapy three times weekly for five weeks is most often recommended [
41], whereas psoralen-UVA (PUVA) photochemotherapy is not recommended for younger patients. In a report of 41 cases with HVLPD, Liu et al. suggested conservative treatment for most Chinese patients with HVLPD, and that chemotherapy should not be used as a first-line treatment [
19]. Similarly, studies from Latin American nations have reported poor outcomes in patients receiving chemotherapy [
54]. If chemotherapy fails to eradicate lymphoma cells, this therapy may worsen the prognosis by inducing immunosuppression and reactivating EBV replication. Among a variety of therapeutic modalities, immunomodulatory therapy for HVLPD is often recommended [
37]. Immunomodulator agents can modulate the host inflammatory response by enhancing innate immunity against viral replication, and can suppress disease aggressiveness.