Clinical data of 20 Mexican children with HVLL
| Case . | Sex . | Age . | Clinical diagnosis . | Symptoms at diagnosis . | Treatment . | Follow-up . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Edema . | Blisters . | Ulcers . | Crusts . | Scars . | FTTH + HS . | ||||||
| 1a | M | 10 | ESVP | Thalidomide, topical steroids | 9 y | ||||||
| 1b | 16 | 3 y history | − | + | − | + | + | + | Remission and recurrences AwD | ||
| 2 | F | 13 | Lymphoma | − | + | + | − | − | − | — | LFU |
| 3 | M | 4 | EVSP | — | LFU | ||||||
| 2 y history | − | + | − | + | + | + | |||||
| 4a | M | 11 | HVLL | Thalidomide, topical steroids | 13 y | ||||||
| 4b | 20 | − | + | − | + | + | + | Chemotherapy | Remission and recurrences | ||
| 4c | 24 | AwD | |||||||||
| 5 | M | 1 | Bullous urticaria | − | + | − | + | + | − | — | LFU |
| 6 | F | 6 | ESVP | Chloroquine | 5 mo improvement | ||||||
| 1.5 y history | + | + | + | + | + | + | LFU | ||||
| 7 | M | 13 | ESVP | − | + | + | — | LFU | |||
| 8a | F | 10 | ESVP | Cyclophosphamide systemic steroids | 3 y remission and recurrences | ||||||
| 8b | 13 | Vasculitis, HMB | − | + | + | + | + | + | LFU | ||
| 9 | M | 6 | ESVP | Thalidomide, systemic steroids, cyclophosphamide | 3 y improved | ||||||
| 2 mo | + | + | + | + | + | + | DwD* | ||||
| 10 | M | 8 | Psoriasiform dermatitis/ESVP | Systemic steroids, IFN | 13 mo improved | ||||||
| 12 mo history | − | + | − | + | + | + | LFU | ||||
| 11 | F | 11 | ESVP 2 y history | Thalidomide | 4 y improved | ||||||
| 15 | PTCL, nos | + | + | + | + | + | − | — | LFU/DoD | ||
| 12 | M | 15 | ESVP | Thalidomide, | 2 y Improved | ||||||
| 12 mo history | + | − | + | + | + | − | Topical steroids, chloroquine | LFU | |||
| 13 | M | 8 | ESVP | Thalidomide, systemic steroids | 1 mo improved | ||||||
| 5 y history | + | + | + | + | + | − | LFU | ||||
| 14 | M | 12 | ESVP | Thalidomide, systemic steroids, cyclophosphamide | 2 mo improved | ||||||
| 18 mo history | − | + | + | + | + | − | LFU | ||||
| 15° | M | 6 | ESVP | Thalidomide, systemic steroids, methrotexate, cyclophosphamide | 12 y | ||||||
| 15b | 7 | + | + | + | + | + | + | AwD | |||
| 15c | 12 | ||||||||||
| 16° | M | 8 | ESVP | Thalidomide | 4 y | ||||||
| 16b | 12 | Nasal NK/T-cell lymphoma | + | + | + | + | + | + | Chemotherapy | DoD | |
| 16c | 12 | ||||||||||
| 17 | F | 6 | ESVP, HMB | — | LFU | ||||||
| 4 y history | − | + | + | + | + | − | |||||
| 18 | F | 6 | ESVP | + | + | + | + | + | − | — | LFU |
| 19 | M | 8 | ESVP, HMB | — | LFU | ||||||
| 3 y history | + | + | + | + | + | − | |||||
| 20 | M | 15 | HVLL, HMB | Chemotherapy | 2 mo | ||||||
| 2 y history | − | + | + | + | + | − | DoD | ||||
| Case . | Sex . | Age . | Clinical diagnosis . | Symptoms at diagnosis . | Treatment . | Follow-up . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Edema . | Blisters . | Ulcers . | Crusts . | Scars . | FTTH + HS . | ||||||
| 1a | M | 10 | ESVP | Thalidomide, topical steroids | 9 y | ||||||
| 1b | 16 | 3 y history | − | + | − | + | + | + | Remission and recurrences AwD | ||
| 2 | F | 13 | Lymphoma | − | + | + | − | − | − | — | LFU |
| 3 | M | 4 | EVSP | — | LFU | ||||||
| 2 y history | − | + | − | + | + | + | |||||
| 4a | M | 11 | HVLL | Thalidomide, topical steroids | 13 y | ||||||
| 4b | 20 | − | + | − | + | + | + | Chemotherapy | Remission and recurrences | ||
| 4c | 24 | AwD | |||||||||
| 5 | M | 1 | Bullous urticaria | − | + | − | + | + | − | — | LFU |
| 6 | F | 6 | ESVP | Chloroquine | 5 mo improvement | ||||||
| 1.5 y history | + | + | + | + | + | + | LFU | ||||
| 7 | M | 13 | ESVP | − | + | + | — | LFU | |||
| 8a | F | 10 | ESVP | Cyclophosphamide systemic steroids | 3 y remission and recurrences | ||||||
| 8b | 13 | Vasculitis, HMB | − | + | + | + | + | + | LFU | ||
| 9 | M | 6 | ESVP | Thalidomide, systemic steroids, cyclophosphamide | 3 y improved | ||||||
| 2 mo | + | + | + | + | + | + | DwD* | ||||
| 10 | M | 8 | Psoriasiform dermatitis/ESVP | Systemic steroids, IFN | 13 mo improved | ||||||
| 12 mo history | − | + | − | + | + | + | LFU | ||||
| 11 | F | 11 | ESVP 2 y history | Thalidomide | 4 y improved | ||||||
| 15 | PTCL, nos | + | + | + | + | + | − | — | LFU/DoD | ||
| 12 | M | 15 | ESVP | Thalidomide, | 2 y Improved | ||||||
| 12 mo history | + | − | + | + | + | − | Topical steroids, chloroquine | LFU | |||
| 13 | M | 8 | ESVP | Thalidomide, systemic steroids | 1 mo improved | ||||||
| 5 y history | + | + | + | + | + | − | LFU | ||||
| 14 | M | 12 | ESVP | Thalidomide, systemic steroids, cyclophosphamide | 2 mo improved | ||||||
| 18 mo history | − | + | + | + | + | − | LFU | ||||
| 15° | M | 6 | ESVP | Thalidomide, systemic steroids, methrotexate, cyclophosphamide | 12 y | ||||||
| 15b | 7 | + | + | + | + | + | + | AwD | |||
| 15c | 12 | ||||||||||
| 16° | M | 8 | ESVP | Thalidomide | 4 y | ||||||
| 16b | 12 | Nasal NK/T-cell lymphoma | + | + | + | + | + | + | Chemotherapy | DoD | |
| 16c | 12 | ||||||||||
| 17 | F | 6 | ESVP, HMB | — | LFU | ||||||
| 4 y history | − | + | + | + | + | − | |||||
| 18 | F | 6 | ESVP | + | + | + | + | + | − | — | LFU |
| 19 | M | 8 | ESVP, HMB | — | LFU | ||||||
| 3 y history | + | + | + | + | + | − | |||||
| 20 | M | 15 | HVLL, HMB | Chemotherapy | 2 mo | ||||||
| 2 y history | − | + | + | + | + | − | DoD | ||||
AwD, alive with disease; DoD, died of disease: DwD, died without disease; ESVP, edematous, scarring vasculitic panniculitis; FTTH, failure to thrive; HS, hepatosplenomegaly; IFN, interferon; LFU, lost to follow-up.
Died of hepatic failure, no active skin lesions.