Table 3.
Characteristics of individual patients from DITRA studies included in the review
| DITRA patient | Country | Age | Gender | Cutaneous presentation | Cutaneous clinical evolution | Fever | Elevated acute-phase reactants | Previous treatment | Mutations | Ref. | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Gestation (weeks) | Onset (days) | Diagnosis (months) | ||||||||||
| #1 | Morocco | 41 | 60 | 17 | M | Multiple pustules appeared in the perioral and diaper area | Recurrent episodes of erythroderma | + | + | Cyclosporine | Homozygous for IL36RN c.80T > C (p.Leu27Pro) mutation | [1] |
| #2 | NA | NA | 1080 | 38 | F | Generalized pustular lesions | Generalized | + | NA |
Antibiotics Corticosteroids Acitretin Cyclosporine |
Compound heterozygous for IL36RN c.227C > T (p.Pro76Leu) and c.338C > T (p.Ser113Leu) mutations | [1] |
| #3 | NA | NA | Infancy | Adolescent | F | Generalized pustular psoriasis | Generalized pustular psoriasis | NA | + |
Corticosteroids Acitretin Methotrexate Cyclosporine Adalimumab Infliximab |
Homozygous for IL36RN c.115 + 6T > C mutation | [1] |
| #4 | Tunisia | NA | 14 years | 3 | M | Erythematous and scaly eruption over the scalp and perineum | Generalized exfoliative dermatitis | No | + |
Corticosteroids Acitretin |
Homozygous for IL36RN c.80T > C (p.Leu27Pro) mutation | [1] |
| #5 | NA | NA | 47 years | 54 years | M | NA | NA | NA | NA |
Acitretin Methotrexate Cyclosporine Infliximab |
Heterozygous for IL36RN c.338C > T (p.Ser113Leu) mutation | [1] |
| #6 | NA | NA | NA | NA | M | Generalized pustular lesions | Generalized erythema | NA | NA |
Corticoids Acitretin Cyclosporine |
Heterozygous mutation for IL36RN c338C > T (pSer113Leu) | [1] |
| #7 | NA | NA | 40 years | 40 years | M | Scaly erythema on elbows | Severe flare of GPP | + | + |
Antibiotics Corticosteroids Methotrexate Cyclosporine Adalimumab |
Compound heterozygous for IL36RN c.142C > T (p.Arg48Trp) and IL36RN c.338C > T (p.Ser113Leu) mutations | [5] |
| #8 | Morocco | 37 | 60 | 8 | F | Initially located at the diaper area | Generalized | + | + |
Antibiotics Corticosteroids Acitretin |
Compound heterozygous for L36RN c.T80C (p.Leu27Pro), c338C > T (p.Ser113Leu) and c.142C > T (p.Arg48Trp) mutations, and NSD1 (5q35.2-q35.3) microduplication | [35] |
| #9 | Denmark | 38 | 90 | 4 | F | Generalized pustular psoriasis | Generalized pustular psoriasis | NA | Normal |
Antibiotics Corticosteroids Methotrexate |
Compound heterozygous for IL36RN c.338C > T (p.Ser113Leu) and NLRP3 c.2107C > A (p.Gln703Lys) | [35] |