| Sahn et al., 1992 |
1 |
30years/F |
At 8 months of age, she presented severe and intermittent scaly lesions associated with blisters. In adolescence, she presented hyperkeratotic plaques in the flexures and joints. At the age of 27, she had intermittent annular hyperkeratotic, erythematous, polycyclic plaques on the trunk and proximal region of the limbs, associated with mild palmoplantar keratoderma with fissures. |
Topical use of 60% propylene glycol and 6% salicylic acid. Partial response to treatment. |
| 2 |
2years/M (son) |
At 6 months of age, he developed papules that developed into boli and crusts. At 2 years of age, he had keratotic plaques on his neck and armpits, in addition to multiple erythematous papules on the trunk, extremities, and ear. No report of palmoplantar keratoderma. |
|
| Joh et al., 1997 |
3 (father) |
33years/M |
Blisters disseminated from birth. From the 1st year of life, intermittent hyperkeratotic and erythematous plaques associated with blisters and itching up to 16 years of age. At the age of 31, he again presented intermittent blisters and hyperkeratotic plaques in the flexures, ankles, and back of the hands associated with annular migratory, polycyclic, scaly, and erythematous plaques in the trunk and proximal region of the limbs. Palmoplantar keratoderma was not reported. |
Oral acitretin 20 mg per day topically associated with ointment containing propylene glycol and 5% urea. Good response to treatment. |
| 4 (daughter of case 3) |
2nd day of life/F |
Generalized bullous lesions and moderate erythema since the 2nd day after birth. At 8 weeks of age, she had erosions in the groin and inner thighs associated with mild desquamation on the dorsum of the feet. No report of palmoplantar keratoderma. |
Unreported. |
| Suga et al., 1998 |
5 |
11years/M |
No history of blisters. Since the age of 7 months, xerotic plates in the flexures and extensor regions. Intermittent episodes of annular, erythematous, scaly, and serpiginous plaques on the trunk and flexures at 11 years of age. No report of palmoplantar keratoderma. |
Unreported |
| Michael et al., 1999 |
6-9 (members of same family) |
NA |
Transient blisters from birth. Intermittent erythematous plaques, non-migratory polycyclic hyperkeratotic plaques on the trunk associated with palmoplantar keratoderma. |
Topical use of corticosteroids, calcipotriene, tazarotene. and salicylic acid. Minimum response to treatment. |
| Sybert et al., 1999 |
10 |
5years/M |
The first hours of life showed bullous lesions and erosions. Since the 1st month, he had intermittent palmoplantar thickening and erythroderma. At 3 years of age, migratory and intermittent, hyperkeratotic, erythematous plaques on the chest, back, and flexures. |
Unreported. |
| 11 (mother of case 10) |
18 years/F |
Intermittent and migratory erythematous plaques. Erythema and skin erosions since birth. Intermittent palmoplantar hyperkeratosis. Intermittent episodes of erythroderma and palmoplantar keratoderma. |
|
| 12 (aunt of case 10) |
NA/F |
|
|
| Yoneda et al., 1999 |
13 and 14 (mother and son) |
48years/F |
Blisters disseminated after birth in both cases with spontaneous improvement in the first months and worsening at around 4 years of age, with annular hyperkeratotic and migratory plaques in the trunk and extremities, maintaining this condition intermittently until adulthood. No report of palmoplantar lesions. |
|
| Etretinate without improvement (did not report dose). |
18years/M |
|
| Naik et al., 2003 |
15 |
21years/F |
No reports of cutaneous lesions at birth. Since the 1st year of life, there were intermittent and migrating polycyclic erythematous plaques on the trunk and hyperkeratotic, verrucous, brownish plaques on the knees, elbows, and ankles. Intermittent palmoplantar keratoderma. |
Oral isotretinoin, topical glucocorticoids, and keratolytics. Topical tazarotene with partial response. |
| Jha et al., 2015 |
16 |
26years/F |
Spreading blisters, recurrent since birth. Intermittent hyperkeratotic plaques on the trunk from the 1st year of life to 8 years of age. She had hyperkeratotic plaques in the flexures from childhood to adulthood. After the age of 23, she had annular, intermittent, hyperkeratotic, scaly erythematous plaques on the trunk and thighs. No report of palmoplantar keratoderma. |
Acitretin 0.5 mg/kg/day. Good response four weeks after starting treatment. |
| 17 |
2nd day of life/F |
Erythroderma at birth. In the first months of life, hyperkeratotic, polycyclic, scaly plaques on the trunk and limbs. No report of palmoplantar keratoderma. |
Use of emollients. |
| Abdul-Wahab et al., 2016 |
18 |
25years/F |
No history of lesions at birth. History of ichthyosiform lesions in the knees, elbows, and flexures in childhood. At 25 years of age, erythematous, scaly, and migratory plaques on the trunk and limbs. No report of palmoplantar lesions. |
Low dose oral isotretinoin with good response. |
| Zaki et al., 2018 |
19 |
5years/F |
Blisters disseminated after birth. Migratory and intermittent erythematous plaques, in addition to hyperkeratotic plaques on the trunk and flexures. Hyperkeratosis and palmoplantar scaling since the first months of life. |
Unreported. |
