A Congenital Ichthyosiform Syndrome With Deafness and Keratitis

Spiney hyperkeratosis with hair shaft alterations, keratitis and deafness

“…Doubtless, they have an important role in the hair alterations described in this syndrome, as generalized alopecia with fault of eyebrows and eyelashes and later development of fine scarce hair, or defects as trichorrhexis nodosa, have been seen in one case [4]. We noted that these thin hairs showed different non-specific alterations, evidenced by scanning eleetronmicroscopy in one case.…”

Spiney hyperkeratosis with hair shaft alterations, keratitis and deafness

“…Doubtless, they have an important role in the hair alterations described in this syndrome, as generalized alopecia with fault of eyebrows and eyelashes and later development of fine scarce hair, or defects as trichorrhexis nodosa, have been seen in one case [4]. We noted that these thin hairs showed different non-specific alterations, evidenced by scanning eleetronmicroscopy in one case.…”

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…In addition to these fatalities, infants and young children with KID syndrome are commonly afflicted by multiple, recurrent, extracutaneous and invasive infections, often requiring hospitalization. Several reports have been made of affected children with recurrent episodes of otitis media (14–16,18,24), otitis externa (11,14,15,17,24), conjunctivitis (13,14,16,17), urinary tract infections (11,12,17,24), pneumonia (11,17,19,23), sinusitis (18), gastroenteritis (22), and oral candidiasis (19).…”

Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…A vascularizing keratitis of the cornea, the third major feature of KID syndrome, occurs in about three‐fourths of patients (1). Gram et al (15) suggest that these corneal changes may be a reflection of the generalized ectodermal disturbance, since the anterior portion of the cornea is similarly derived from the ectoderm. Development of keratitis has been reported to occur as late as the age of 2 years (1,10).…”

Spiney hyperkeratosis with hair shaft alterations, keratitis and deafness

“…Doubtless, they have an important role in the hair alterations described in this syndrome, as generalized alopecia with fault of eyebrows and eyelashes and later development of fine scarce hair, or defects as trichorrhexis nodosa, have been seen in one case [4]. We noted that these thin hairs showed different non-specific alterations, evidenced by scanning eleetronmicroscopy in one case.…”

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…In addition to these fatalities, infants and young children with KID syndrome are commonly afflicted by multiple, recurrent, extracutaneous and invasive infections, often requiring hospitalization. Several reports have been made of affected children with recurrent episodes of otitis media (14–16,18,24), otitis externa (11,14,15,17,24), conjunctivitis (13,14,16,17), urinary tract infections (11,12,17,24), pneumonia (11,17,19,23), sinusitis (18), gastroenteritis (22), and oral candidiasis (19).…”

Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…A vascularizing keratitis of the cornea, the third major feature of KID syndrome, occurs in about three‐fourths of patients (1). Gram et al (15) suggest that these corneal changes may be a reflection of the generalized ectodermal disturbance, since the anterior portion of the cornea is similarly derived from the ectoderm. Development of keratitis has been reported to occur as late as the age of 2 years (1,10).…”

Spiney hyperkeratosis with hair shaft alterations, keratitis and deafness

“…Doubtless, they have an important role in the hair alterations described in this syndrome, as generalized alopecia with fault of eyebrows and eyelashes and later development of fine scarce hair, or defects as trichorrhexis nodosa, have been seen in one case [4]. We noted that these thin hairs showed different non-specific alterations, evidenced by scanning eleetronmicroscopy in one case.…”

Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…In addition to these fatalities, infants and young children with KID syndrome are commonly afflicted by multiple, recurrent, extracutaneous and invasive infections, often requiring hospitalization. Several reports have been made of affected children with recurrent episodes of otitis media (14–16,18,24), otitis externa (11,14,15,17,24), conjunctivitis (13,14,16,17), urinary tract infections (11,12,17,24), pneumonia (11,17,19,23), sinusitis (18), gastroenteritis (22), and oral candidiasis (19).…”

Keratitis, Ichthyosis, and Deafness (KID) Syndrome

“…A vascularizing keratitis of the cornea, the third major feature of KID syndrome, occurs in about three‐fourths of patients (1). Gram et al (15) suggest that these corneal changes may be a reflection of the generalized ectodermal disturbance, since the anterior portion of the cornea is similarly derived from the ectoderm. Development of keratitis has been reported to occur as late as the age of 2 years (1,10).…”