Hypoplastic Nails

Ichthyosiform 2016 Newborn

Figure 1.230. The feet of the same infant as in Figure 1.228 and 1.229 show the thick, rigid, hard, cracked skin and poorly developed toes with hypoplastic nails.

Hypoplastic Nails

Figure 1.231. The CHILD syndrome is an X-linked dominant disorder (lethal to males) which is characterized by congenital Kemidysplasia, with ichthyosiform erythroderma, and iimb defects. The hallmark of the disorder is the sharp mid-line demarcation and the ipsilateral involvement of the skin and the extremities. The face is spared, and limb defects range in severity from hypoplasia of digits to complete agenesis of an extremity. In addition to the dermal and musculoskele-tal involvement, other organs may be abnormal (viscera and occasionally the central nervous system).

Figure 1.231. The CHILD syndrome is an X-linked dominant disorder (lethal to males) which is characterized by congenital Kemidysplasia, with ichthyosiform erythroderma, and iimb defects. The hallmark of the disorder is the sharp mid-line demarcation and the ipsilateral involvement of the skin and the extremities. The face is spared, and limb defects range in severity from hypoplasia of digits to complete agenesis of an extremity. In addition to the dermal and musculoskele-tal involvement, other organs may be abnormal (viscera and occasionally the central nervous system).

Figure 1.232. Following a spontaneous vaginal delivery in this term infant, desquamation of the skin was noted with the usual drying off in the delivery room. No bullae or blisters were noted. There was superficial desquamation over about 70% of the total body surface area. The Nikolsky's sign was positive. The diagnosis of "peeling skin syndrome" was confirmed by skin biopsy. This is an unusual congenital ichthyosis which is probably an autosomal recessive disease characterized by lifelong peeling of the epidermis with easy separation of the stratum corneum.

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Figure 1.233. Another view of the same infant as in Figure 1.232 with the "peeling skin syndrome." This condition must be differentiated from non-bullous ichdiyosiform erythroderma. Histopathologic features include intraepidermal separation between the stratum corneum and stratum granulosum. There may be a biochemical marker of moderate generalized aminoaciduria and low tryptophan levels.

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Responses

  • reagan
    What is hypoplastic nails?
    7 years ago
  • phillipp
    What is cracked skin on the toes?
    7 years ago

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