PMC4320795_01
Clinical appearance and dermatologic findings of the patient. Note the alopecia with an absence of scalp hair, eyebrows, and lashes (a). Spiky follicular hyperkeratosis on the trunk. Skin was dry and scaly and erythematous follicular papules were observed over posterior aspect of the neck and groin ((c), (d), and (e)), cheilitis (b), and dystrophic nails (f).
CRIM2015-450937.001.jpg
PMC4320795_01_CRIM2015-450937.001.jpg
fig1
The patient kept widespread severe itching and exudative eczema mainly in the posterior aspect of the neck and groin with great interference in sleep (Figure 1).
A skin biopsy from left thigh performed at 12 months showed epidermis with irregular acanthosis and orthokeratotic lamellar hyperkeratosis (a). The hair follicles demonstrate absence of hair shaft and are fulfilled by parakeratotic hyperkeratosis (b).
CRIM2015-450937.002.jpg
PMC4320795_01_CRIM2015-450937.002.jpg
fig2
A new skin biopsy from left thigh performed at 12 months showed epidermis with irregular acanthosis and orthokeratotic lamellar hyperkeratosis (Figure 2).