PMC5958583_01
(a) Patient with dry, scaly skin seen all over the body. (b) Skin biopsy showing orthokeratosis, diminished granular layer with mild perivascular lymphocytic infiltrate in superficial dermis
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F1
On examination, skin was dry scaly all over the body [Figure 1a] with similar skin manifestation in his first cousins. Skin biopsy confirmed the diagnosis of IV [Figure 1b].