A Case of Ichthyosis Hystrix: Unusual Manifestation of This Rare Disease
A 46-year-old male patient presented with generalized severely thick skin since early childhood. According to his mother he was born normal and healthy. He gradually developed thickening of skin with adherent scales starting from face and progressively involving other parts of the body. None of the family members were affected. On examination, he had hyperkeratotic, thick, dry, adherent, mud-colored scales covering most of the body especially the face, scalp, and extremities including the flexures. Few areas had porcupine like spikes. Facial skin was so thickened and rugose that it became expressionless giving him a masked appearance []. His trunk was least involved with islands of normal skin []. Both, follicular prominence and atrophy were seen over the flexures of the forearm. He had associated nongradient palmoplantar keratoderma. Hairs were sparse and less over occipital area as it was covered with thick scale. He also had teeth and nail abnormality for the same duration. Most of the teeth were absent and canine was peg-shaped []. Subungual hyperkeratosis and prominent onychogryphosis were found in most of the nails []. There were no apparent ophthalmological or skeletal abnormalities, neither any hearing loss. His complete hemogram and chest X-ray were normal. On histopathology, we found orthokeratotic hyperkeratosis with sparse parakeratosis, irregular acanthosis, follicular plugging, and multiple binucleated keratinocytes in the epidermis along with upper dermal lymphocytic infiltrate, suggestive of ichthyosis [Figure and ]. On the basis of clinical features and histopathology, we arrived at the diagnosis of ichthyosis hystrix.