A new neuroectodermal syndrome (designated CHIME syndrome) was described in 1983 with a total of four patients reported, it is presumed to be an autosomal recessive disorder because of recurrence in sibs. The main features include ocular colobomas, congenital heart disease, early onset migratory ichthyosiform dermatosis, mental retardation, conductive hearing loss, seizures, and typical facial ...

Background: Antiseptics are commonly used for personal hygiene in Taiwan. We have previously reported a series of ichthyosiform irritant contact dermatitis induced by cetrimide-containing antiseptics. Objectives: To report a new series of antiseptics-induced ichthyosiform irritant contact dermatitis and bring attention to the potential irritation effects of antiseptics. Methods: We reviewed the...

BACKGROUND In addition to the well-defined hereditary primary ichthyoses, many sporadic or less well-defined keratinization disorders with or without systemic manifestations have been reported. Herein we describe ichthyosiform dermatosis associated with type 2 diabetes mellitus. OBSERVATIONS The patients were members of a large Arab family with heavy consanguinity. Eighteen members were affec...

Background: Netherton syndrome is a rare autosomal recessive disorder consisting of ichthyosiform dermatosis, hair shaft abnormalities and an atopic diathesis that presents as widespread erythematous skin. The aim of these reports is emphasis on the importance of the examination of hair as a diagnose route. Case presentation: Case 1: A 6 months old boy with respiratory distress and severe eryth...

Hundreds of syndromes may be associated with hearing impairment. Within recent years, there has been heightened research and clinical interest in hearing and syndromes, probably due to both advances in molecular genetics and improved techniques for auditory assessment of infants and young children. An example of the current appreciation for the possible relation between auditory dysfunction and...

The term CHILD syndrome is proposed as an acronym for congenital hemidysplasia with ichthyosiform erythroderma and limb defects. The syndrome is characterized by unilateral erythema and scaling, with a distinct demarcation in the middle of the trunk. The dermatosis is either present at birth or develops during the first weeks of life. Ipsilateral limb defects may vary from hypoplasia of some fi...

Ichthyosiform mycosis fungoides (MF) is a rare variant of MF, occurring in 3.5% of MF patients according to one study (1, 2). Patients with ichthyosiform MF exhibit clinical manifestations similar to those of acquired ichthyosis; histologically, however, they show epidermotropism of atypical lymphocytes. Ichthyosiform MF is characterized by indolent course and usually good response to photother...

Acquired ichthyosis is a known paraneoplastic sign of lymphoproliferative malignancies with non‌specific histopathologic findings revealing no implication of the underlying neoplasm. However, ichthyosiform eruption is considered as a specific manifestation of mycosis fungoides (MF), i.e., ichthyosiform MF. There are a few reports of ichthyotic manifestation of MF, but only one report of t...

Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients hav...