BACKGROUND: Inherited ichthyoses belong to a large, clinically and etiologically
heterogeneous group of mendelian disorders of cornification, typically involving the entire …

Ichthyoses constitute a large group of cornification disorders that affect the entire
integument. The skin is characterized by visible scaling and in many cases by inflammation …

Ichthyoses are genetically determined Mendelian disorders of cornification (MEDOC) that
are characterized by universal scaling. Today we distinguish between non‐syndromic and …

Focal dermal hypoplasia (FDH) is an X-linked dominant multisystem birth defect affecting
tissues of ectodermal and mesodermal origin. Using a stepwise approach of (i) genetic …

Generalized peeling skin disease is an autosomal-recessive ichthyosiform erythroderma
characterized by lifelong patchy peeling of the skin. After genome-wide linkage analysis, we …

The barrier function of the human epidermis is supposed to be governed by lipid
composition and organization in the stratum corneum. Disorders of keratinization, namely …

Ichthyoses are a heterogeneous group of cornification disorders characterized by a
generalized scaling of the skin. Common types such as ichthyosis vulgaris and X-linked …

Uncombable hair syndrome (UHS), also known as" spun glass hair syndrome,"" pili trianguli
et canaliculi," or" cheveux incoiffables" is a rare anomaly of the hair shaft that occurs in …

The coexistence of psoriasis arthritis (PsA) and psoriasis vulgaris in about 20% of patients
with psoriasis leads to a need for rheumatologic-dermatologic team work. We summarise the …

Autosomal-recessive exfoliative ichthyosis presents shortly after birth as dry, scaly skin over
most of the body with coarse peeling of nonerythematous skin on the palms and soles …