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Disorders of keratinization and other genodermatoses
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
The disease is characterized by scaling, which is prominent over extensors, particularly over the shins (Figure 15.2). The scales here are often dark-colored and polygonal in shape. A fine white scaling is usually present over other areas of the body, like the trunk. The flexures are mostly spared. The affected individuals often have hyperlinear palms, thickening of the skin (keratoderma) of palms and soles, and keratosis pilaris (Figure 15.3). Asthma, atopic dermatitis, and other manifestations of atopy are frequently associated. Symptoms are worse during the winter when the climate is dry.
The Ichthyosis (ic) Mutation, Chromosome 1
Published in John P. Sundberg, Handbook of Mouse Mutations with Skin and Hair Abnormalities, 2020
John P. Sundberg, Mark R. Pittelkow
The annular constrictions of the tail resemble ainhum, a human disease in which a narrow strip of hardened skin forms a constricting ring on the little toe at the level of the digitoplantar fold and progresses to spontaneous amputation of the digit.21 Forms of this disease are familial and considered as subtypes of restrictive keratodermas.22–24
Tylosis with Esophageal Cancer
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Palmoplantar keratoderma (hyperkeratosis palmaris et plantaris) is a focal thickening of the skin that often appears as areas of yellowish thickened plaques restricted to areas of weight bearing and/or friction on the palms and soles (e.g., the heels and forefeet). Occasionally, it may be confluent over the palms and soles. It usually emerges by 7–8 years of age but may occur as late as puberty. Other complications include discomfort, pruritus, deep and painful fissures, infection, follicular papules, and cutaneous horns [1].
Managing pediatric psoriasis: update on treatments and challenges—a review
Published in Journal of Dermatological Treatment, 2022
A. A. Hebert, J. Browning, P. C. Kwong, A. M. Duarte, H. N. Price, E. Siegfried
Other, distinct phenotypes can occur at any age, including skin-fold (“inverse”), palmoplantar, and pustular PsO. Some phenotypes, such as napkin and inverse PsO, are more common in infants than in children and adolescents (29). A phenotype with features of both PsO and eczema often referred to as “psoriasiform dermatitis” or “overlap,” may be more common in children than adults (30). Rare forms of severe, early-onset skin disease with pustular PsO-like features, osseous involvement, and autoinflammatory morbidities have been associated with specific mutations in IL1RN and referred to as deficiency of the IL-1 receptor antagonist, or DIRA. Deficiency of the IL-36 receptor antagonist, or DITRA, is a similar condition but lacks bony involvement and is linked to mutations in IL36RN (31,32). An atypical psoriatic eruption featuring facial involvement and palmoplantar keratoderma is associated with a mutation in CARD14 (33). Recognition of these genotypes and treatment with targeted biologic therapy can result in dramatic clearing (34,35).
Naxos disease – a narrative review
Published in Expert Review of Cardiovascular Therapy, 2020
Marianna Leopoulou, Gustav Mattsson, Jo Ann LeQuang, Joseph V Pergolizzi, Giustino Varrassi, Marita Wallhagen, Peter Magnusson
The Naxos disease phenotype is categorized into cardiac manifestations and extracardiac characteristics. Typically, woolly, rough, dull hair that was apparent from birth [9], and diffuse non-epidermolytic palmoplantar keratoderma, which developed during the first year of life, as soon as the child started using hands and feet, were present in all patients [9]. Some patients present with short fingers, curved nails, and small arms and hands [2]. In more detail, patients’ lesions were described as tight woolly hair and diffuse palmoplantar keratosis, occasionally erythematous, not extending to the dorsal area. Furthermore, those lesions are reported to have clear borders [14]. Hypo/oligodontia has also been reported in association with the phenotype of woolly hair, keratoderma, and cardiomyopathy [15].
Punctate porokeratosis—pruritic and hyperkeratotic papules on the palms and feet
Published in Baylor University Medical Center Proceedings, 2020
Patrick Michael Jedlowski, Gina Rainwater, So Yeon Paek
Other clinical entities may mimic PP, including keratosis puncta of the palmar creases, arsenical keratosis, and nevus comedonicus. Keratosis puncta of the palmar creases is a benign condition and variant of punctate keratoderma that occurs most commonly in African American patients. It is typified by hyperkeratotic pits limited to the palmar creases, as opposed to the diffuse palmoplantar lesions in our patient. Arsenical keratosis occurs due to chronic ingestion of arsenic most commonly via contaminated well water and presents with pigmentary changes of truncal skin and mucous membranes, palmoplantar keratosis, and Mee’s lines of the nails.9 Arsenical palmoplantar keratoses range in clinical appearance from indurated, gritty millimeter palmoplantar papules to yellow, verrucous papules and plaques.9 On histology, arsenical keratosis is characterized by compact hyperkeratosis and a thickened stratum granulosum but lacks the columns of parakeratosis seen in our case.9 Nevus comedonicus is a hamartomatous growth of the pilosebaceous unit distinguished by dilated follicles with pigmented, keratinaceous plugs and histopathology displaying epidermal invagination, hyperkeratosis and follicular plugging, which is not seen in this case.