Ichthyosis (Xeroderma)

Any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. Most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome.

Also Known As:

Networked: 809 relevant articles (35 outcomes, 44 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Psoriasis (Pustulosis Palmaris et Plantaris)
2.	Acne Vulgaris
3.	Skin Diseases
4.	Lichen Planus (Lichen Ruber Planus)
5.	Lamellar Ichthyosis (Harlequin Ichthyosis)

Experts

1.	Fischer, Judith: 15 articles (01/2015 - 03/2006)
2.	Akiyama, Masashi: 13 articles (07/2015 - 11/2003)
3.	Traupe, Heiko: 12 articles (02/2014 - 02/2003)
4.	Vahlquist, Anders: 12 articles (04/2013 - 01/2003)
5.	Elias, Peter M: 9 articles (04/2015 - 04/2004)
6.	Oji, Vinzenz: 9 articles (02/2014 - 07/2006)
7.	Richard, Gabriele: 9 articles (11/2011 - 03/2003)
8.	Hausser, Ingrid: 8 articles (01/2015 - 04/2009)
9.	Sprecher, Eli: 8 articles (04/2011 - 04/2003)
10.	Shimizu, Hiroshi: 8 articles (10/2010 - 11/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ichthyosis:

1.	RetinoidsIBA 01/01/2013 - "Careful surveillance and management of mucous membrane, laboratory, skeletal, and teratogenic side effects has made systemic retinoids the mainstay of therapy for ichthyosis and related skin types." 01/01/2013 - "Systemic retinoids in the management of ichthyoses and related skin types." 11/01/2007 - "If an ichthyosis cannot be controlled by topical therapy alone, retinoids can be used. " 09/01/1986 - "[Retinoids in the treatment of ichthyosis]." 03/01/1984 - "These results demonstrate that (1) the synthetic retinoids hold promise as topical agents; (2) irritation is not an absolute requirement for topical retinoid activity; and (3) the diazacholesterol-fed mouse offers a new assay of topical retinoid potency in a well-defined animal model of ichthyosis."
2.	tazarotene (Tazorac)FDA Link 10/01/1999 - "Short-term topical application of tazarotene 0.05% gel is a very effective and well-tolerated treatment modality in different forms of congenital ichthyoses and may be an alternative to systemic retinoid therapy." 01/01/2006 - "[Topical tazarotene for severe congenital ichthyosis]." 10/01/1999 - "The clinical efficacy and tolerability of the topical receptor-selective retinoid tazarotene in the treatment of congenital ichthyoses was investigated. " 10/01/1999 - "Effect of topical tazarotene in the treatment of congenital ichthyoses." 05/01/2013 - "We describe a patient with recessive ichthyosis for whom daily application of topical tazarotene produced rapid and persistent improvement of bilateral lower eyelid ectropion without adverse effects. "
3.	Urea (Carbamide)FDA LinkGeneric 01/01/1998 - "Efficacy of urea therapy in children with ichthyosis. " 01/01/1998 - "The analysis of the global estimation of severity of ichthyosis showed improvements being stronger in the body areas treated with Laceran 10% urea lotion (from 4.8 to 2.0 points) than in the areas treated with the urea-free Laceran lotion base (from 4.8 to 2.5 points). " 01/01/1989 - "[Urea and urea combinations in ichthyoses]." 04/01/1982 - "The use of 25 percent urea in an emollient lotion appears to relieve the ichthyosis and, consequently, encourages the continued use of this drug." 12/01/1970 - "The treatment of ichthyosis and hyperkeratotic conditions with urea."
4.	EtretinateFDA Link 01/01/1985 - "The authors show the noteworthy efficacy of the aromatic retinoid Ro 10-9359 in the treatment of ichthyosis linearis circumflexa. " 01/01/1984 - "Light and electron microscopy study of ichthyosis and its evolution with oral etretinate treatment]." 10/01/1992 - "Bone changes and their significance in children with ichthyosis on long-term etretinate therapy." 01/01/1987 - "At 12, treatment with etretinate failed to improve the ichthyosis. " 01/01/1985 - "[Comèl's ichthyosis linearis circumflexa: treatment with Ro 10-9359. "
5.	Tacrolimus (Prograf)FDA LinkGeneric 03/01/2000 - "A case of ichthyosis linearis circumflexa successfully treated with topical tacrolimus." 05/01/2001 - "Case reports document efficacy in recalcitrant pyoderma gangrenosum, mucosal lichen planus, and ichthyosis linearis trolled trials and case reports indicate oral tacrolimus is effective in recalcitrant plaque psoriasis. " 01/01/2002 - "It has also been suggested that topical tacrolimus is useful in the treatment of alopecia areata, erosive lichen planus, isolated ichthyosis linearis circumflexa, steroid-induced rosacea, and in the prevention of allograft rejection, however more clinical trials need to be performed."
6.	Acquired ichthyosisIBA 06/01/2003 - "We report a case of acquired ichthyosis with original clinical features and we propose to coin the term "acquired circinated ichthyosis". " 08/01/1995 - "A few cases of acquired ichthyosis associated with SLE has been reported, and ichthyosis developed only in active stage of SLE. " 01/01/2009 - "Pityriasis rotunda is a disorder of keratinization, which is thought to be a form of acquired ichthyosis, a delayed presentation of congenital ichthyosis, or a cutaneous manifestation of systemic disease. " 01/01/2010 - "We report a 45-year-old woman with a several year history of dyspnea on exertion and panuveitis who presented to a county hospital with acquired lower extremity ichthyosis and a biopsy consistent with both acquired ichthyosis and noncaseating, granulomatous sarcoidosis. " 07/01/2010 - "We describe a child with acquired ichthyosis in whom an autoimmune hypothyroidism was detected; furthermore, replacement therapy with L-thyroxine resulted in complete remission of ichthyosis."
7.	EmollientsIBA 08/01/2002 - "At that time he also had widespread mild ichthyosis that cleared completely with the use of emollients. " 11/01/2000 - "The patient was treated with emollients (aquaphor) twice daily with continuing improvement in ichthyosis. " 01/01/2015 - "Like for other congenital ichthyoses, topical treatments (mainly emollients and keratolytics) are symptomatic and offer only temporary relief. " 01/01/2014 - "Controlling symptoms with emollients is the goal of treatment for ichthyosis since it has no cure. " 01/01/2013 - "A newborn with congenital ichthyosis cared for in our clinic failed his initial treatment of topical emollients. "
8.	calcipotriene (calcipotriol)FDA LinkGeneric 10/01/1994 - "We investigated the clinical efficacy of topically applied calcipotriol in six patients with congenital ichthyosis, using a double-blind, bilaterally paired, comparative approach. " 09/01/1997 - "Recent reports suggest that certain ichthyoses (particularly the hyperproliferative variants) and keratodermas may respond to topical calcipotriene ointment. " 05/01/1995 - "Short-term treatment with calcipotriol ointment (50 micrograms/g) used in amounts up to about 100 g/wk is moderately efficacious, well-tolerated, and safe in adult patients with various ichthyoses." 05/01/1995 - "At the end of the treatment regimen, calcipotriol ointment had an effect on the improvement of the ichthyoses, although to a variable degree. " 10/01/1994 - "Effect of topical calcipotriol on congenital ichthyoses."
9.	liarozoleIBA 05/01/2007 - "The results of this trial warrant further clinical trials to confirm efficacy and safety of liarozole as an orphan drug in ichthyosis." 03/01/2005 - "Topical liarozole in ichthyosis: a double-blind, left-right comparative study followed by a long-term open maintenance study." 01/01/1997 - "This open study showed that oral liarozole treatment was efficacious and well tolerated in the treatment of different types of ichthyosis. " 01/01/1997 - "Twelve male patients with ichthyosis were given oral liarozole, 150 mg twice daily, in an open study for 12 weeks. " 10/01/2001 - "Liarozole has already been found to be effective in the treatment of retinoid-responsive conditions such as chronic plaque psoriasis and ichthyoses. "
10.	Vitamin A (Retinol)FDA LinkGeneric 11/01/1953 - "[Electroencephalographic study of a case of congenital ichthyosis before and after vitamin A therapy]." 01/01/1990 - "The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. " 08/01/1980 - "A female patient with congenital ichthyosis took vitamin A in an uncontrolled manner throughout 3 years totalling 60 Mill. " 03/01/1973 - "[Local application of vitamin A in the treatment of congenital ichthyosis and inchtyosis connected to the sex of the patient]." 11/01/1971 - "[Histochemical changes in the skin of patients with ichthyosis during treatment with vitamin A]."

Therapies and Procedures

1.	Homologous Transplantation (Allograft) 01/01/2002 - "It has also been suggested that topical tacrolimus is useful in the treatment of alopecia areata, erosive lichen planus, isolated ichthyosis linearis circumflexa, steroid-induced rosacea, and in the prevention of allograft rejection, however more clinical trials need to be performed."
2.	Hydrotherapy (Whirlpool Bath) 11/01/2011 - "To assess the short- and medium-term efficacy of hydrotherapy on QoL and clinical symptoms of patients with inherited ichthyosis. " 11/01/2011 - "Short- and medium-term efficacy of specific hydrotherapy in inherited ichthyosis." 11/01/2011 - "In this 9-month prospective, open-label, multicentre study, 20 children and 24 adults with ichthyosis were enrolled in several French reference and competence centres, 2 months before undergoing a 3-week treatment with specific hydrotherapeutic management at Avène Hydrotherapy Centre. " 11/01/2011 - "A 3-week treatment at Avène Hydrotherapy Centre provided significant and persisting improvement of QoL and clinical symptoms in patients with inherited ichthyoses." 11/01/2011 - "At baseline (2 months before hydrotherapy), beginning (D0) and end of hydrotherapy (D18), and 3 and 6 months later at the reference and competence centres, patients self-assessed QoL using the Dermatology Life Quality Index (DLQI) or its paediatric version (Children's DLQI), and investigators evaluated ichthyosis severity using a specific clinical ichthyosis score. "
3.	Transplants (Transplant) 01/01/2010 - "Living related hemi-face skin transplant using radial forearm free flap for a xeroderma pigmentosa patient: early outcome." 06/01/2013 - "Owing to cholestasis, severe intractable pruritus, and poor quality of life, he underwent a living-related liver transplant from his mother, and his ichthyosis and pruritus dramatically improved. " 01/01/2008 - "Vitiligo, psoriasis, alopecia areata, contact hypersensitivity, lichen planus, pyoderma gangrenosum, ichthyosis linearis circumflexa, and skin grafting/transplant are a few unapproved indications and uses, in addition to miscellaneous dermatoses. " 02/01/1997 - "We analyzed DNA extracted from 39 keratoacanthomas from 22 "at-risk" patients (nine patients undergoing UV light and/or anthralin therapy for psoriasis, 10 solid organ transplant recipients, one patient with xeroderma pigmentosa, one patient with acquired immunodeficiency syndrome, and one patient undergoing therapy for non-Hodgkin's lymphoma) for the presence of HPV. "
4.	Curettage 04/01/1986 - "It is recommended that when ichthyosis uteri is detected the uterus should be removed, since the changes cannot be eliminated by curettage and there is a risk of malignant degeneration within a relatively short time." 01/01/2011 - "Here, we present two cases of ichthyosis uteri that were both diagnosed in the curettage material. "
5.	Cochlear Implantation 02/01/2012 - "Cochlear implantation in keratitis-ichthyosis-deafness syndrome: 10-year follow-up of two patients." 02/01/2010 - "A novel dominant and a de novo mutation in the GJB2 gene (connexin-26) cause keratitis-ichthyosis-deafness syndrome: implication for cochlear implantation." 06/01/2008 - "Cochlear implantation and connexin expression in the child with keratitis-ichthyosis-deafness syndrome." 02/01/2012 - "The objective of this study was to describe long-term outcomes after cochlear implantation in keratitis-ichthyosis-deafness (KID) syndrome, often caused by GJB2 mutations, in the context of other reported cases. " 09/01/2009 - "Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: outcomes in three cases."