Non-bullous congenital ichthyosiform erythroderma characterized by fine whitish scaling on an erythrodermal background; larger brownish scales are present on the buttocks, neck and legs. [ read more ] 22246504 [nectarmutation.org]

Non-bullous congenital ichthyosiform erythroderma characterized by fine whitish scaling on an erythrodermal background; larger brownish scales are present on the buttocks, neck and legs. [uniprot.org]

Case Presentation A 47-year-old Caucasian male presented to the emergency department (ED) for diffuse erythema and pruritus of three weeks’ duration. [cureus.com]

The timing of onset is perhaps the most important feature, as erythroderma present at birth has fewer potential causes than later presentation. [dovepress.com]

Table 2 shows the comparison of previous studies with present study for etiology of erythroderma. [ncbi.nlm.nih.gov]

• Sepsis

  A female patient, born as a collodion baby with MRSA sepsis and bilateral pneumonia, was described. She exhibited generalized thick dry skin with ichthyosis on the soles, but by the age of 10 months, her skin condition greatly improved. [cags.org.ae]

  Finally, systemic complications of erythroderma include cutaneous infection, sepsis, fluid and electrolyte dysregulation, thermoregulatory problems and high output cardiac failure due to shunting large volumes of blood to the skin [3,9]. [cureus.com]

  Most erythrodermic infants receive an initial sepsis evaluation and empiric treatment with antimicrobial medications. [dovepress.com]

  Dermatoses Atopic Dermatitis Psoriasis Pityriasis Rubra Pilaris Cutaneous T Cell Lymphoma Blistering Diseases Pemphigus and variants: Bullous Pemphigoid Acute or Chronic Contact Dermatitis Ichthyoses Infections Dermatophyte Scabies Deep fungal Infections HIV Sepsis [cancertherapyadvisor.com]

• Short Stature

  Most common symptoms of CONGENITAL NON-BULLOUS ICHTHYOSIFORM ERYTHRODERMA Short stature Hearing impairment Failure to thrive Alopecia Pruritus More info about CONGENITAL NON-BULLOUS ICHTHYOSIFORM ERYTHRODERMA SOURCES: UMLS ORPHANET ICHTHYOSIS, CONGENITAL [mendelian.co]

  […] finger Stubby finger Stubby fingers [ more ] 0009381 Short stature Decreased body height Small stature [ more ] 0004322 Short toe Short toes Stubby toes [ more ] 0001831 Small nail Small nails 0001792 Thin nail Thin nails 0001816 Percent of people who [rarediseases.info.nih.gov]

  […] activity causes short stature bone weakening in adults due to lack of vitamin D, prone to vertebral and femoral fractures markedly decreased bone mass, prone to fractures from mild stress osteoclast dysfunction "marble bone disease", brittle bones due [quizlet.com]

• Malaise

  Commonest symptoms were generalized scaling (100%), redness (100%), chills (93.3%), malaise (93.3%) and itching (86.6%). Fever was present in 43.3% of cases and 26.6% had oliguria. None of the patients had dermatogenic enteropathy. [ncbi.nlm.nih.gov]

• Nausea

  […] osteoporosis drugs must decrease incidence of vertebral fractures approved for post-menopausal osteoporosis treatment [5 years beyond] dosed intranasally and IM/SQ reserved as alternate for women who cannot take other agents rhinitis, rarely epistaxis, nausea [quizlet.com]

• Hearing Impairment

  impairment Deafness Hearing defect [ more ] 0000365 Keratitis Corneal inflammation 0000491 Palmoplantar keratoderma 0000982 5%-29% of people have these symptoms Erythema 0010783 Everted lower lip vermilion Drooping lower lip Outward turned lower lip [rarediseases.info.nih.gov]

  Most common symptoms of CONGENITAL NON-BULLOUS ICHTHYOSIFORM ERYTHRODERMA Short stature Hearing impairment Failure to thrive Alopecia Pruritus More info about CONGENITAL NON-BULLOUS ICHTHYOSIFORM ERYTHRODERMA SOURCES: UMLS ORPHANET ICHTHYOSIS, CONGENITAL [mendelian.co]

• Alopecia

  In classical CIE (unlike LI) there is little eclabion (eversion of the lips), ectropion and alopecia (hair loss). Many people with ACRI don't fit neatly into the definition of LI or CIE but have characteristics of both diseases. [en.wikipediam.org]

  Some affected persons exhibit scarring alopecia, and many have secondary anhidrosis (summary by Eckl et al., 2005 ). [mendelian.co]

  Some affected persons exhibit scarring alopecia, and many have secondary anhidrosis (summary by {14:Eckl et al., 2005}). [diseaseinfosearch.org]

• Erythema

  Conclusions Erythroderma is defined clinically as generalized erythema and scaling of the skin. [cureus.com]

  In LI, the scales are large, adherent, dark, and pigmented with no skin erythema. Overlapping phenotypes may depend on the age of the patient and the region of the body. [diseaseinfosearch.org]

  Erythema may be very mild and almost\ninvisible. Some affected persons exhibit scarring alopecia, and many\nhave secondary anhidrosis (summary by Eckl et al., 2005). [lifemodules.org]

  Erythema appeared first followed by scaling over a period of 4-5 days. In acute cases, scales were large and easily detachable and in chronic cases, they were of smaller size. [ncbi.nlm.nih.gov]

  When acute, the patient lay look like a toxic erythema or even like an intense sunburn. [cancertherapyadvisor.com]

• Pruritus

  Case Presentation A 47-year-old Caucasian male presented to the emergency department (ED) for diffuse erythema and pruritus of three weeks’ duration. [cureus.com]

  In addition to erythroderma, other co-existent features included pruritus, fever, lymphadenopathy, and edema. [ncbi.nlm.nih.gov]

  […] frequent symptoms Patients with TGM1 gene alterations may also develop some of the following symptoms and phenotypes: Not very common - Between 30% and 50% cases Short stature Dry skin Hyperkeratosis Everted lower lip vermilion Abnormality of the nail Pruritus [mendelian.co]

  Showing of 29 | 80%-99% of people have these symptoms Ectropion Eyelid turned out 0000656 Erythroderma 0001019 Hypohidrosis Decreased ability to sweat Decreased sweating Sweating, decreased [ more ] 0000966 Ichthyosis 0008064 Pruritus Itching Itchy skin [rarediseases.info.nih.gov]

• Hypertrichosis

  The most common skin paraneoplastic manifestations are acanthosis nigricans, erythromelalgia, thrombotic thrombocytopenic purpura, acrokeratosis paraneoplastica, paraneoplastic hypertrichosis lanuginosa acquisita, dermatomyositis, systemic sclerosis, [ncbi.nlm.nih.gov]

• Decrease in Height

  Showing of 29 | 80%-99% of people have these symptoms Ectropion Eyelid turned out 0000656 Erythroderma 0001019 Hypohidrosis Decreased ability to sweat Decreased sweating Sweating, decreased [ more ] 0000966 Ichthyosis 0008064 Pruritus Itching Itchy skin [rarediseases.info.nih.gov]

• Confusion

  The distinctive clinical featuresof harlequin ichthyosis (HI) (see below) are unlikelyto be confused with the less severe appearance ofthe collodion baby, but a small proportion ofaffected neonates fall between the two presentations.These have been referred [docslide.com.br]

  […] and pelvic flat bones histologically tumor looks like lots of lymphocytes, stains with CD99 antibody many multinucleated giant cells contain > 40 nuclei malignant soft tissue tumor that resembles skeletal muscle [it really doesn't but whatever] [dont' confuse [quizlet.com]

Once the erythroderma is being managed, further workup for underlying pathology is imperative as the therapies vary depending on the underlying disease processes. B. Anticipated length of Stay. [cancertherapyadvisor.com]

Initial diagnostic workup is included in Table 2. Figure 5 Algorithm for diagnosis of the erythrodermic infant. [dovepress.com]

Hopefully, RAMBAs will be further developed as orphan drugs for treatment of moderate-to-severe LI. An additional possibility is that anti-inflammatory agents might prove effective in the treatment of ARCI. [docslide.net]

All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder. [firstskinfoundation.org]

You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice . [uniprot.org]

Treatment is highly dependent on underlying etiology but in cases where the underlying cause remains elusive empiric treatment can be considered with agents such as systemic corticosteroids, methotrexate, cyclosporine or mycophenolate mofetil. [cureus.com]

Prognosis and Patient Counseling. Prognosis is good but depends somewhat on underlying disease processes. VI. Patient Safety and Quality Measures. A. Core Indicator Standards and Documentation. NA B. [cancertherapyadvisor.com]

Conflicting results have been published regarding the prognosis of patients with erythroderma. On follow-up, we found six deaths (5.8%) related to the underlying causes of erythroderma, all of them from progression of the CTCL. [ncbi.nlm.nih.gov]

A severe burn-like erythema has been described in this group and is associated with a worse prognosis. [dovepress.com]

In 16.6% of cases, etiology could not be ascertained. Clinico-histopathological correlation could be established in 73.3% of cases. Conclusion: Clinical features were identical irrespective of etiology. [ncbi.nlm.nih.gov]

Etiology is certainly of interest with erythroderma because the differential is extensive. [cureus.com]

Elimination of infectious etiologies and the temporal association of new drug ingestion, and absence of hypotension would support this etiology. [cancertherapyadvisor.com]

[…] are rarer etiologies, but important to recognize early to prevent harmful sequelae. [dovepress.com]

Relevant External Links for ALOX12B Genetic Association Database (GAD) ALOX12B Human Genome Epidemiology (HuGE) Navigator ALOX12B Atlas of Genetics and Cytogenetics in Oncology and Haematology: ALOX12B No data available for Genatlas for ALOX12B Gene A [genecards.org]

It is considered a rare disease with few published studies concerning epidemiology [5]. Etiology is certainly of interest with erythroderma because the differential is extensive. [cureus.com]

Von Hebra first described generalized cutaneous inflammation in 1868 [ 3 ].The incidence of erythroderma is high in the Indian subcontinent although epidemiological data on a global scale are lacking. [ncbi.nlm.nih.gov]

The human 12R-LOX product, 12R-HETE, appears to be involved in the pathophysiology of psoriasis and other proliferative skin diseases ; a role in normal skin development is implied from the spatial and temporal expression patterns of the 12R-LOX in the [wikigenes.org]

Erythrodermic psoriasis: pathophysiology and current treatment perspectives. Psoriasis. 2016, 6:93-104. 10.2147/ptt.s101232 Tan GF, Kong YL, Tan AS, Tey HL: Causes and features of erythroderma. Ann Acad Med Singapore. 2014, 43:391-394. [cureus.com]

Pathophysiologic basis for growth failure in children with ichthyosis: an evaluation of cutaneous ultrastructure, epidermal permeability barrier function, and energy expenditure. J Pediatr. 2004;145(1):82–92. 45. Taïeb A, Labrèze C. [dovepress.com]

Gene mutations impair the respective protein's function or structure within the epidermis, which prevents this outermost layer of skin from being an effective barrier before and after birth. [ghr.nlm.nih.gov]

Interleu-kin-1 alpha blockade prevents hyperkeratosis in an invitro model of lamellar ichthyosis. Hum Mol Genet2010;19:25942605.546 Pediatric Dermatology Vol. 31 No. 5 September/October 2014 [documents.tips]

[…] increased risk of breast and uterine cancer associated with estrogen does not prevent hot flashes, same VTE risk as estrogen 2nd line bc only decreases vertebral fractures bisphosphonates available (4) alendronate, risendronate, ibandronate, zoledronic [quizlet.com]

Topical ointment for preventing infection in preterm infants. Cochrane Database Syst Rev. 2004;(1):CD001150. 47. Gastmeier P, Groneberg K, Weist K, Rüden H. [dovepress.com]