We present a 25‐year‐old man from Iran with consanguineous parents, who presented with congenital erythroderma, hyperhidrosis and diffuse hyperkeratosis with coarse palmoplantar peeling of the skin, aggravated by exposure to water and by occlusion. [doi.org]

We present a 25-year-old man from Iran with consanguineous parents, who presented with congenital erythroderma, hyperhidrosis and diffuse hyperkeratosis with coarse palmoplantar peeling of the skin, aggravated by exposure to water and by occlusion. [ncbi.nlm.nih.gov]

At birth, patients present with generalized erythroderma, characterized by erythema and scaling, with or without edema over 90% of the skin [7]. Erosions, blisters, and peeling are typically present [7]. [austinpublishinggroup.com]

• Failure to Thrive

  Two month old female child presented with three cardinal features, arthrogryposis, renal tubular acidosis and cholestasis, along with failure to thrive, giant platelets and paucity of bile ducts on histopathology of liver. [casereports.in]

  […] to thrive, with a mortality of 30–40% during this period. [adc.bmj.com]

  GATA3 mutations (which traditionally associates with hypoparathyroidism and deafness syndomre – HDR -syndrome) have been identified in a patient with congenital ichthyosis, hypoparathyroidism, and failure to thrive who later developed sensorineural hearing [bredagenetics.com]

  The systemic signs revealed dehydration (42.6%), fever (34.0%), tachycardia (29.8%), failure to thrive (29.8%), hepatomegaly (17.0%), edema (12.8%), and splenomegaly (6.4%). [astrocyte.in]

  Affected patients may present with failure to thrive and recurrent secondary infections (especially with Staphylococcus aureus). [dermatologyadvisor.com]

• Hepatomegaly

  Hepatomegaly with span 9 cm was noted and rest of systemic examination was normal. [casereports.in]

  Lymph node enlargement, splenomegaly, and hepatomegaly were found in 75%, 66.7%, and 62.5% of immunodeficiency syndrome cases, respectively. [astrocyte.in]

  Other clinical findings include lymphadenopathy, hepatomegaly, splenomegaly, edema of the foot or ankle 4, 6 and gynecomastia. 19 The scaling that occurs in exfoliative dermatitis can have severe metabolic consequences, depending on the intensity and [aafp.org]

• Sweating

  Overview Anhidrosis is the inability to sweat normally. When you don't sweat (perspire), your body can't cool itself, which can lead to overheating and sometimes to heatstroke — a potentially fatal condition. [mayoclinic.org]

  They may have the following problems: overheating – as a result of a reduced ability to sweat limited movement – dry skin can make it too painful to move certain parts of the body skin infection – after cracking and splitting of the skin impaired hearing [nhs.uk]

  The skin is fragile and is prone to blistering (caused by mild trauma or sweating).[6] After a few months hyperkeratosis develops with a dark grey or brown, ridged appearance on the ankles, knees and elbows.[4] Palms and soles are generally unaffected [wikidoc.org]

  At some point during their life people with severe ichthyosis may come against some of the following problems: Overheating: ichthyosis may affect normal temperature control by reducing the ability to sweat Limited movement: dry, scaly skin may make it [dermnetnz.org]

  Scattered throughout the middle layers are hair follicles, oil and sweat glands, and nerve endings. The upper layer is constantly flaking off and being replaced from beneath by new tissue. [encyclopedia.com]

• Corneal Opacity

  X-linked Ichthyosis : as like IV but a consultation with ophthalmologist (for corneal opacity) and surgeon (for cryptorchidism) is needed. [slideshare.net]

  opacities in 50% Only affects males Undescended testicles in 5–20% Autosomal recessive congenital ichthyosis -1 (ARCI1) The baby is encased by collodion membrane at birth, which cracks and is then shed; 10% are self-healing Harlequin ichthyosis : severe [dermnetnz.org]

• Burning Sensation

  Patients may complain of erythema, pruritus, or a burning sensation in the exposed denuded area. The history is not significant for systemic manifestations or atopy. [dermatologyadvisor.com]

Oncology is a very rapidly changing speciality, with many new treatments and even treatment modalities coming on stream with bewildering speed. [books.google.com]

Your response to the treatment; iii. Any adverse effects of the treatment; and iv. The expected duration of the treatment. b. [ssa.gov]

[…] urea treatment performing better than glycerol-based treatments ( a commonly-recommended ingredient to treat ichthyosis). [viabuff.com]

Anti-drug antibodies in psoriasis: a critical evaluation of clinical significance and impact on treatment response. Expert Rev Clin Immunol. 2013 Oct. 9(10):949-58. [Medline]. [emedicine.medscape.com]

Ionizing Radiation Electromagnetic Radiation Water Toxicity Sewage Treatment. 0.0 (0) Download. Embed. Description:.Thrombophlebitis Allergie. [ifuxorecylaxynof.xpg.uol.com.br]

Overall prognosis was poor in patients with immunodeficiency. [astrocyte.in]

PMID: 12383092 Prognosis Pigors M, Sarig O, Heinz L, Plagnol V, Fischer J, Mohamad J, Malchin N, Rajpopat S, Kharfi M, Lestringant GG, Sprecher E, Kelsell DP, Blaydon DC Am J Hum Genet 2016 Aug 4;99(2):430-6. [ncbi.nlm.nih.gov]

The prognosis of this disorder is extremely poor, as patients die in infancy. Most deaths are due to recurrent infection, acidosis and critical bleeding [ 2 ]. [casereports.in]

Clinical Course, Prognosis and Sequelae Even though exfoliative dermatitis is a complex disorder involving many factors, the underlying disease is usually the key determinant of the course and prognosis. [aafp.org]

Age of onset primarily is related to etiology. [33, 34] Prognosis The prognosis of exfoliative dermatitis depends largely on underlying etiology. [emedicine.medscape.com]

No other clinical or laboratory findings were associated with any specific etiology. [astrocyte.in]

Describe the presentation and etiology of epidermolytic hyperkeratosis. Identify and describe the two major autosomal recessive primary ichthyoses and their etiologies. the primary technique for dry skin care. [pedsinreview.aappublications.org]

Age of onset primarily is related to etiology. [33, 34] Prognosis The prognosis of exfoliative dermatitis depends largely on underlying etiology. [emedicine.medscape.com]

Although the etiology is often unknown, exfoliative dermatitis may be the result of a drug reaction or an underlying malignancy. [aafp.org]

PMID: 19615015 Etiology Seidl-Philipp M, Schatz UA, Gasslitter I, Moosbrugger-Martinz V, Blunder S, Schossig AS, Zschocke J, Schmuth M, Gruber R J Dtsch Dermatol Ges 2020 Jan;18(1):17-25. Epub 2019 Oct 23 doi: 10.1111/ddg.13968. [ncbi.nlm.nih.gov]

Synonym(s): - Autosomal recessive exfoliative ichthyosis - Ichthyosis exfoliativa Classification (Orphanet): - Rare genetic disease - Rare skin disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]

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

Strontium ranelate [29] Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfisoxazole Sulfonamides Sulfonylureas Sulindac Terbinafine [30] Tetracycline Tobramycin Tocilizumab [31] Trazodone Trifluoperazine Trimethoprim Vancomycin Verapamil Warfarin [32] Epidemiology [emedicine.medscape.com]

Pathophysiology An increased skin blood perfusion occurs in exfoliative dermatitis (ED) that results in temperature dysregulation (resulting in heat loss and hypothermia) and possible high-output cardiac failure. [emedicine.medscape.com]

Prevention Anhidrosis often can't be prevented, but serious heat-related illnesses can. To stay safe: Wear loose, light clothing when it's warm. Stay cool indoors on hot days. Use a spray bottle containing water to cool yourself. [mayoclinic.org]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

These listings are only examples of common skin disorders that we consider severe enough to prevent you from engaging in any gainful activity. [ssa.gov]

Increased humidity of the ambient air is also helpful in preventing skin dryness. [encyclopedia.com]