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Peeling Skin Syndrome Type A

PSS3


Presentation

  • Patients are usually in good general health but may present with mild failure to thrive.[orpha.net]
  • Expert reviewer(s): Dr Nathalie JONCA - Pr Juliette MAZEREEUW-HAUTIER - Last update: September 2013 The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Most of the type B PSS cases presented remarkably high serum IgE compared with the present case, suggesting that the relative mildness of symptoms and the relatively lower serum IgE level may correlate with less barrier function deficiency in our patient[karger.com]
  • Mild lymphocytic infiltrate was present in epidermis Click here to view PSS is a genodermatosis commonly affecting communities where consanguinity of marriage is present.[idoj.in]
  • In our case, the patient presented with generalized type of skin peeling disorder (type A) but with severe involvement of acral areas which is a rare presentation since acral involvement in generalized type is rarely present and is usually mild.[e-ijd.org]
Recurrent Infection
  • infection syndrome STAT3 Hyper-IgE recurrent infection syndrome, autosomal recessive DOCK8 Hypertrophic osteoarthropathy type 1 HPGD Hypertrophic osteoarthropathy type 2 SLCO2A1 Hypochondroplasia FGFR3 Hypophosphatemic rickets with hypercalciuria SLC34A3[centogene.com]
  • There is also a single case report of a patient with type B PSS with recurrent infections and IgA nephropathy. Treatment Acral PSS: There is no specific treatment for APSS, and current treatment is aimed at symptomatic relief.[dermatologyadvisor.com]
Failure to Thrive
  • Patients are usually in good general health but may present with mild failure to thrive.[orpha.net]
  • It is characterized by sparse, kinky hair; failure to thrive; and progressive deterioration of the nervous system.[checkrare.com]
  • Affected patients may present with failure to thrive and recurrent secondary infections (especially with Staphylococcus aureus).[dermatologyadvisor.com]
  • Whereas the surgical management for PA is straightforward, septicemia, electrolyte imbalance, protein loss and failure to thrive complicates the severe exudative skin lesions, often leading to death, and it has led to the recommendation that surgical[ijpd.in]
Hyperkeratosis
  • In contrast, our case showed neither stuck hyperkeratosis nor subcorneal cleavage, and the image was rather characterized by basket weave-type hyperkeratosis and possible shedding of the corneal sheet amid the stratum corneum.[karger.com]
  • Histological examination of skin lesion biopsies reveals a slight hyperkeratosis, thinning of the granular layer and a separation of the stratum corneum from the underlying stratum granulosum or an intracorneal split.[orpha.net]
  • Histological examination of skin lesion biopsies reveals acanthosis, hyperkeratosis, parakeratosis and extensive detachment of the stratum corneum from the subjacent stratum granulosum.[orpha.net]
  • A biopsy specimen shows hyperkeratosis and splitting of the epidermis between the granular layer and the stratum corneum. This chronic disease has no known effective established treatment.[scopemed.org]
  • There is a very marked variation in the number, size, shape, distribution and location of the lesions Over time the hyperkeratosis develops, which may be generalised or localised with well-defined, yellow-brown, thickened, rough, hyperkeratotic plaques[pcds.org.uk]
Skin Lesion
  • Histological examination of skin lesion biopsies reveals a slight hyperkeratosis, thinning of the granular layer and a separation of the stratum corneum from the underlying stratum granulosum or an intracorneal split.[orpha.net]
  • Histological examination of skin lesion biopsies reveals acanthosis, hyperkeratosis, parakeratosis and extensive detachment of the stratum corneum from the subjacent stratum granulosum.[orpha.net]
  • Keywords Stratum Corneum Granular Layer Congenital Ichthyosis Autosomal Reces Erythematous Skin Lesion These keywords were added by machine and not by the authors.[link.springer.com]
  • lesions and nonbilious vomiting since birth [Figure 1].[ijpd.in]
Koilonychia
  • Mild to moderate nail changes are sometimes reported (koilonychia, onycholysis, thickness, yellowish appearance).[orpha.net]
  • Sometimes light to moderate nail abnormalities (koilonychia, onycholysis, thickening, yellow color of the nail bed).[sites.uclouvain.be]
  • Disease associations have been reported and include fragile hair, koilonychia, onycholysis, chapping, keratoderma, sexual dysfunction, anosmia, short stature, primary amenorrhoea, cheilitis, keratosis pilaris, melanonychia, clubbing and hyperhidrosis.[casereports.bmj.com]
Angioedema
  • Atopic manifestations are frequent (e.g. food allergies, episodes of angioedema, urticaria and asthma) and Staphylococcus aureus skin infections are reported. Patients are usually in good general health but may present with mild failure to thrive.[orpha.net]
  • Atopic manifestations are frequent (food allergy, angioedema, urticaria and asthma) and Staphylococcus aureus infections have been reported. Patients are generally in good health but may present with growth retardation.[sites.uclouvain.be]
  • A review of the patient’s history will reveal atopy that can manifest as food allergies, severe pruritus, asthma, angioedema, and urticaria.[dermatologyadvisor.com]
Suggestibility
  • Diagnostic methods Clinical presentation is highly suggestive of the disease. Total IgE levels can be markedly elevated.[orpha.net]
  • Therefore, I suggest distinguishing between the peeling-skin syndrome, type A (cases similar to that of Fox) and the peeling-skin syndrome, type B (cases similar to those of Wile).[link.springer.com]
  • Peeling Skin Treatments - more information Peeling Skin - Frequently asked questions 4 Peeling Skin remedies suggested by our users Zinc for peeling skin suggested by [unspecified] on Monday, February 18, 2008 Natural exfoliant suggested by Corina on[home-remedies-for-you.com]
  • Two main PSS subtypes have been suggested.[uniprot.org]
Headache
  • Symptoms to watch out for with cellulitis are swelling, tenderness, bruising, blistering, headache, fever and skin that is reddened and warm to the touch over the affected area. Only a physician can correctly diagnose cellulitis.[reliawire.com]
  • Sudden fever, low blood pressure, chills, muscle aches, headache, vomiting, diarrhea, abdominal pain, dizziness, and confusion may occur.[healthline.com]

Workup

  • Keeping these differentials in mind further workup was done. Laboratory examination revealed leukocytosis, hyponatremia, and hypokalemia. Blood culture was negative.[ijpd.in]

Treatment

  • Management and treatment No effective treatment has been reported, especially for pruritus. Emollients are often used to reduce skin peeling.[orpha.net]
  • Management and treatment No effective treatment has been reported. Emollients are often used to reduce skin peeling. Prognosis Life expectancy is normal. No significant impairment in quality of life is reported.[orpha.net]
  • CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes.[diseaseinfosearch.org]
  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.de]
  • The disease can be life-threatening and needs treatment. Treatment usually requires a hospital stay, often in the burn or intensive care unit of the hospital. Treatment includes antibiotic medicine, replacing fluids, and skin care.[hopkinsmedicine.org]

Prognosis

  • Prognosis Life expectancy is normal. No significant impairment in quality of life is reported.[orpha.net]
  • Prognosis The severity of PSD is variable. Life expectancy is normal but the disease may severely impair the quality of life because of pruritus, lack of effective treatment and complications due to associated manifestations.[orpha.net]
  • With timely treatment, the prognosis is excellent. Treatment involves antibiotics given intravenously. Certain types of staphylococci bacteria secrete toxic substances that cause the top layer of the epidermis to split from the rest of the skin.[merckmanuals.com]
  • (Outcomes/Resolutions) The prognosis of Scalded Skin Syndrome in most children is excellent.[dovemed.com]

Etiology

  • Etiology A mutation in the CHST8 gene (19q13.1) encoding GalNAc4-ST1, a transmembrane sulfotransferase has been identified in one family.[orpha.net]
  • Etiology Mutations have been reported in the CDSN gene (6p21.3) encoding corneodesmosin, an adhesive protein specific to corneodesmosomes, the structures responsible for maintaining stratum corneum integrity.[orpha.net]
  • Etiology is still unknown with an autosomal recessive inheritance. Less than 100 cases have been reported in the medical literature. We present a 32-year-old man having asymptomatic peeling of skin since birth.[idoj.in]
  • (Etiology) Scalded Skin Syndrome is caused by the bacteria Staphylococcus aureus that produce a toxin causing the skin to peel-off and giving it a burnt appearance Staphylococcus aureus bacteria produce two types of toxins that may be Type A or ETA and[dovemed.com]

Epidemiology

  • Summary Epidemiology The prevalence is unknown.[orpha.net]
  • Summary Epidemiology The prevalence is unknown; the disease is rare with approximately 30 families reported in the literature to date. Clinical description Skin anomalies manifest at birth or shortly thereafter.[orpha.net]
  • As a result there is likely to be a spectrum of disease and there are likely to be a number of milder cases of adult SSSS that go undiagnosed. [ 5 ] Epidemiology [ 3 ] SSSS typically affects neonates and children aged under 5 years, but may also occur[patient.info]
  • Nosocomial outbreak of staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control. J Hosp Infect . 2005 Oct. 61(2):130-8. [Medline] . Ginsburg CM. Staphylococcal toxin syndromes.[emedicine.medscape.com]
  • Epidemiology • predominantly in infants and children younger than 5 years of age and rarely occurs in adults • Due to circulating antibodies and renal excretion of toxins • most cases are caused by type 71 strain (75%) • no differences in incidence based[de.slideshare.net]
Sex distribution
Age distribution

Pathophysiology

  • PATHOPHYSIOLOGY 7. PATHOPHYSIOLOGY 8. DIAGNOSIS • Gram stain or Culture: from the remote site of infection • Skin biopsy or Frozen Section • PCR 9. Management 10.[de.slideshare.net]

Prevention

  • Prevention Preventing sunburn in the first place is the best way to prevent the skin from peeling due to sunburn. One of the simplest ways to prevent sunburn is to apply an appropriate sunscreen before going outside, even on overcast days.[diseasesdic.com]
  • If care is not taken to prevent scratching, peeling skin could lead to further skin infections.[home-remedies-for-you.com]
  • […] hand-foot syndrome Follow these tips for up to a week after receiving chemotherapy treatment to help prevent leakage: Avoid prolonged heat exposure on hands and feet.[breastcancer.org]
  • Prevention and treatments The best way to prevent skin problems of this nature is to maintain a healthy diet. Make sure to consume all necessary nutrients in order to maintain healthy skin.[ic.steadyhealth.com]
  • IV fluids are also given to prevent dehydration. Much of the body's fluid is lost through open skin. Moist compresses to the skin may improve comfort. You can apply a moisturizing ointment to keep the skin moist.[medlineplus.gov]

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