Edit concept Question Editor Create issue ticket

Epidermolysis Bullosa Pruriginosa

Epidermolysis Bullosa Prurigia


Presentation

  • We present one family with both dystrophic and pruriginosa phenotypes of epidermolysis bullosa.[ncbi.nlm.nih.gov]
Lichenification
  • Epidermolysis bullosa pruriginosa Clinical features : Imported from Human Phenotype Ontology (HPO) Abnormality of connective tissue Atypical scarring of skin Abnormality of the integument Abnormality of the nail Atypical scarring of skin Fragile skin Lichenification[familydiagnosis.com]
  • The rarity of intact blisters, prominent nature of some of the scars and the marked lichenification with severe pruritus lead to a confusion with commoner disorders, like lichen simplex chronicus, lichen planus hypertrophicus and dermatitis artefacta.[e-ijd.org]
  • Histological features included subepidermal blistering, vascular proliferation, subtle fibrosis, milia, marked lichenification, and mild to moderate perivascular lymphocytic infiltrates.[jamanetwork.com]
Normal Hair
  • hair and mucosa with the histopathology revealing a subepidermal cleft.[e-ijd.org]
Pruritic Rash
  • Case report A 53-year-old Singaporean Chinese first presented to the National Skin Centre, Singapore, 14 years ago with severely pruritic rashes on the lower limbs.[ijdvl.com]
Thin Skin
  • […] pruriginosa Clinical features : Imported from Human Phenotype Ontology (HPO) Abnormality of connective tissue Atypical scarring of skin Abnormality of the integument Abnormality of the nail Atypical scarring of skin Fragile skin Lichenification Milia Pruritus Thin[familydiagnosis.com]
Denial
  • […] site of hand and foot after brith.The frictional vesicle and bulla diminished gradully with age, but papules and nodules were noted on his shins,dorsa,and extremities with severe pruritus.The same condition was formed in this mother and relatives.with denial[globethesis.com]
Compulsive Disorder
  • Psychological examination revealed no psychopathological base consistent with self-inflicted compulsive disorders that the patient denied (dermatitis artefacta) or admitted (automutilation). Fig. 1. Both patients seen at initial consultation.[medicaljournals.se]
Incontinence
  • Pigment incontinence was noted. Histological features were suggestive of a noninflammatory subepidermal bullous disorder favoring a diagnosis of DEB.[ijpd.in]

Treatment

  • C. 2013 48 A combination of nonoperative treatment modalities used for treatment of keloids Yan, Daojin / Zhao, Bocheng / Yang, Haiping / Zhu, Bin / Wang, Jing 2013 52 Multiple cutaneous malignancies and cherry hemangiomas in a vitiligo patient treated[tib.eu]
  • Treatment Treatment ** The aims of treatment are : 1- Reducing pruritus 2- Preventing scarring 3- Improving the patient’s quality of life. ** The most frequently used treatments for EBP are potent topical steroids under occlusion, or intralesional steroids[interactivederma.com]

Prognosis

  • The prognosis was explained to the patient and he was given symptomatic treatment.[e-ijd.org]
  • Prognosis Prognosis varies considerably and is based on both EB subtype and the overall health of the patient. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Prognosis. Generally, the prognosis in EBS is good, particularly in the common localized type, with the great majority of patients having a normal life expectancy.[plasticsurgerykey.com]
  • . • Bistering in mouth is mild and teeth normal• Perianal lesions – intense pain• Clinically often impossible to distinguish from Dominant DEB• Good long term prognosis 50.[slideshare.net]
  • Prognosis varies considerably and is based on both EB subtype and the overall health of the patient.[ojrd.biomedcentral.com]

Etiology

  • Folliculitis decalvans (FD) is a primary neutrophilic scarring alopecia of unclear etiology affecting the scalp of young adults [ 3 ].[karger.com]
  • Etiology Each EB subtype is known to arise from mutations within the genes coding for several different proteins, each of which is intimately involved in the maintenance of keratinocyte structural stability or adhesion of the keratinocyte to the underlying[orpha.net]
  • ( J Clin Invest 2009;119:1784 ) Junctional: blisters within lamina lucida, skin appears normal Dystrophic: blisters are sub-lamina densa; dominant or recessive Kindler syndrome: blisters at multiple levels (intra-lamina lucida and sub-lamina densa) Etiology[pathologyoutlines.com]
  • […] is characterized by generalized erosions, palmoplantar keratoderma, painful fissures, and some nail dystrophy and hair loss. [5] Molecular pathology of junctional epidermolysis bullosa Junctional epidermolysis bullosa has a highly variable molecular etiology[emedicine.medscape.com]

Epidemiology

  • Source: Journal of Investigative Dermatology - April 20, 2019 Category: Dermatology Authors: Tags: Epidemiology Source Type: research We report long term follow-up results of a single-center phase 1/2a study of 7 RDEB patients who underwent grafting of[medworm.com]
  • Summary Epidemiology All types and subtypes of EB are rare; the overall incidence and prevalence of the disease in the United States are approximately 1/53,000 live births and 1/125,000, respectively, and similar estimates have been obtained in some European[orpha.net]
  • Google Scholar Fine JD: Epidemiology and the study of genetic diseases. Edited by: Grob JJ, MacKie R, Stern R, Weinstock M. Epidemiology and prevention of skin diseases London: Blackwell Science; 1996.[ojrd.biomedcentral.com]
  • Fine JD (1999) The epidemiology of inherited EB: findings within American, Canadian and European study populations.[link.springer.com]
  • The epidemiology of inherited epidermolysis bullosa. Findings in US, Canadian, and European study populations. In: Fine JD, Bauer EA, McGuire J, Moshell A, editors.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of EB pruriginosa remains unexplained.[ncbi.nlm.nih.gov]
  • […] have been investigated, but thusfar no specific insight into EB-Pr has emerged.2 Also, meta-bolic factors, including immunoglobulin E levels, iron defi-ciency and biochemical or endocrinological abnormalities,have been studied but do not explain the pathophysiology[documents.tips]
  • Tang Tags: Clinical Research: Pathophysiology and Therapeutics Source Type: research Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited mechanobullous disorder associated with blisters, chronic wounds, scars, contractures, pain, and itch[medworm.com]
  • , raised IgE levels, iron deficiency, and renal, liver, and thyroid dysfunction, 10 , 11 as well as matrix metalloproteinase 1 gene polymorphisms, 12 filaggrin gene mutations 13 and interleukin 31 gene haplotype. 14 The cause of the pruritus and the pathophysiology[jamanetwork.com]

Prevention

  • Treatment Treatment ** The aims of treatment are : 1- Reducing pruritus 2- Preventing scarring 3- Improving the patient’s quality of life. ** The most frequently used treatments for EBP are potent topical steroids under occlusion, or intralesional steroids[interactivederma.com]
  • To attempt to prevent her from scratching, we provided a physical barrier for the patient’s face; a custom made perforated silicone mask worn at night. However, the mask worsened the pruritus due to condensation and heat accumulation.[medicaljournals.se]
  • Wound care Open wounds must be dressed to encourage healing and prevent adherence to clothing.[gosh.nhs.uk]
  • The keratinous cysts have been linked to be of eccrine origin. [10] The mainstay of treatment for this condition includes prevention of trauma and local wound care.[ijpd.in]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!