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The clinical features of bullous pemphigoid are extremely polymorphous. Several atypical forms of bullous pemphigoid have been described, and the diagnosis critically relies on immunopathological findings. We describe three bullous pemphigoid patients characterized by palmoplantar keratoderma, diffused hyperkeratotic cutaneous lesions and extremely high levels of immunoglobulin E serum. The diagnosis of bullous pemphigoid should be taken into account in patients presenting diffused hyperkeratotic cutaneous lesions and palmoplantar keratoderma, even in the absence of blisters. Alteration of the keratinization process, that could occur in patients with genetic mutations in desmosomal and hemidesmosomal genes, may also be due to circulating autoantibodies against hemidesmosomal proteins in these bullous pemphigoid patients.
This article was published in the following journal.
Name: The Journal of dermatology
Nagashima-type palmoplantar keratoderma (NPPK) is an autosomal recessive type of PPK with non-progressive, diffuse, transgradient hyperkeratosis. Clinical findings usually include mild diffuse palmopl...
Vohwinkel syndrome belongs to the group of hereditary palmoplantar keratoderma, having an autosomal dominant inheritance. In this report, the authors present a case of a four-year-old boy with diffuse...
Bullous pemphigoid (BP) is the most common autoimmune bullous disease. Whether there is an increased risk for subsequent BP among cancer patients is still unclear.
Reply to: Comment on "Oral diabetes medications other than dipeptidyl peptidase-4 inhibitors are not associated with bullous pemphigoid: A Finnish nationwide case control study" and a case report of glucagon-like peptide-1 receptor agonist induced bullous pemphigoid.
Bullous pemphigoid is the most common autoimmune blistering disorder in the elderly. It affects people aged 70 years or older. Clinically it is characterized by intensely pruritic eruption consistin...
Bullous pemphigoid affects mainly elderly patients. It is often associated with neurologic disorders, which represent a major risk factor of the disease
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease of the skin in European countries, including France. Immunologically, BP is characterized by the productio...
The aim of the study is to assess that it will be possible to control with a single cycle of rituximab patient with bullous pemphigoid.
Patients diagnosed with bullous pemphigoid were confirmed based on the investigators national diagnostic criteria. Patients who meet all inclusion criteria and conflict the exclusion crite...
Bullous pemphigoid (BP) is the most common blistering auto-immune disease of skin with an incidence estimated to 400 new cases per year. Topical corticosteroid therapy is considered the st...
An autosomal dominant disorder characterized by a widely distributed, well-demarcated hyperkeratosis of the palms and soles. There is more than one genotypically distinct form, each of which is clinically similar but histologically distinguishable. Diffuse palmoplantar keratoderma is distinct from palmoplantar keratoderma (KERATODERMA, PALMOPLANTAR), as the former exhibits autosomal dominant inheritance and hyperhidrosis is frequently present.
An itching, autoimmune, bullous SKIN disease that occurs during the last two trimesters of PREGNANCY and PUERPERIUM. It also affects non-pregnant females with tissue of PLACENTA origin, such as CHORIOCARCINOMA; or HYDATIDIFORM MOLE. It exhibits antigenic and clinical similarity to bullous pemphigoid (PEMPHIGOID, BULLOUS). This disease does not involve herpes viruses (old name, herpes gestationis).
Group of mostly hereditary disorders characterized by thickening of the palms and soles as a result of excessive keratin formation leading to hypertrophy of the stratum corneum (hyperkeratosis).
An autosomal dominant hereditary skin disease characterized by epidermolytic hyperkeratosis that is strictly confined to the palms and soles. It has been associated with mutations in the gene that codes for KERATIN-9.
An autosomal dominant skin disease characterized by transient and variable noninflammatory ERYTHEMA and hyperkeratosis. It has been associated with mutations in the genes that code for CONNEXINS. Erythrokeratodermia variabilis inherited in an autosomal recessive fashion has also been reported. Affected individuals often develop PALMOPLANTAR KERATODERMA.
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include microorganisms (such as bacteria, fungi, parasites, and viruses) and chemicals. Antibodies may be produc...