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Pemphigus Erythematosus

Senear-Usher Syndrome

Pemphigus erythematosus is considered to be a variant of pemphigus foliaceus that includes features of systemic lupus erythematosus in its clinical presentation. Blistering lesions that are typical for all subtypes of pemphigus exhibit a characteristic "butterfly" pattern of appearance on the face, together with a seborrheic erythematous rash. The diagnosis is made on clinical grounds and identifying deposition of immunoglobulins along the basement membrane and intercellular membrane.


Presentation

Pemphigus erythematosus (also known as Senear-Usher syndrome, named after the two authors who first described the disease) is a rather conspicuous disorder that exhibits clinical manifestations of two illnesses - pemphigus foliaceus (a blistering autoimmune disease in which intercellular glycoproteins desmoglein-1 are a target of immunoglobulin (Ig) G) and systemic lupus erythematosus (SLE) [1] [2] [3]. For this reason, some authors interpret pemphigus erythematosus as a disorder carrying multiple autoimmune mechanisms of pathogenesis [2]. The clinical presentation of pemphigus erythematosus is comprised of small vesicles and bullae that exhibit a "butterfly pattern" of distribution on the face (the cheeks and the nose, as well as the nasolabial folds and preauricular areas), which is typical for erythematous lesions seen in SLE that may be concomitantly present [1] [2]. Lesions are also distributed on the scalp, the upper chest, and the back, with some authors hypothesizing that the location of lesions is somehow related to sun exposure [1] [4]. The erythematous base of blistering lesions is visible upon their rupture, while signs of yellowish crusting and scaling are frequently observed [1]. An important distinguishing feature of pemphigus erythematosus (and pemphigus foliaceus) from pemphigus vulgaris is the absence of lesions on the mucosal surfaces (the vulva and the oral cavity) [1].

Pathologist
  • Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M.[books.google.com]
Fatigue
  • Mycophenolate mofetil (30-40 mg/kg/day divided twice daily) is generally well tolerated, although side effects of fatigue, gastrointestinal upset, and tremor are not uncommon, particularly at higher doses.[clinicaladvisor.com]
Fever
  • Call your provider if you have been treated for PV and you develop any of the following symptoms: Chills Fever General ill feeling Joint aches Muscle aches New blisters or ulcers Amagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L, eds.[medlineplus.gov]
  • If you’ve been diagnosed with pemphigus already, you should contact your doctor if you develop: new blisters or sores a rapid spread in the number of sores fever redness or swelling chills weakness or achy muscles or joints Some people get better without[healthline.com]
Amyloidosis
  • 85 143 1843 Erythroderma 14 1907 215 1943 Section Five Vesiculobullous diseases 1953 Other vesiculobullous diseases Vesiculobullous and erosive diseases in the newborn Section Six Adnexal diseases 1959 Section Seven Rheumatologic diseases Mucinoses Amyloidosis[books.google.com]
Myopathy
  • Although they are generally considered safe, some serious adverse effects, such as myositis, myopathy, and rhabdomyolysis can rarely occur.[ncbi.nlm.nih.gov]
Blister
  • The erythematous base of blistering lesions is visible upon their rupture, while signs of yellowish crusting and scaling are frequently observed.[symptoma.com]
  • Senear-Usher Syndrome, or pemphigus erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and lupus erythematosus.[ncbi.nlm.nih.gov]
  • A skin eruption marked by scaling blisters and patches on scalp, face, and trunk. Read Also: Pemphigus foliaceus pemphigus foliaceus pemphigus fo·li·a·ce·us (fō’lē-ā’shē-əs, -sē-əs) n.[definithing.com]
  • A punch or shave biopsy for histology should be obtained at the lateral edge of a fresh blister. Biopsy of an old blister can confuse the diagnosis due to necrosis of the roof of the blister and/or re-epithelialization of the base.[clinicaladvisor.com]
Necrolytic Migratory Erythema
  • The differential diagnosis for more widespread cases of PF include Staphylococcal scalded skin syndrome, necrolytic migratory erythema, Stevens-Johnson syndrome, toxic epidermal necrolysis, and other rare autoimmune blistering diseases.[clinicaladvisor.com]
Dysesthesia
  • […] cutaneous diseases Arne Kanig and Rudolf Happle Ichthyoses erythrokeratodermas and related disorders Keratodermas Kro Dariers disease and HaileyHailey disease Daniel Hohl Theodora Mauro and leanPhilippe Gorog Mosaicism and linear lesions Pruritus and dysesthesia[books.google.com]

Workup

A meticulous clinical, immunological, and histological workup is necessary in order to make the diagnosis of pemphigus erythematosus. The role of a proper physical examination is perhaps of essential importance in recognizing the spectrum of lesions that may suggest either pemphigus or systemic lupus erythematosus. Their distribution and stage (blisters, plaques, scales, crusts, etc.) should be carefully assessed, after which immediate sampling of the lesion and subsequent histological examination is recommended. To distinguish between different forms of pemphigus, the bullae in pemphigus erythematosus stem in the more superficial levels of the epidermis (just below the stratum corneum), while bullae in pemphigus vulgaris and pemphigus vegetans arise in more deep layers of the skin (just above the stratum basale) [4]. To establish a firm diagnosis, however, immunofluorescent antibody testing is the gold standard. The pemphigus group (including pemphigus foliaceus and pemphigus erythematosus) exhibits intercellular space staining of keratinocytes on immunofluorescence (due to antibody attachment to desmoglein-1) [5], but concomitant acantholysis and accumulation of immunoglobulins in desmosomes and at the dermal-epidermal junction along the basement membrane (also known as the lupus band test) is a unique feature of pemphigus erythematosus [1] [2] [6]. Furthermore, serological testing for antinuclear antibodies (ANAs) are positive in a significant number of cases, thus proving the concomitant presence of systemic lupus erythematosus [1].

Microsporum
  • Pritchett E.N....Cusack C.A. 2015 5 Pemphigus erythematosus relapse associated with atorvastatin intake. ( 25258514 ) Lo Schiavo A....Cozzi R. 2014 6 Cutaneous dirt-adherent disease on a base of pemphigus erythematosus. ( 24117279 ) Jun L....Sun Q.N. 2013 7 Microsporum[malacards.org]

Treatment

  • With this treatment, all patients have been virtually free of symptoms, have remained well, and have had normal laboratory values.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis : good. Print off the owner factsheet on Pemphigus Pemphigus to give to your client. Pathogenesis Pathophysiology Same as P. vulgaris Skin: pemphigus vulgaris. Depigmentation of lesions sun aggravates photodermatitis.[vetstream.com]
  • In general, PF and PE have a better prognosis than pemphigus vulgaris. However, prior to the advent of glucocorticoid therapy, the mortality rate was as high as 60%.[dermnet.com]
  • Rather than lump these different diseases under one term, he proposed that pemphigus erythematosus be used to describe a localized form of PF with a better prognosis.[clinicaladvisor.com]
  • Prognosis The prognosis of pemphigus erythematosus is better than that of pemphigus vulgaris. With good dermatologic care, patients with pemphigus erythematosus are often able to live normal lives.[emedicine.medscape.com]
  • Diagnosis of PE with pustules will aid in determining the prognosis and course of the disease. Clinicians should to be vigilant for the rare occurrence of pustules in PE. References Bystryn JC, Rudolph JL (2005) Pemphigus. Lancet 366: 61-73.[omicsonline.org]

Etiology

  • […] be considered—either that manifestations resembling lupus erythematosus can occur as an atypical feature of pemphigus or that we were dealing in this instance with a syndrome in which pemphigus occurs in combination with lupus erythematosus, a common etiologic[jamanetwork.com]
  • McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J.[books.google.com]
  • Etiology Superficial skin blisters in pemphigus erythematosus, as in pemphigus foliaceus, are caused by autoantibodies against desmoglein 1.[clinicaladvisor.com]
  • Etiology Patients with pemphigus develop an autoimmune response directed against desmosomes. [3] In patients with pemphigus foliaceus and its variant, pemphigus erythematosus, the target antigen is desmoglein 1.[emedicine.medscape.com]

Epidemiology

  • Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 1995;104:302–5. CrossRef PubMed Google Scholar Beissert S, Mimouni D, Kanwar AJ, et al.[link.springer.com]
  • […] lateral extension, the AsboeñHansen sign Healing is often accompanied by postñinflammatory hyperpigmentation but no scarring Fatal if untreated because oral erosions impair swallowing Deaths are due to staphylococcal infection or pulmonary embolism Epidemiology[pathologyoutlines.com]
  • The epidemiology of pemphigus erythematosus is unknown, although it is a rare subset of pemphigus foliaceus, which itself is a rare disease (estimates of PF incidence in Western Europe are approximately 0.5-1.0 per million per year).[clinicaladvisor.com]
  • Relapse of pemphigus erythematosus has been associated with atorvastatin intake. [4] Epidemiology Frequency The incidence of pemphigus is 0.5-3.2 cases per 100,000 population per year.[emedicine.medscape.com]
  • Epidemiology PV shows has an approximately equal prevalence among men and women. Peak incidence 30-60 years of age (i). Mean onset of age is 50 to 60 years (ranges on subtype and can affect persons at all ages).[pemphigus.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathogenesis Pathophysiology Same as P. vulgaris Skin: pemphigus vulgaris. Depigmentation of lesions sun aggravates photodermatitis.[vetstream.com]
  • However, because the desmoglein ELISA measures both pathogenic as well as nonpathogenic antibodies (see “Pathophysiology” below), positive desmoglein ELISA index values may still be observed in patients in remission.[clinicaladvisor.com]
  • […] deposits at the dermoepidermal junction For a thorough description and introduction to the possible causes of pemphigus, see the article " Pemphigus: An Acronym for a Disease with Multiple Causes ", published by the International Pemphigus Society. [2] Pathophysiology[emedicine.medscape.com]
  • Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms. [ 1 ] Pathophysiology The particular autoimmune characteristic associated with pemphigus was first described in the 1960s.[patient.info]

Prevention

  • Statins, also known as 3-hydroxy-3-methylglutaril-CoA reductase inhibitors, are well-tolerated drugs used for prevention of atherosclerosis and cardiovascular events.[ncbi.nlm.nih.gov]
  • Department of Dermatology, Second University of Naples, Naples, Italy; 2 Department of Dermatology, AORN "A Cardarelli", Naples, Italy Abstract: Statins, also known as 3-hydroxy-3-methylglutaril-CoA reductase inhibitors, are well-tolerated drugs used for prevention[dovepress.com]
  • These can prevent the blisters from getting infected if they break open. If blisters cover a lot of your skin, you might need to stay in the hospital for treatment. Doctors and nurses will clean and bandage your sores to prevent infection.[healthline.com]
  • It also aims to prevent complications, especially infections.[medlineplus.gov]
  • The primary aim of treatment is to prevent new areas from developing bacterial infections and promote healing of affected areas. Topical treatment with corticosteroids is usually all that is necessary for mild pemphigus foliaceus.[dermnetnz.org]

References

Article

  1. James KA, Culton DA, Diaz LA. Diagnosis & Clinical Features of Pemphigus Foliaceus. Dermatol Clin. 2011;29(3):405-412.
  2. Pérez-Pérez ME, Avalos-Díaz E, Herrera-Esparza R. Autoantibodies in Senear-Usher Syndrome: Cross-Reactivity or Multiple Autoimmunity? Autoimmune Dis. 2012;2012:296214.
  3. Grando S. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity. 2012;45:7–35.
  4. Ioannides D, Hytiroglou P, Phelps RG, Bystryn JC. Regional variation in the expression of pemphigus foliaceus, pemphigus erythematosus, and pemphigus vulgaris antigens in human skin. J Invest Dermatol. 1991;96(2):159-161.
  5. Amagai M, Stanley JR. Desmoglein as a target in skin disease and beyond. Journal of Investigative Dermatology. 2012;132:776–784
  6. Maize JC, Green D, Provost TT. Pemphigus foliaceus: a case with serologic features of Senear-Usher syndrome and other autoimmune abnormalities. J Am Acad Dermatol. 1982;7(6):736-741.

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Last updated: 2019-06-28 10:33