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].

• Pain

  Pain management. Pain medicine or other pain management strategies may be used to help with painful blisters. Follow up. This condition may return, even after successful treatment. Go to all follow-up appointments. [cedars-sinai.org]

  NHS support is also available to help you deal with the physical and emotional effects of living with long-term pain. Read more about living with pain. Page last reviewed: 02/10/2018 Next review due: 02/10/2021 [nhs.uk]

  Pemphigus vulgaris (PV) is a rare and serious condition causing painful blisters to develop on the skin and lining of the mouth, nose, throat and genitals. [nidirect.gov.uk]

  Pain medicines if local pain relief is not enough Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. [medlineplus.gov]

• Weight Loss

  loss and malnutrition because of pain when eating and drinking tooth decay and gum disease because it can be painful to look after your teeth and gums pain during sex and pain when peeing or pooing (if your genitals or anus are affected) You'll have [nhs.uk]

  Among them: Skin infections Infections that get into the bloodstream (sepsis) Being unable to eat, leading to weight loss and malnutrition Depression Some forms of the condition can cause death if not treated The medications that you take for pemphigus [webmd.com]

  Mouth lesions may be tender, preventing adequate food intake that leads to weight loss. The lesions may be accompanied by weakness and malaise, and a history of epistaxis, dysphagia, and hoarseness. [aafp.org]

  Mouth and throat lesions can interfere with eating and drinking and so cause weight loss. [skinhealthinfo.org.uk]

  Subsequently, it worsened with dysphagia, odynophagia, poor oral intake, weight loss and general malaise. She denied recent antibiotic use, traveling, exposure to chemical agents, changes in diet or sick contacts at home. [dermatoljournal.com]

• Fishing

  Fish Oil and Coconut Oil: Fish oil and coconut oil contain fatty acids which significantly reduce inflammation throughout the body. Coconut oil is also a very potent anti-bacterial, anti-fungal and anti-viral agent. [life-saving-naturalcures-and-naturalremedies.com]

  Al-Shatter Observations on reproductive organs and tissues of two freshwater cyprinid fishes [View Abstract] [Full Article - PDF] PP. 764 - 773 J. Med. Med. [interesjournals.org]

• Tachycardia

  Its advantage is the safety profile, with few side effects (headache, dyspnea, tachycardia, and abdominal discomfort). [anaisdedermatologia.org.br]

• Blister

  The erythematous base of blistering lesions is visible upon their rupture, while signs of yellowish crusting and scaling are frequently observed. [symptoma.com]

  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. [dermatologyadvisor.com]

  It offers readers a succinct clinical description for quick recognition of different types of blistering diseases and their relative urgency. [books.google.com]

• Skin Lesion

  Initially, most of his skin lesions appeared on the scars of the surgical incisions and suture sites three months after he underwent thymectomy. [ncbi.nlm.nih.gov]

  Here, we report an atypical pemphigus erythematosus with erythematous papules, plaques, and pustules skin lesions. [repository.uki.ac.id]

• Ulcer

  This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. [ncbi.nlm.nih.gov]

  Treatment may involve: Antibiotics and antifungal medicines to control or prevent infections Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers IV feedings if there are severe mouth ulcers Numbing (anesthetic) mouth lozenges [medlineplus.gov]

  Both, oral and conjunctival mucosa, had extensive ulcerative lesions over an erythematous base with crusting and serosanguineous fluid (Figure 2). [dermatoljournal.com]

• Eruptions

  A 69-year-old man with recurrent eruptions of pyoderma gangrenosum for 4 years is described. The patient also suffered from paraproteinemia (increased IgA with M-component) and transient acantholytic dermatosis resembling pemphigus erythematosus. [ncbi.nlm.nih.gov]

  a relatively benign form of chronic pemphigus that is characterized by the eruption especially on the face and trunk of lesions resembling those which occur in systemic lupus erythematosus Dictionary Entries Near pemphigus erythematosus Cite this Entry [merriam-webster.com]

  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]

  The disease has a chronic benign, relapsing course and the pustular eruption has the distinctive histology of a subcorneal bulla containing neutrophils. [drmhijazy.com]

• Skin Rash

  This may result in severe dehydration for those suffering this debilitating skin disease. Symptoms may occasionally disappear however, the painful blisters and skin rashes will usually return if untreated. [researchtrials.org]

  It can cause skin rashes to form because your body’s healthy cells attack your skin’s tissues. What resources are available for people with pemphigus? There are several resources to help support people with pemphigus. [my.clevelandclinic.org]

  Drug rashes Renal damage Hypoalbuminaemia Cholestasis Psychoses Reversible male infertility. [drmhijazy.com]

  Infusion reactions, which may be reduced with previous administration of analgesics, antihistamines, and corticosteroids, include anaphylaxis, fever, hypotension, chills, headache, nausea, pruritus, and skin rash. [anaisdedermatologia.org.br]

• Malar Rash

  (b) Erythematous rash over the malar region sparing the nasolabial folds. [dermsinica.org]

  rash on face Otherwise identical to pemphigus foliaceus combined with features of lupus erythematosus Usually middle aged adults ( Clin Exp Dermatol 2009;34:708 ) Pemphigus foliaceus: Endemic in Brazil; called fogo selvagem or "wild fire" in Portugese [pathologyoutlines.com]

  Characteristic features on physical examination Classically, pemphigus erythematosus affects only the face, similar in distribution to the malar rash of systemic lupus erythematosus. [dermatologyadvisor.com]

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].

• Staphylococcus Aureus

  Impetigo with bullae is a contagious infection of the skin in children, with purulence, typically caused by staphylococcus aureus. [longdom.org]

  Desmoglein 1 is cleaved by exfoliative toxins elaborated by certain strains of Staphylococcus aureus. [dermatologyadvisor.com]

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

Rapid response of treatment-resistant pemphigus foliaceus to the anti-CD20 antibody rituximab. Br J Dermatol. 2003 Oct. 149(4):899-901. [Medline]. Mrowietz U. Treatment targeted to cell surface epitopes. Clin Exp Dermatol. 2002 Oct. 27(7):591-6. [emedicine.medscape.com]

(eds) European Handbook of Dermatological Treatments. [link.springer.com]

Pemphigus vulgaris is a rare bullous autoimmune dermatosis whose evolution and prognosis are unpredictable. Aim. [hindawi.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]

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]

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. [dermatologyadvisor.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

[…] 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]

This article reviews the available research and discusses a clinical approach to the etiology, diagnosis, and treatment of pemphigus vulgaris of the nail. [escholarship.org]

Zhu X Pan J Yu Z, Epidemiology of pemphigus vulgarisin the Northeast China: a 10-year retrospective study. J Dermatol 2014;41 (1):70–75. doi:10.1111/1346-8138.12286. [30]. Kridin K Schmidt E. Epidemiology of Pemphigus. [journals.lww.com]

Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol 1995; 104: 302–305. [medicaljournals.se]

Pemphigus vulgaris and pemphigus foliaceus: differences in epidemiology and mortality. [medicaljournalssweden.se]

Epidemiology Bullous pemphigoid: BP is the most frequent blistering disease of the skin (and mucosa) affecting typically the elderly (>65 years), but can occur at any age and in any race. [pemphigus.org]

[…] 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]

Pathogenesis Pathophysiology Same as P. vulgaris Skin: pemphigus vulgaris. Depigmentation of lesions → sun aggravates → photodermatitis. [vetstream.com]

Another proposal of the pathophysiology in PV that may occur in addition to the above explanation is the desmoglein non-assembly depletion hypothesis. [statpearls.com]

Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2] Synonyms and keywords: Overview Pemphigus vulgaris is a chronic blistering skin disease with skin lesions that are rarely pruritic, but which are often painful.[1]:561 Historical Perspective Classification Pathophysiology [wikidoc.org]

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. [dermatologyadvisor.com]

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]

Prevention There are no current guidelines to prevent pemphigus because the cause is unknown. [winchesterhospital.org]

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]

Dermatologist-approved lesson plans, activities you can use Free to everyone, these materials teach young people about common skin conditions, which can prevent misunderstanding and bullying. [aad.org]

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.