| Pemphigus foliaceus | |
|---|---|
| Specialty | Dermatology  |
| Treatment | Typically treated with topical corticosteroids in mild cases. In more severe treatment it is treated similarly to pemphigus vulgaris. |
Pemphigus foliaceus is anautoimmuneblistering disease of the skin.[1]Pemphigus foliaceus causes a characteristicinflammatory attack at the subcorneal layer ofepidermis, which results in skin lesions that are scaly or crusted erosions with anerythematous (red) base.[2]Mucosal involvement is absent even with widespread disease.[3]
If there is an autoimmuneIgG buildup in theepidermis, then nearly all of theantibodies are aimed againstdesmoglein 1. The effect of the antibodies and the immunological pathway is most likely one of three mechanisms:
TheNational Institute of Arthritis and Musculoskeletal and Skin Diseases describes the disease thus:
The characteristic lesions are crusted, scaly erosions on an erythematous base. In more localized and early disease, lesions are well demarcated and have seborrheic distribution (face, upper trunk, scalp). Small flaccid vesicles, unlikepemphigus vulgaris, are not found. The disease may develop slowly or may rapidly progress, resulting in an exfoliativeerythroderma. UV radiation exacerbates pemphigus foliaceus. Patients experience burning and pain. The colloquial term for Brazilian endemic pemphigus, fogo selvagem (Portuguese for “wild fire”) and for the rare Hungarian variant, Csíp mint az erős Pista (Hungarian for "strong burning by Pista"), takes into account many of the clinical aspects of this disease: the burning feeling of the skin, the exacerbation of disease by the sun, and the crusted lesions that make the patients appear as if they had been burned.[5][6]
Pemphigus foliaceus is diagnosed base on history, biopsy of the affected skin, and testing either a blood sample or a skin sample for the antibodies that cause pemphigus.[citation needed]
The differential diagnosis includes other forms of pemphigus,bullous impetigo, subcorneal pustular dermatosis, subacute cutaneousLE, andseborrheic dermatitis. As discussed earlier, the demonstration of IgG autoantibodies against epidermal cell surfaces is essential for separating these disorders from the pemphigus family. A complete review of medications should be done to exclude the possibility of drug-induced pemphigus foliaceus. Because the lesions of pemphigus foliaceus may become secondarily infected, the finding of bacteria does not confirm a diagnosis of bullous impetigo. Likewise, a clinical flare or recalcitrant disease may represent a superimposed disorder, e.g.tinea corporis, especially in patients on systemic corticosteroids.[6]
Patients with this type of pemphigus do not necessarily require treatment with systemic therapy; the use of topical corticosteroids may suffice.[5] When the disease is active and widespread, however, the therapy for pemphigus foliaceus is, in general, similar to that for pemphigus vulgaris. In general, immunosuppressive agents, such asazathioprine,mycophenolate mofetil andcyclophosphamide, when combined with corticosteroids, may result in gaining early control of the disease and an increased percentage of clinical remissions.[6]
The prevalence of pemphigus vulgaris and pemphigus foliaceus in men and women is approximately equal. The mean age of onset of disease is 50 to 60 years, although the range is broad and disease arising in the elderly and in children has been described. In most countries, pemphigus vulgaris is more common than pemphigus foliaceus; exceptions include Finland, Tunisia and Brazil. Patients with fogo selvagem (Brazilian endemic pemphigus) are clinically, histologically and immuno-pathologically similar to patients with sporadic pemphigus foliaceus. However, fogo selvagem occurs in an endemic fashion in certain regions of Brazil (especially along inland riverbeds)[2] and is thought to be caused by an environmental factor(s).[6]
Pierre Louis Alphée Cazenave first described the disease in 1844.[7]
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