Pemphigus foliaceus in Adult
Alerts and Notices
SynopsisPemphigus foliaceus (PF) is an autoimmune skin disorder characterized by acantholysis of the epidermis (dissolution of the bridges between epidermal cells) resulting in superficial crusted erosions, typically in a seborrheic distribution. The predominant pathogenic antibodies, mostly immunoglobulin G4 (IgG4), are directed against desmoglein 1 (anti-Dsg1), although other antibody class subtypes against Dsg1 and autoantibodies against other antigenic targets have been described. PF has a geographic predilection, particularly South America and North Africa. There is no sex / gender predilection.
PF is generally considered a more benign form of pemphigus, and most patients do not become severely ill. However, in rare cases, lesions can progress to exfoliative erythroderma, potentially causing metabolic derangements. Oral lesions are rare, but skin lesions can persist for years.
PF may be seen in any age group but tends to be a disease of adults. An exception is the endemic subtype of PF (fogo selvagem) that can affect children and young adults in endemic areas (ie, Brazil, other parts of Latin America, and Tunisia). Pathogenesis of endemic PF is complex, involving genetic, environmental, and immunological factors. Environmental factors include the bite of some insects, such as the Similium black fly (Simulium nigrimanum). Pemphigus erythematosus (Senear-Usher syndrome) is another PF subtype and is considered a less severe form of the disease that typically affects the malar region and, rarely, also affects the seborrheic areas. Pemphigus erythematosus may coexist with other autoimmune disorders, such as myasthenia gravis or lupus erythematosus. It has also been reported in association with medications such as penicillamine. See Drug Reaction Data for more information.
Sun exposure or ionizing radiation are known triggers for PF.
L10.2 – Pemphigus foliaceous
35154004 – Pemphigus foliaceus
Differential Diagnosis & Pitfalls
- Pemphigus vulgaris – PF and pemphigus vulgaris (PV) can be difficult to distinguish on hematoxylin and eosin (H&E) and direct immunofluorescence (DIF). A combination of clinical (based on presence of mucosa involvement in PV) and serologic evidence when available (typically PF is only positive for anti-Dsg 1 antibodies) can help differentiate. However, in the real world, patients with mixed features do exist, such as those with anti-desmoglein 3 Ig positivity who lack mucosal lesions.
- Erythrodermic psoriasis
- Paraneoplastic pemphigus
- Erythema multiforme
- Exfoliative dermatitis (erythroderma)
- Atopic dermatitis
- Seborrheic dermatitis
- Scarlet fever
- Staphylococcal scalded skin syndrome
- Stevens-Johnson syndrome
- Toxic epidermal necrolysis
- Toxic shock syndrome
- Generalized contact dermatitis (see also systemic contact dermatitis)
- Lichen planus
- Pityriasis rubra pilaris
- Drug hypersensitivity syndrome
- Drug-induced photosensitive reaction
- Subacute cutaneous lupus erythematosus
- Graft-versus-host disease
- Systemic lupus erythematosus
- Pemphigus herpetiformis
Drug Reaction DataBelow is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
Pemphigus foliaceus in Adult