Pemphigus foliaceus in Child
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 blister formation. The autoantibody in question is an immunoglobulin G (IgG) directed against a cell adhesion molecule, desmoglein 1 (although an IgA form has been reported).
PF is associated with human leukocyte antigen (HLA) class II alleles DR1, DR4, and DR14. PF presents in two major forms: endemic (fogo selvagem) and sporadic. The endemic form primarily affects pediatric and young adults, whereas the sporadic form primarily affects middle-aged and older adults.
The endemic form of PF is primarily seen in Brazil, other parts of Latin America, and Tunisia. Pathogenesis is complex, involving genetic, environmental, and immunological factors. Environmental factors include the bite of some insects, such as the Simulium black fly (Simulium nigrimanum). With modern treatment, disease course is typically benign, but fulminant cases have rarely been reported.
Sporadic forms of PF have rarely been reported in children. Triggers in pediatric sporadic PF are similar to those in adults and include sun exposure and medication. Infectious causes have also been suggested but not proven. Prognosis of sporadic pediatric PF is typically good, with a short and benign course, although death related to the skin disease has been reported.
Pemphigus erythematosus (Senear-Usher syndrome), a subtype of PF that typically affects the malar region and may rarely affect the seborrheic areas, has also been reported in children but is typically seen in adults. Pemphigus erythematosus may coexist with other autoimmune disorders, such as myasthenia gravis or lupus erythematosus. Pemphigus erythematosus patients may progress to classic PF over time.
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.
- Tinea faciei and corporis
- 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 Child