Pemphigus vulgaris in Child
Pemphigus vulgaris typically affects adults, with a mean age of onset in the 5th and 6th decades of life. Childhood PV, which refers to disease in children younger than 12 years of age, and juvenile PV, which refers to disease in adolescents 13-18 years of age, is rare, comprising less than 5% of all cases. Although there are 2 subtypes of PV, the mucosal-dominant type and mucocutaneous type, the mucocutaneous type is more frequently observed in children and adolescents.
PV is characterized by painful erosions on the oral mucosa and flaccid bullae and erosions on the skin. Before the introduction of systemic corticosteroids, the mortality of PV was 75%. Still, severe cases of PV can be life threatening, and currently complications are related to the use of chronic immunosuppression, secondary infection, loss of the skin barrier, and poor oral intake.
For more information on familial pemphigus vulgaris, see OMIM.
L10.0 – Pemphigus vulgaris
49420001 – Pemphigus vulgaris
- Pemphigus foliaceus
- Drug-induced pemphigus – Penicillamine, captopril, and thiol-containing compounds are common offending agents.
- Paraneoplastic pemphigus – Associated with underlying neoplasms (Castleman disease is the most common in children and adolescents).
- IgA pemphigus
- Pemphigus herpetiformis
- Erythema multiforme – This condition may clinically appear identical to paraneoplastic pemphigus. Patients may have a history of previous episodes that resolved within 2-4 weeks. If classic "target" lesions of the skin are present, this would be more consistent with erythema multiforme.
- Mycoplasma-induced rash and mucositis (MIRM) – Mucositis is a prominent feature and is usually associated with a sparse, polymorphic eruption.
- Stevens-Johnson syndrome – Usually drug-induced, accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after the inciting medication is started.
- Staphylococcal and streptococcal toxic shock syndrome and toxic shock-like syndrome – Look for the sudden onset of an exanthematous eruption.
- Epidermolysis bullosa acquisita
- Linear IgA dermatosis
- Acute graft-versus-host disease – Clinical history of bone marrow transplantation.
- Drug-induced hypersensitivity syndrome (drug reaction with eosinophilia and systemic symptoms [DRESS]) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other visceral involvement.
- Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome)
- Hailey-Hailey disease
- Dermatitis herpetiformis
- Drug-induced erythroderma
- Erythrodermic psoriasis
- Atopic dermatitis with erythroderma
- Contact dermatitis
- Sézary syndrome (see cutaneous T-cell lymphoma)
- Phototoxic reaction
- Photoallergic reaction
- Lichen planus – The erosive oral lesions of lichen planus are usually surrounded by characteristic radiating keratotic striae.
- Cicatricial pemphigoid – This condition mostly affects the mucosa at various sites, with only 20% showing cutaneous bullae.
- Herpes simplex virus (HSV) – The oral ulcerations may be diffuse but are comprised of smaller lesions (1-2 mm) that coalesce.
- Erythema multiforme – Hemorrhagic crusting of lips is typical. Intraoral erosions occur.
- Mycoplasma-induced rash and mucositis (MIRM) – Mucositis resembling erythema multiforme is a prominent feature.
- Aphthous stomatitis
- Cutaneous involvement in herpetic stomatitis would be unusual.
Last Updated: 04/20/2018