Pemphigus vulgaris in Child
See also in: Anogenital,Oral Mucosal LesionAlerts and Notices
Synopsis
PV typically affects adults, with a mean age of onset in the fifth and sixth decades of life. Variants of the ST18 gene have been found to confer increased risk of PV in some populations.
Childhood PV, which refers to disease in children younger than 12 years, and juvenile PV, which refers to disease in adolescents aged 13-18 years, are rare, comprising less than 5% of all cases. 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. Severe cases of PV can be life-threatening, and complications can be related to immunosuppression from drugs used to treat severe PV, secondary infections, loss of the skin barrier, and poor oral intake.
Codes
ICD10CM:L10.0 – Pemphigus vulgaris
SNOMEDCT:
49420001 – Pemphigus vulgaris
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- 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 – Patients may have a history of previous episodes that resolved within 2-4 weeks. If classic targetoid lesions of the skin are present, this would be more consistent with erythema multiforme.
- Reactive infectious mucocutaneous eruption (RIME) – 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% of patients showing cutaneous involvement.
- Herpes simplex virus (HSV) – The oral ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce.
- Erythema multiforme – Hemorrhagic crusting of lips is typical. Intraoral erosions occur.
- RIME – Mucositis resembling erythema multiforme is a prominent feature.
- Candidiasis
- Aphthous stomatitis
- Cutaneous involvement in herpetic stomatitis would be unusual.
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below 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.Subscription Required
References
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Last Reviewed:01/09/2022
Last Updated:02/16/2022
Last Updated:02/16/2022
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Pemphigus vulgaris in Child
See also in: Anogenital,Oral Mucosal Lesion