ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (2)
Lichen planus pemphigoides
Print
Other Resources UpToDate PubMed

Lichen planus pemphigoides

Print Images (2)
Contributors: Tyler Werbel, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Lichen planus pemphigoides (LPP) is a very rare, acquired, autoimmune blistering disorder with characteristics of both lichen planus (LP) and bullous pemphigoid (BP). The condition typically presents in the fifth decade of life with a slight female predominance. However, it is not uncommon to present in children, and in such cases, there is a male predominance.

LPP is usually idiopathic but has been associated with several medications, most commonly angiotensin-converting enzyme (ACE) inhibitors. Other implicated agents include Chinese herbs, cinnarizine, interferons, narrowband UVB, psoralen plus UVA (PUVA), ribavirin, simvastatin, and weight-reduction products.

Initially, patients present with lesions characteristic of LP: pink-to-violaceous, flat-topped, pruritic, polygonal papules and plaques that localize most commonly to flexural surfaces but may be widespread. Within months, tense vesicles and bullae develop on the LP lesions as well as on uninvolved skin, usually in an acral distribution. Concomitant onset of both conditions has also been described. Oral mucosal involvement is not uncommon.

The pathogenesis of LPP is not completely understood. However, it is thought that injury to basal keratinocytes exposes basement membrane and hemidesmosome antigens, such as BP180 and BP230, leading to autoantibody formation.

Codes

ICD10CM:
L43.8 – Other lichen planus

SNOMEDCT:
238653005 – Lichen planus pemphigoides

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

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

Subscription Required

Last Reviewed: 05/16/2018
Last Updated: 06/19/2018
Copyright © 2019 VisualDx®. All rights reserved.
Lichen planus pemphigoides
Print 2 Images
View all Images (2)
(with subscription)
Lichen planus pemphigoides : Acral, Flexural, Symmetric extremities, Widespread, Pruritus
Copyright © 2019 VisualDx®. All rights reserved.