Familial bullous lichen planus (FBLP): Pedigree analysis and clinical characteristics

Journal of Cutaneous Medicine and Surgery
Changzheng HuangYouwen Zhou

Abstract

Familial bullous lichen planus (FBLP) is a rare condition. The clinical features dearly have been described. We report the largest patient series of FBLP and describe its clinical characteristics and inheritance pattern. In this retrospective chart review, we analyzed nine consecutive familial pedigrees of FBLP with 36 affected individuals who presented to the Department of Dermatology at the Wuhan Union Hospital, a tertiary referral hospital in central China. Parameters analyzed include age of onset, gender predilection, lesional distribution, nail and mucosal involvement, clinical course, and inheritance pattern. Thirty-six of 85 individuals in the nine families were affected (42.4%). Females were more likely to be affected than males (58.3% vs 35.7%, G(chi 2 = 3.99. P < 0.05). A bimodal disease onset was found, with one peak at 1-3 years and another at 13-17 years. The shin is the most commonly affected area (97%) followed by the upper limbs and the thighs. Involvement of the torso is relatively rare. Only a minority of cases involves the oral mucosa. The disease tends to follow a chronic and progressive course. The inheritance pattern is autosomal dominant with variable penetrance. Familial bullous lichen planus is a chroni...Continue Reading

References

Aug 1, 1991·Histopathology·E WillsteedP H McKee
Jan 1, 1991·Annales de dermatologie et de vénéréologie·T CognatG Moulin
Jan 1, 1990·Dermatologica·V KatzenelsonM Sandbank
Mar 1, 1989·Clinical and Experimental Dermatology·D J GawkrodgerP K Buxton
Sep 1, 1987·Archives of Dermatology·S KaurS Sehgal
Jul 1, 1985·Journal of the American Academy of Dermatology·M L Kofoed, G L Wantzin
Dec 1, 1984·Journal of the American Academy of Dermatology·C CamisaJ J Yohn
Dec 1, 1993·Pediatric Dermatology·S Handa, A J Kanwar

❮ Previous
Next ❯

Citations


❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Journal of Computer Assisted Tomography
S FarookiD Resnick
Advances in Experimental Medicine and Biology
Matthew A Brown
The Journal of Business Strategy
H C LucasS Cox
Current Opinion in Investigational Drugs
Andrea LeBlanc
Issues in Brief
Jacqueline E DarrochJosefina V Cabigon
© 2021 Meta ULC. All rights reserved