Abstract
Background: There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders. Summary: Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases. Key Messages: Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.
References
1.
Ito
T
, Ito
N
, Saathoff
M
, Stampachiacchiere
B
, Bettermann
A
, Bulfone-Paus
S
, et al. Immunology of the human nail apparatus: the nail matrix is a site of relative immune privilege
. J Invest Dermatol
. 2005
;125
(6
):1139
–48
. 2.
Cameli
N
, Picardo
M
, Pisani
A
, Ortonne
JP
, Tosti
A
. Characterization of the nail matrix basement membrane zone: an immunohistochemical study of normal nails and of the nails in Herlitz junctional epidemolysis bullosa
. Br J Dermatol
. 1996
;134
(1
):182
–4
. 3.
Tosti
A
, Piraccini
BM
, Wolff
K
, Frank Austen
K
, Goldsmith
LA
, Stephen
K
. Biology of nails
. Fitspatrick’s dermatology in general medicine
. New York
; 2003
. p. 159
–63
. Freedberk IM, Elsen AZ.4.
Christoph
T
, Müller-Röver
S
, Audring
H
, Tobin
DJ
, Hermes
B
, Cotsarelis
G
, et al. The human hair follicle immune system: cellular composition and immune privilege
. Br J Dermatol
. 2000
;142
(5
):862
–73
. 5.
Tosti
A
, Fanti
PA
, Morelli
R
, Bardazzi
F
. Trachyonychia associated with alopecia areata: a clinical and pathologic study
. J Am Acad Dermatol
. 1991
;25
(2 Pt 1
):266
–70
. 6.
Tosti
A
, Bardazzi
F
, Piraccini
BM
, Fanti
PA
, Cameli
N
, Pileri
S
. Is trachyonychia, a variety of alopecia areata, limited to the nails
. J Invest Dermatol
. 1995
;104
(5 Suppl l
):27S
–28S
. 7.
Yuan
H
, Zhou
S
, Liu
Z
, Cong
W
, Fei
X
, Zeng
W
, et al. Pivotal role of lesional and perilesional T/B lymphocytes in pemphigus pathogenesis
. J Invest Dermatol
. 2017
;137
(11
):2362
–70
. 8.
Pietkiewicz
P
, Bowszyc-Dmochowska
M
, Gornowicz-Porowska
J
, Dmochowski
M
. Involvement of nail apparatus in pemphigus vulgaris in ethnic Poles is infrequent
. Front Med
. 2018
;5
:227
. 9.
Carducci
M
, Calcaterra
R
, Franco
G
, Mussi
A
, Bonifati
C
, Morrone
A
. Nail involvement in pemphigus vulgaris
. Acta Derm Venereol
. 2008
;88
(1
):58
–60
. 10.
Habibi
M
, Mortazavi
H
, Shadianloo
S
, Balighi
K
, Ghodsi
SZ
, Daneshpazhooh
M
, et al. Nail changes in pemphigus vulgaris
. Int J Dermatol
. 2008
;47
(11
):1141
–4
. 11.
Schlesinger
N
, Katz
M
, Ingber
A
. Nail involvement in pemphigus vulgaris
. Br J Dermatol
. 2002
;146
(5
):836
–9
. 12.
Baghad
B
, Chiheb
S
. Nail involvement during pemphigus
. Skin Appendage Disord
. 2019
;5
(6
):362
–5
. 13.
De
D
, Kumar
S
, Handa
S
, Mahajan
R
. Fingernail involvement in pemphigus and its correlation with disease severity and other clinicodemographic parameters
. Br J Dermatol
. 2019
;180
(3
):662
–3
. 14.
Gopal
V
, Shenoy
MM
, Bejai
V
, Nargis
T
. Nail changes in autoimmune blistering disorders: a case-control study
. Indian J Dermatol Venereol Leprol
. 2018
;84
(3
):373
. 15.
Kadu
PP
, Kura
MM
. Nail changes in immuno-bullous disorders: a cross-sectional study
. Indian J Dermatol
. 2023
;68
(2
):233
. 16.
Cao
S
, Cui
X
, Li
J
, Pan
F
, Yan
X
, Yang
Q
, et al. Nail changes in pemphigus and bullous pemphigoid: a single-center study in China
. Front Med
. 2022
;9
:933608
. 17.
Tuchinda
P
, Boonchai
W
, Prukpaisarn
P
, Maungprasat
C
, Suthipinittharm
P
. Prevalence of onychomycosis in patients with autoimmune diseases
. J Med Assoc Thail Chotmaihet Thangphaet
. 2006
;89
(8
):1249
–52
.18.
Tosti
A
, André
M
, Murrell
DF
. Nail involvement in autoimmune bullous disorders
. Dermatol Clin
. 2011
;29
(3
):511
–3
. 19.
Kolivras
A
, Gheeraert
P
, André
J
. Nail destruction in pemphigus vulgaris
. Dermatol Basel Switz
. 2003
;206
(4
):351
–2
. 20.
Engineer
L
, Norton
LA
, Ahmed
AR
. Nail involvement in pemphigus vulgaris
. J Am Acad Dermatol
. 2000
;43
(3
):529
–35
. 21.
Patsatsi
A
, Sotiriou
E
, Devliotou-Panagiotidou
D
, Sotiriadis
D
. Pemphigus vulgaris affecting 19 nails
. Clin Exp Dermatol
. 2009
;34
(2
):202
–5
. 22.
Rivera Diaz
R
, Alonso Llamazares
J
, Rodriguez Peralto
JL
, Sebastian Vanaclocha
F
, Iglesias Diez
L
. Nail involvement in pemphigus vulgaris
. Int J Dermatol
. 1996
;35
(8
):581
–2
. 23.
El-Komy
MM
, Abdel Halim
DM
, Samir
N
, Hegazy
RA
, Gawdat
HI
, Shoeb
SA
. Nail changes in female pemphigus vulgaris patients on immunosuppressive therapy
. Int J Womens Dermatol
. 2015
;1
(2
):82
–4
. 24.
Zawar
V
, Pawar
M
, Kumavat
S
. Recurrent paronychia as a presenting manifestation of pemphigus vulgaris: a case report
. Skin Appendage Disord
. 2017
;3
(1
):28
–31
. 25.
Lee
HE
, Wong
WR
, Lee
MC
, Hong
HS
. Acute paronychia heralding the exacerbation of pemphigus vulgaris
. Int J Clin Pract
. 2004
;58
(12
):1174
–6
. 26.
Akiyama
C
, Sou
K
, Furuya
T
, Saitoh
A
, Yasaka
N
, Ohtake
N
, et al. Paronychia: a sign heralding an exacerbation of pemphigus vulgaris
. J Am Acad Dermatol
. 1993
;29
(3
):494
–6
. 27.
Kim
BS
, Song
KY
, Youn
JI
, Chung
JH
. Paronychia: a manifestation of pemphigus vulgaris
. Clin Exp Dermatol
. 1996
;21
(4
):315
–7
. 28.
Apalla
Z
, Chaidemenos
G
, Karakatsanis
G
. Nail unit involvement during severe initial pemphigus vulgaris development
. Eur J Dermatol
. 2009
;19
(3
):290
–1
. 29.
30.
Reich
A
, Wiśnicka
B
, Szepietowski
JC
. Haemorrhagic nails in pemphigus vulgaris
. Acta Derm Venereol
. 2008
;88
(5
):542
. 31.
Böckers
M
, Bork
K
. [Multiple simultaneous hematomas of the finger and toe nails with subsequent onychomadesis in pemphigus vulgaris]
. Hautarzt
. 1987
;38
(8
):477
–8
.32.
Dhawan
SS
, Zaias
N
, Pena
J
. The nail fold in pemphigus vulgaris
. Arch Dermatol
. 1990
;126
(10
):1374
–5
. 33.
de Almeida
HL
, Neugebauer
MGP
, Guarenti
IM
, Aoki
V
. Pemphigus vegetans associated with verrucous lesions: expanding a phenotype
. Clin Sao Paulo Braz
. 2006
;61
(3
):279
–82
. 34.
Török
L
, Husz
S
, Ocsai
H
, Krischner
A
, Kiss
M
. Pemphigus vegetans presenting as acrodermatitis continua suppurativa
. Eur J Dermatol
. 2003
;13
(6
):579
–81
.35.
Sossau
D
, Kofler
L
, Schaller
M
, Yazdi
A
. Pemphigus vegetans initiating on the finger and nail
. Eur J Dermatol
. 2019
;29
(2
):209
–10
. 36.
Leroy
D
, Lebrun
J
, Maillard
V
, Mandard
JC
, Deschamps
P
. [Pemphigus vegetans, a clinical type of chronic pustular dermatitis of Hallopeau]
. Ann Dermatol Venereol
. 1982
;109
(6–7
):549
–55
.37.
Ayoubi
N
, Rudnick
E
, Motaparthi
K
. Pemphigus vegetans with paronychia-like changes resistant to rituximab therapy
. Dermatol Ther
. 2020
;33
(4
):e13515
. 38.
Sukakul
T
, Varothai
S
. Chronic paronychia and onychomadesis in pemphigus vegetans: an unusual presentation in a rare autoimmune disease
. Case Rep Med
. 2018
;2018
:5980937
. 39.
Song
Z
, Li
Q
, Lin
J
, Han
S
. Pemphigus vegetans with the manifestations of acrodermatitis continua: a rare variant
. J Dermatol
. 2018
;45
(2
):e41
–2
. 40.
Namba
Y
, Koizumi
H
, Kumakiri
M
, Hashimoto
T
, Muramatsu
T
, Ohkawara
A
. Bullous pemphigoid with permanent loss of the nails
. Acta Derm Venereol
. 1999
;79
(6
):480
–1
. 41.
Gualco
F
, Cozzani
E
, Parodi
A
. Bullous pemphigoid with nail loss
. Int J Dermatol
. 2005
;44
(11
):967
–8
. 42.
Tomita
M
, Tanei
R
, Hamada
Y
, Fujimura
T
, Katsuoka
K
. A case of localized pemphigoid with loss of toenails
. Dermatol Basel Switz
. 2002
;204
(2
):155
. 43.
Barth
JH
, Wojnarowska
F
, Millard
PR
, Dawber
RP
. Immunofluorescence of the nail bed in pemphigoid
. Am J Dermatopathol
. 1987
;9
(4
):349
–50
. 44.
Benmously-Mlika
R
, Hammami-Ghorbel
H
, Mokhtar
I
. Onychomadesis during bullous pemphigoid
. J Am Acad Dermatol
. 2013
;69
(6
):e306
–307
. 45.
Horváth
ON
, Bagci
IS
, Ruzicka
T
, Sárdy
M
. Atypical anti-p200 pemphigoid with nail involvement and blisters over the joints
. J Dtsch Dermatol Ges J Ger Soc Dermatol JDDG
. 2019
;17
(12
):1289
–90
. 46.
Delaporte
E
, Piette
F
, Janin
A
, Cozzani
E
, Joly
P
, Thomine
E
, et al. [Pemphigoid mimicking epidermolysis bullosa acquisita]
. Ann Dermatol Venereol
. 1995
;122
(1–2
):19
–22
.47.
Haneke
E
, Borradori
L
. Pterygium in bullous pemphigoid: an unusual complication
. JAAD Case Rep
. 2020
;6
(8
):737
–9
. 48.
Alkeraye
S
, Alsukait
SF
. Mucous membrane pemphigoid-associated paronychia with onychomadesis
. BMC Dermatol
. 2019 23
;19
(1
):3
. 49.
Burge
SM
, Powell
SM
, Ryan
TJ
. Cicatricial pemphigoid with nail dystrophy
. Clin Exp Dermatol
. 1985
;10
(5
):472
–5
. 50.
Ludwig
RJ
. Clinical presentation, pathogenesis, diagnosis, and treatment of epidermolysis bullosa acquisita
. ISRN Dermatol
. 2013
;2013
:812029
. 51.
Shimanovich
I
, Petersen
EE
, Weyers
W
, Sitaru
C
, Zillikens
D
. Subepidermal blistering disease with autoantibodies to both the p200 autoantigen and the alpha3 chain of laminin 5
. J Am Acad Dermatol
. 2005
;52
(5 Suppl 1
):S90
–92
. 52.
Zillikens
D
, Kawahara
Y
, Ishiko
A
, Shimizu
H
, Mayer
J
, Rank
CV
, et al. A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone
. J Invest Dermatol
. 1996
;106
(6
):1333
–8
. 53.
Prost
C
, Labeille
B
, Chaussade
V
, Guillaume
JC
, Martin
N
, Dubertret
L
. Immunoelectron microscopy in subepidermal autoimmune bullous diseases: a prospective study of IgG and C3 bound in vivo in 32 patients
. J Invest Dermatol
. 1987
;89
(6
):567
–73
. 54.
Prost-Squarcioni
C
, Caux
F
, Schmidt
E
, Jonkman
MF
, Vassileva
S
, Kim
SC
, et al. International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita
. Br J Dermatol
. 2018
;179
(1
):30
–41
. 55.
Koga
H
, Prost-Squarcioni
C
, Iwata
H
, Jonkman
MF
, Ludwig
RJ
, Bieber
K
. Epidermolysis bullosa acquisita: the 2019 update
. Front Med
. 2019
;5
:362
. 56.
Bernett
CN
, Fong
M
, Yadlapati
S
, Rosario-Collazo
JA
. Linear IGA dermatosis
. StatPearls [internet]
. Treasure Island (FL)
: StatPearls Publishing
; 2023
. [cited 2023 Aug 30]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK526113/.57.
Matsumoto
T
, Nakamura
S
, Ishii
N
, Umemoto
N
, Kawase
M
, Demitsu
T
, et al. Erythrodermic linear IgA/IgG bullous dermatosis
. Eur J Dermatol
. 2019
;29
(2
):220
–1
. 58.
Sarikaya Solak
S
, Ficicioglu
S
. Cephalosporin-induced linear IgA dermatosis in a child: case report and literature review
. Dermatol Ther
. 2019
;32
(4
):e12927
. 59.
Wang
H
, Wang
X
, Yang
B
, Liu
H
, Zhang
F
. Image gallery: onychomadesis in linear IgA bullous dermatosis
. Br J Dermatol
. 2020
;182
(5
):e160
. 60.
Sinclair
RD
, Wojnarowska
F
, Leigh
IM
, Dawber
RP
. The basement membrane zone of the nail
. Br J Dermatol
. 1994
;131
(4
):499
–505
. 61.
Contestable
JJ
, Edhegard
KD
, Meyerle
JH
. Bullous systemic lupus erythematosus: a review and update to diagnosis and treatment
. Am J Clin Dermatol
. 2014
;15
(6
):517
–24
. © 2024 S. Karger AG, Basel
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
2024
You do not currently have access to this content.
