Background: Necrobiosis lipoidica (NL) is a rare granulomatous disorder of unknown aetiology. Randomized controlled studies are not available due to it being an orphan disease. Objectives: We evaluated patients in 2 dermatological centres to cluster data about epidemiology, the therapeutic approaches for NL, and their efficacy. Materials and Methods: Comorbidity and the efficacy of the applied treatment was assessed for 98 patients. Results: We identified 54% of patients with concomitant diabetes and 19% with thyroidal disorders. Topical steroids (85.7%) were predominantly used followed by calcineurin inhibitors (31%) and phototherapy (41.8%). Systemically, fumaric acid esters were more frequently applied (26.8%) than steroids (24.4%) and dapsone (24.4%). Steroids, compression therapy, calcineurin inhibitors, phototherapy, fumaric acid esters, and dapsone showed remarkable efficacy. Conclusion: Therapeutic options were chosen individually in accordance with the severity of NL and presence of ulceration. Topical calcineurin inhibitors, systemic application of fumaric acid esters, and dapsone represent effective alternatives to the use of steroids.

1.
Reid
SD
,
Ladizinski
B
,
Lee
K
,
Baibergenova
A
,
Alavi
A
.
Update on necrobiosis lipoidica: a review of etiology, diagnosis, and treatment options
.
J Am Acad Dermatol
.
2013
Nov
;
69
(
5
):
783
91
.
[PubMed]
0190-9622
2.
Ngo
B
,
Wigington
G
,
Hayes
K
,
Huerter
C
,
Hillman
B
,
Adler
M
, et al
Skin blood flow in necrobiosis lipoidica diabeticorum
.
Int J Dermatol
.
2008
Apr
;
47
(
4
):
354
8
.
[PubMed]
0011-9059
3.
Holland
C
,
Givens
V
,
Smoller
BR
.
Expression of the human erythrocyte glucose transporter Glut-1 in areas of sclerotic collagen in necrobiosis lipoidica
.
J Cutan Pathol
.
2001
Jul
;
28
(
6
):
287
90
.
[PubMed]
0303-6987
4.
Oikarinen
A
,
Mörtenhumer
M
,
Kallioinen
M
,
Savolainen
ER
.
Necrobiosis lipoidica: ultrastructural and biochemical demonstration of a collagen defect
.
J Invest Dermatol
.
1987
Feb
;
88
(
2
):
227
32
.
[PubMed]
0022-202X
5.
Erfurt-Berge
C
,
Dissemond
J
,
Schwede
K
,
Seitz
AT
,
Al Ghazal
P
,
Wollina
U
, et al
Updated results of 100 patients on clinical features and therapeutic options in necrobiosis lipoidica in a retrospective multicentre study
.
Eur J Dermatol
.
2015
Nov-Dec
;
25
(
6
):
595
601
.
[PubMed]
1167-1122
6.
Eberle
FC
,
Ghoreschi
K
,
Hertl
M
.
Fumaric acid esters in severe ulcerative necrobiosis lipoidica: a case report and evaluation of current therapies
.
Acta Derm Venereol
.
2010
;
90
(
1
):
104
6
.
[PubMed]
0001-5555
7.
Stanway
A
,
Rademaker
M
,
Newman
P
.
Healing of severe ulcerative necrobiosis lipoidica with cyclosporin
.
Australas J Dermatol
.
2004
May
;
45
(
2
):
119
22
.
[PubMed]
0004-8380
8.
Erfurt-Berge
C
,
Seitz
AT
,
Rehse
C
,
Wollina
U
,
Schwede
K
,
Renner
R
.
Update on clinical and laboratory features in necrobiosis lipoidica: a retrospective multicentre study of 52 patients
.
Eur J Dermatol
.
2012
Nov-Dec
;
22
(
6
):
770
5
.
[PubMed]
1167-1122
9.
Hashemi
DA
,
Brown-Joel
ZO
,
Tkachenko
E
,
Nelson
CA
,
Noe
MH
,
Imadojemu
S
, et al
Clinical Features and Comorbidities of Patients With Necrobiosis Lipoidica With or Without Diabetes
.
JAMA Dermatol
.
2019
Apr
;
155
(
4
):
455
9
.
[PubMed]
2168-6068
10.
Goffrier
B
,
Schulz
M
,
Bätzing-Feigenbaum
J.
Administrative Präferenzen und Inzidenzen des Diabetes mellitus von 2009 bis 2015. Zentralinstitut für kassenärztliche Versorgung in Deutschland;
2017
. Report No.: 17/03.
11.
O’Toole
EA
,
Kennedy
U
,
Nolan
JJ
,
Young
MM
,
Rogers
S
,
Barnes
L
.
Necrobiosis lipoidica: only a minority of patients have diabetes mellitus
.
Br J Dermatol
.
1999
Feb
;
140
(
2
):
283
6
.
[PubMed]
0007-0963
12.
Muller
SA
,
Winkelmann
RK
.
Necrobiosis lipoidica diabeticorum. A clinical and pathological investigation of 171 cases
.
Arch Dermatol
.
1966
Mar
;
93
(
3
):
272
81
.
[PubMed]
0003-987X
13.
Magro
CM
,
Crowson
AN
,
Regauer
S
.
Granuloma annulare and necrobiosis lipoidica tissue reactions as a manifestation of systemic disease
.
Hum Pathol
.
1996
Jan
;
27
(
1
):
50
6
.
[PubMed]
0046-8177
14.
Neuhauser
H
,
Kuhnert
R
,
Born
S
.
12-Monats-Prävalenz von Bluthochdruck in Deutschland
.
Journal of Health Monitoring
.
2017
;
2
(
1
):
57
63
.
15.
Mensink
GB
,
Schienkiewitz
A
,
Haftenberger
M
,
Lampert
T
,
Ziese
T
,
Scheidt-Nave
C
.
SChienkiewitz A, Haftenberger M, Lampert T, Ziese T, Scheidt-Nave C. Overweight and obesity in Germany
.
Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz
.
2013
;
56
(
5-6
):
786
94
.
[PubMed]
1436-9990
16.
Melchert
H
,
Görsch
B
,
Thierfelder
W
.
Schilddrüsenhormone und Schilddrüsenmedikamente bei Probanden in den Nationalen Gesundheitssurveys
.
Beiträge zur Gesundheitsberichterstattung des Bundes
;
2002
.
17.
O’Reilly
K
,
Chu
J
,
Meehan
S
,
Heller
P
,
Ashinoff
R
,
Gruson
L
.
Necrobiosis lipoidica
.
Dermatol Online J
.
2011
Oct
;
17
(
10
):
18
.
[PubMed]
1087-2108
18.
Eisendle
K
,
Baltaci
M
,
Kutzner
H
,
Zelger
B
.
Detection of spirochaetal microorganisms by focus floating microscopy in necrobiosis lipoidica in patients from central Europe
.
Histopathology
.
2008
Jun
;
52
(
7
):
877
84
.
[PubMed]
0309-0167
19.
Haralambous
S
,
Blackwell
C
,
Mappouras
DG
,
Weir
DM
,
Kemmett
D
,
Lymberi
P
.
Increased natural autoantibody activity to cytoskeleton proteins in sera from patients with necrobiosis lipoidica, with or without insulin-dependent diabetes mellitus
.
Autoimmunity
.
1995
;
20
(
4
):
267
75
.
[PubMed]
0891-6934
20.
Lowitt
MH
,
Dover
JS
.
Necrobiosis lipoidica
.
J Am Acad Dermatol
.
1991
Nov
;
25
(
5 Pt 1
):
735
48
.
[PubMed]
0190-9622
21.
Berking
C
,
Hegyi
J
,
Arenberger
P
,
Ruzicka
T
,
Jemec
GB
.
Photodynamic therapy of necrobiosis lipoidica—a multicenter study of 18 patients
.
Dermatology
.
2009
;
218
(
2
):
136
9
.
[PubMed]
1018-8665
22.
De Rie
MA
,
Sommer
A
,
Hoekzema
R
,
Neumann
HA
.
Treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet A
.
Br J Dermatol
.
2002
Oct
;
147
(
4
):
743
7
.
[PubMed]
0007-0963
23.
Kreuter
A
,
Knierim
C
,
Stücker
M
,
Pawlak
F
,
Rotterdam
S
,
Altmeyer
P
, et al
Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study
.
Br J Dermatol
.
2005
Oct
;
153
(
4
):
802
7
.
[PubMed]
0007-0963
24.
Peckruhn
M
,
Tittelbach
J
,
Elsner
P
.
Update: treatment of necrobiosis lipoidica
.
J Dtsch Dermatol Ges
.
2017
Feb
;
15
(
2
):
151
7
.
[PubMed]
1610-0387
25.
Binamer
Y
,
Sowerby
L
,
El-Helou
T
.
Treatment of ulcerative necrobiosis lipoidica with topical calcineurin inhibitor: case report and literature review
.
J Cutan Med Surg
.
2012
Nov-Dec
;
16
(
6
):
458
61
.
[PubMed]
1203-4754
26.
Borgia
F
,
Vaccaro
M
,
Cantavenera
LG
,
Aragona
E
,
Cannavò
SP
.
Ulcerative necrobiosis lipoidica successfully treated with photodynamic therapy: case report and literature review
.
Photodiagn Photodyn Ther
.
2014
Dec
;
11
(
4
):
516
8
.
[PubMed]
1572-1000
27.
Narbutt
J
,
Torzecka
JD
,
Sysa-Jedrzejowska
A
,
Zalewska
A
.
Long-term results of topical PUVA in necrobiosis lipoidica
.
Clin Exp Dermatol
.
2006
Jan
;
31
(
1
):
65
7
.
[PubMed]
0307-6938
28.
Kaae
J
,
Philipsen
PA
,
Wulf
HC
.
Photodynamic therapy of necrobiosis lipoidica using methyl aminolevulinate: A retrospective follow-up study
.
Photodiagn Photodyn Ther
.
2018
Jun
;
22
:
223
6
.
[PubMed]
1572-1000
29.
Meissner
M
,
Valesky
EM
,
Kippenberger
S
,
Kaufmann
R
.
Dimethyl fumarate - only an anti-psoriatic medication?
J Dtsch Dermatol Ges
.
2012
Nov
;
10
(
11
):
793
801
.
[PubMed]
1610-0387
30.
Czarnecki
DB
,
Gin
D
.
The response of generalized granuloma annulare to dapsone
.
Acta Derm Venereol
.
1986
;
66
(
1
):
82
4
.
[PubMed]
0001-5555
31.
Durupt
F
,
Dalle
S
,
Debarbieux
S
,
Balme
B
,
Ronger
S
,
Thomas
L
.
Successful treatment of necrobiosis lipoidica with antimalarial agents
.
Arch Dermatol
.
2008
Jan
;
144
(
1
):
118
9
.
[PubMed]
0003-987X
32.
Chung
CG
,
Rosengrant
A
,
Helm
KF
,
Shupp
DL
.
Necrobiosis lipoidica occurring in a patient with rheumatoid arthritis on concurrent tumor necrosis factor-α inhibitor therapy
.
Int J Dermatol
.
2015
Nov
;
54
(
11
):
1294
6
.
[PubMed]
0011-9059
33.
Gualdi
G
,
Monari
P
,
Farisoglio
C
,
Calzavara-Pinton
P
.
Juvenile ulcerated necrobiosis lipoidica successfully treated with oral cyclosporin A
.
G Ital Dermatol Venereol
.
2013
Dec
;
148
(
6
):
703
4
.
[PubMed]
0392-0488
34.
Leister
L
,
Körber
A
,
Dissemond
J
.
[Successful treatment of a patient with ulcerated necrobiosis lipoidica non diabeticorum with adalimumab]
.
Hautarzt
.
2013
Jul
;
64
(
7
):
509
11
.
[PubMed]
0017-8470
35.
Jockenhöfer
F
,
Kröger
K
,
Klode
J
,
Renner
R
,
Erfurt-Berge
C
,
Dissemond
J
.
Cofactors and comorbidities of necrobiosis lipoidica: analysis of the German DRG data from 2012
.
J Dtsch Dermatol Ges
.
2016
Mar
;
14
(
3
):
277
84
.
[PubMed]
1610-0379
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.
You do not currently have access to this content.