of pancreatitis have been previously reported: a 14-year- 3. Söreide K, Söiland H, Kömer H, Haga H, Söreide JA.

Acute pan-
old girl with acute idiopathic pancreatitis, for whom no
genetic analyses were performed, and a 28-year-old patient
with acute recurrent idiopathic pancreatitis; a heterozygous
mutation was found in the CFTR gene, but not in PRSS1 or
SPINK1 [3, 4].
Usual causes of exocrine pancreatic insufficiency (due to
chronic pancreatitis) are mostly linked to cystic fibrosis;
fibrosing pancreatitis, hereditary chronic pancreatitis, trop-
ical calcific pancreatitis, and inborn errors of metabolism
are rarer [5]. In almost 20% of cases, no aetiological factor
is found.
Four main genes associated with idiopathic pancreatitis are
known: PRSS1, SPINK1, CTRC and CFTR [6-9]. SPINK1
and SPINK5 are located on the same chromosome (5q31-
q32) and both encode for protease inhibitors [10]. SPINK1
is a serine protease inhibitor in the pancreas and its mutation
leads to autodigestion of the gland by proteases. We sus-
pected a pathophysiological link between pancreatitis and
Netherton syndrome because of the hypothesis of altered
inhibition of serine proteases in both pancreatic and epider-
mal tissues. However, the lack of expression of LEKTI in
normal pancreatic tissue (Fraitag, unpublished data) does
not support this hypothesis. Our patient had no mutation
of the currently-known genes associated with pancreatitis.
Therefore, our data does not support a pathophysiological
link between Netherton syndrome and pancreatitis. 
creatitis in a young girl with the Netherton syndrome. J Pediatr Surg
2005; 40: e69-72.
4. Fougerousse A, Taïed A, Ezzedine K, Milpied B, Seneschal J. Pan-
créatite chronique et syndrome de Netherton: une nouvelle association.
Ann Dermatol Venereol 2013; (12S1): 572.
5. Nydegger A, Couper RT, Oliver MR. Childhood pancreatitis. J Gas-
troenterol Hepatol 2006; 21: 499-509.
6. Derikx MH, Drenth JP. Genetic factors in chronic pancreatitis: impli-
cations for diagnosis, management and prognosis. Best Pract Res Clin
Gastroenterol 2010; 24: 251-70.
7. Witt H, Werner L, Hennies HC, et al. Mutations in the
gene encoding the serine protease inhibitor, Kazal type 1
are associated with chronic pancreatitis. Nat Genet 2000; 25:
213-6.
8. Schneider A. Serine protease inhibitor Kazal type 1 muta-
tions and pancreatitis. Gastroenterol Clin North Am 2004; 33:
789-806.
9. Rosendahl J, Witt H, Szmola R, et al. Chymotrypsin C (CTRC) vari-
ants that diminish activity or secretion are associated with chronic
pancreatitis. Nat Genet 2008; 40: 78-82.
10. Lee SE, Jeong SK, Lee SH. Protease and protease-activated
receptor-2 signaling in the pathogenesis of atopic dermatitis. Yonsei
Med J 2010; 51: 808-22.
doi:10.1684/ejd.2016.2761

Disclosure. Acknowledgement: We are indebted to Pro- Recalcitrant steroid-induced rosacea suc-

fessor Claude Ferec from the Laboratory of Molecular cessfully treated with 0.03% tacrolimus
Genetics, University Hospital Center of Brest. and 595-nm pulsed dye laser
Financial support: none. Conflict of interest: none.
Steroid-induced rosacea (SIR) is a common symptom of
topical corticosteroid (TCS) overuse. Although discontin-
1
University François Rabelais Pauline MACHET1,2 uation of TCSs is necessary to stop rosacea, a rebound
Tours, 37000 Tours Christine BODEMER3 reaction may occur if the discontinuation is too abrupt. As a
2
CHRU Tours, Gérard LORETTE1,2 solution, dermatologists have prescribed low-potency TCSs
Department of Dermatology, Sylvie FRAITAG4 to create a tapering effect, and topical antibiotics, such as
Unit of Paediatric Dermatology, Martine RAYNAUD1,5 tetracycline and antihistamines, are commonly the replace-
37044 Tours Stéphanie WILLOT1,6 ment treatments [1]. However, it can take a long time before
3
Department of Dermatology and Annabel MARUANI1,2 SIR responds to these tapering treatments.
Reference Center for A 47-year-old woman visited our hospital with erythema-
Genodermatoses and Rare Skin tous patches on both of her cheeks (figure 1A). The patient
Diseases (MAGEC), University had been applying TCSs for a year, after being diagnosed
Paris Descartes - Sorbonne Paris with allergic contact dermatitis, and SIR broke out as a side
Cité, Institute Imagine, University effect of the TCSs. She took minocycline and an antihis-
Hospital Necker-Enfants Malades,
tamine at a local clinic, but symptoms did not improve for
APHP, 75015 Paris
4 a year. For the SIR, she was given a treatment including
Department of Pathology,
University Hospital Necker-Enfants
a topical tacrolimus 0.03% ointment and an antihistamine
Malades, APHP, 75015 Paris twice daily for two weeks. Afterwards, we observed an
5
CHRU Tours, Department of improvement in the inflammation levels (figure 1B) and
Genetics, 37044 Tours thus maintained the medication. Three treatments with a
6
CHRU Tours, Department of 595-nm pulsed dye laser (PDL) were added to the affected
Paediatrics, 37044 Tours, area (Vbeam Perfecta; Syneron-Candela, Wayland, MA,
France USA), with two weeks between each laser session. The
<annabel.maruani@univ-tours.fr> PDL treatments were performed according to the following
parameters: a spot size of 7 mm, a pulse duration of 10 ms,
1. Bitoun E, Michelson A, Lamant L, et al. A LEKTI proteolytic process- and a fluence of 7 to 9 J/cm2 . The erythema improved after
ing in human primary keratinocytes, tissue distribution and defective
expression in Netherton syndrome. Hum Mol Genet 2003; 12: 2417-
eight weeks of treatment (figure 1C).
30. Tacrolimus inhibits the initiation of cytokine transcription
2. Chavanas S, Bodemer C, Rochat A, et al. Mutations in SPINK5, and activation of T cells. It does this by means of bind-
encoding a serine protease inhibitor, cause Netherton syndrome. Nat ing itself to immunophilins as well as blocking calcineurin
Genet 2000; 25: 141-2. phosphatase activity. It is also known to inhibit the release of

312 EJD, vol. 26, n◦ 3, May-June 2016

A of topical calcineurin inhibitor induced rosacea caused
B ciated with rosacea [8]. Treatment with a 595-nm PDL
C treatment methods, applying topical tacrolimus ointment
Figure 1. A) When the patient first arrived at our hospital,
erythematous patches were visible on both of her cheeks; (B)
two weeks after using topical tacrolimus; (C) eight weeks after
beginning treatment.
histamine from skin mast cells and to interfere with epider-
mal cytokine networks [2]. As of today, there are no known
direct vascular side effects from using tacrolimus ointment.
After observing the benefits of using tacrolimus ointment
on erythema, we speculate that both T-cell dysfunction and
mast cell degranulation could play an important role in SIR
pathogenesis. It has been reported that 0.1% and 0.075%
tacrolimus are effective treatments for SIR [3, 4]. This case
reveals that a lower concentration of tacrolimus, 0.03%, is
also effective for treating SIR, which leads to the expec-
tation that it can be used as treatment while minimising
adverse effects, such as irritation.
Although Chu reported that pimeclorimus is more effec-
tive for treating papulopustular lesions than tacrolimus [5],
we found tacrolimus to produce a positive outcome in not
only the erythema area, but also when used on papulopus-
tular lesions. Also, while it has been reported that cases
EJD, vol. 26, n◦ 3, May-June 2016
by pimecrolimus exhibit less severe symptoms than those
caused by tacrolimus, we determine that this is due to pime-
crolimus being more water-based, while tacrolimus has
stronger occlusive properties [6, 7]. However, approaching
this from the perspective of SIR treatment, we believe that
tacrolimus would produce a more positive response due
to its hydration effect. However, as there has not yet been
any research performed on the use of tacrolimus and pime-
crolimus as treatments for SIR, there is a need for further
studies in order to accurately compare between their effi-
cacy.
PDL is known to be effective for treating telangiectasias
and erythema. It is also useful for treating symptoms asso-
precisely targets oxyhaemoglobin in the cutaneous vascu-
lature, and it is currently widely used for treating vascular
lesions. This destroys the vascular component of the dermis,
yielding a clinical improvement through use of selective
photothermolysis. Using the 595-nm PDL to treat rosacea
has been effective for treating telangiectasias and erythema,
as well as for improving other signs of photodamage, such
as enlarged pores and textural irregularities [9, 10].
The misuse of TCS on the face remains a widespread
practice, and many people have regarded it as a mir-
acle compound. This is due to the false notion that it
can resolve all types of facial imperfections. For SIR
patients whose symptoms were not relieved by conventional
during the initial signs of rosacea and continuing the treat-
ment synergistically with a 595-nm PDL improves facial
erythema. 
Disclosure. Financial support: none. Conflict of interest:
none.
Department of Dermatology, Joon SEOK
Chung-Ang University College of Sun Young CHOI
Medicine, Kapsok LI
Seoul, Beom Joon KIM
Korea Myeung Nam KIM
<sun02ya@naver.com> Chang Kwun HONG
1. Ljubojeviae S, Basta-Juzbasiae A, Lipozenèiae J. Steroid dermati-
tis resembling rosacea: aetiopathogenesis and treatment. J Eur Acad
Dermatol Venereol 2002; 16: 121-6.
2. Hanifan JM, Chan S. Biochemical and immunologic mechanisms
in atopic dermatitis: new targets for emerging therapies. J Am Acad
Dermatol 1999; 41: 72-7.
3. Bamford JT, Elliott BA, Haller IV. Tacrolimus effect on rosacea. J Am
Acad Dermatol 2004; 50: 107-8.
4. Goldman D. Tacrolimus ointment for the treatment of steroid-
induced rosacea: a preliminary report. J Am Acad Dermatol 2001; 44:
995-8.
5. Chu CY. An open-label pilot study to evaluate the safety and effi-
cacy of topically applied pimecrolimus cream for the treatment of
steroid-induced rosacea-like eruption. J Eur Acad Dermatol Venereol
2007; 21: 484-90.
6. Hu L, Alexander C, Velez NF, Yang C, et al. Severe tacrolimus-
induced granulomatous rosacea recalcitrant to oral tetracyclines. J
Drugs Dermatol 2015; 14: 628-30.
7. Lübbe J, Stucky L, Saurat J-H. Rosaceiform dermatitis with
follicular demodex after treatment of facial atopic dermati-
tis with 1% pimecrolimus cream. Dermatology 2003; 207:
205-7.
313
8. Tan SR, Tope WD. Pulsed dye laser treatment of rosacea improves A
erythema, symptomatology and quality of life. J Am Acad Dermatol
2004; 51: 592-9.
9. Jasim ZF, Woo WK, Handley JM. Long-pulsed (6-ms)
pulsed dye laser treatment of rosacea-associated telangiectasia
using subpurpuric clinical threshold. Dermatol Surg 2004; 30:
37-40.
10. Bernstein EF, Kligman A. Rosacea treatment using the new-
generation, high-energy, 595 nm, long pulse-duration pulsed-dye laser.
Lasers Surg Med 2008; 40: 233-9.

doi:10.1684/ejd.2016.2757

Axillary web syndrome following granulo- B

matous inflammation after folliculitis

Axillary web syndrome (AWS) is a skin disorder char-

acterised by a visible taut cord within the axillary skin.
AWS typically manifests as a visible web of axillary skin
overlying palpable and non-erythematous cords of tissue
upon arm abduction. The lesion may also extend from
the mid- axilla down the ipsilateral arm and across the
antecubital fossa. AWS may occasionally extend to the
radial aspect of the wrist at the base of the thumb [1-3].
The fibrous cord feels similar to a rope, band, tendon, a
piece of string or taut wire under the skin, with multiple
cords bound together as a web [4]. The involved shoul-
der abduction is limited to 90 degrees or less in 74% of
all patients with AWS [5]. Pathological analysis reveals C
fibrotic bands, and tends to show sclerosed or thrombosed
vessels with surrounding fibrosis. AWS usually presents
in the early postoperative course after axillary dissection
or biopsy. The incidence ranges from 6-72% due to the
complexity of surgery [1]. The condition is caused by the
blockage of normal lymphatic flow through nodes replaced
by a tumour. One recent report described AWS development
without prior surgery. Herein, we report a non-surgical case
of AWS associated with granulomatous inflammation after
folliculitis.
A 27-year-old woman was referred to our institution for
the treatment of a cord-like depression that had been
present in the right axilla for approximately six weeks.
The patient had an episode of folliculitis in this site one
year prior to presentation. However, no treatment had been Figure 1. Elevation of the arm revealed a cord-like structure
applied. The condition evolved into a small nodule that in the left axilla, extending to the medial face of the ipsilateral
ultimately became painful during shoulder abduction. Top- arm (A). There was a discrete collection of histiocytes and
ical application of non-steroidal anti-inflammatory drugs a variable number of multinucleated giant cells, and further
was ineffective. The patient’s past medical history was inflammatory cell infiltration throughout the entire dermis of
non-contributory. Physical examination revealed a dark- the nodule (B) (H and E; ×100). The pathology of the cord
red, bean-sized nodule located in the central right axilla. revealed sclerosed veins with surrounding prominent fibrosis.
The nodule was not tender in texture and had a rubbery con- The blood vessels were highly dilated and congested with red
sistency upon palpation. A visible and palpable cord during blood cells (C) (H and E; ×100).
shoulder abduction at the nodular end was present, extend-
ing 5 cm from the anterior axillary line to the medial surface cells and other inflammatory cells (figure 1B). The cord
of the arm (figure 1A). The cord appeared normal except for had sclerosed veins with surrounding fibrosis (figure 1C).
the fibrous texture, and there was no evidence of erythema or PAS stain was negative. These data suggested the diagno-
other symptoms. B-ultrasonographic examination showed sis of AWS with folliculitis. The nodules and the fibrous
no blood flow signal. The nodule was excised and biopsied. cord completely disappeared after three months, and there
Microscopic examination showed a discrete collection of was no functional impairment of the arm ipsilateral to the
histiocytes and variable numbers of multinucleated giant folliculitis.

314 EJD, vol. 26, n◦ 3, May-June 2016