it is advisable long-term follow-up for the development of cuta-
neous malignancy, potential complication of both EPDS and KS.
The patient gave written consent to the publication of the
photographs, and medical personal data.
L. Atzori,* M. Lai, A. Lappi, M.A. Brundu, F. Rongioletti
Section of Dermatology, AOU of Cagliari - Department of Medical Science
and Public Health, University of Cagliari, Cagliari, Italy
*Correspondence: L. Atzori. E-mail: firstname.lastname@example.org
1 Pye RJ, Peachey RD, Burton JL. Erosive pustular dermatosis of the scalp.
Br J Dermatol 1979; 100: 559–566.
2 Patton D, Lynch PJ, Fung MA, Fazel N. Chronic atrophic erosive der-
matosis of the scalp and extremities: a recharacterization of erosive pus-
tular dermatosis. J Am Acad Dermatol 2007; 57: 421–427.
3 Mastroianni A, Cota C, Ardig
o M, Minutilli E, Berardesca E. Erosive pus-
tular dermatosis of the scalp: a case report and review of the literature.
Dermatology 2005; 211: 273–276.
4 Rongioletti F, Chinazzo C, Javor S. Erosive pustular dermatosis of the
scalp induced by ingenol mebutate. J Eur Acad Dermatol Venereol 2016;
5 Van Exel CE, English JC. 3rd Erosive pustular dermatosis of the scalp and
non scalp. J Am Acad Dermatol 2007; 57:11–14.
6 Semkova K, Tchernev G. Wollina U Erosive pustular dermatosis (chronic
atrophic dermatosis of the scalp and extremities). Clin Cosmet Investig
Dermatol 2013; 6: 177–182.
7 Lai-Cheong JE, Tanaka A, Hawche G et al. Kindler syndrome: a focal
adhesion genodermatosis. Br J Dermatol 2009; 160: 233–242.
8 Yousseﬁan L, Vahidnezhad H, Uitto J. Kindler Syndrome. In: Pagon RA,
Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD,
Ledbetter N, Mefford HC, Smith RJH, Stephens K, eds, GeneReviewsâ
[Internet], Seattle (WA): University of Washington, Seattle; 1993-2017.
[updated 2016 Dec 1].
9 Barbosa NM, Visioli F, Martins MD, Martins MA, Munerato MC. Oral
manifestations in Kindler syndrome: case report and discussion of litera-
ture ﬁndings. Spec Care Dentist 2016; 36: 223–230.
10 Darwich E, Mu
noz-Santos C, Mascaro JM Jr. Erosive pustular der-
matosis of the scalp responding to acitretin. Arch Dermatol 2011;
Three new cases of bullous
pemphigoid during anti-PD-1
Anti-programmed death 1 (PD-1) antibodies are revolutioniz-
ing the treatment of many cancers, including melanoma.
Cutaneous adverse events (AE) of anti-PD-1 antibodies are
common (20%) and mainly non-speciﬁc. Bullous pem-
phigoids (BP) are very rare immune-related AEs induced by
anti-PD-1 antibodies, with only 12 cases previously published.
We report here three new cases of BP during anti-PD-1
therapy with nivolumab.
The ﬁrst patient was a 66-year-old man treated for a meta-
static choroidal melanoma with nivolumab (
Table 1). He pre-
sented with a moderate diffuse pruritic papular eruption on his
back 4 months after nivolumab initiation, associated 3 months
later with tense bullae on the trunk (
Fig. 1a). A skin biopsy con-
ﬁrmed the diagnosis of BP with a subepidermal blister associated
with an eosinophilic inﬁltrate and linear IgG and C3 immune
deposits along the dermoepidermal junction (DEJ) (Fig. 1b).
Serum BP 180 antibodies by enzyme-linked immunosorbent
assay (ELISA) were positive (34 U/mL), and BP 230 antibodies
were not detected. As the patient was responding to nivolumab,
this latter was continued and oral prednisone was initiated at a
daily dose of 0.5 mg/kg. BP was controlled within 2 weeks, and
prednisone doses were gradually tapered. With a 14-month fol-
low-up after initiating corticosteroids, BP is still controlled, at a
daily dose of prednisone of 0.1 mg/kg, and metastatic melanoma
still responds to nivolumab.
The second patient was a 78-year-old man, treated as a ﬁrst-
line treatment with nivolumab for a metastatic melanoma
(Table 1). He developed diffuse pruritus after 3 months of treat-
ment and at the 4th month, an urticarial rash on the trunk with
tense blisters. Skin biopsy examination showed an inﬂammatory
inﬁltrate with eosinophils. DIF conﬁrmed the diagnosis of BP
with linear IgG and C3 deposits along the DEJ. Nivolumab was
continued, and oral corticosteroids at 0.5 mg/kg were initiated
leading to disappearance of prurit and bullae within 2 weeks.
Unfortunately, 1 month later, because of a deterioration of the
general status due to melanoma progression, palliative care was
The third patient was a 68-year-old woman treated for a
metastatic non-small-cell lung cancer with nivolumab (Table 1).
Four months after the ﬁrst infusion, a diffuse pruritic
Figure 1 (a) Tense bullae on the trunk associated with pruritic
papular lesion and excoriation (case 1). (b) Direct immunoﬂuores-
cence revealing linear IgG and C3 immune deposits along the base-
ment membrane at the blister cavity roof (case 1). (c) Tense blister
on the right shoulder (case 3). (d) Linear C3 deposits along the der-
moepidermal junction in direct immunoﬂuorescence (case 3).
© 2017 European Academy of Dermatology and Venereology
2018, 32, e86–e121
Letters to the Editor