Acta Dermato-Venereologica 99-9CompleteContent | Page 20
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SHORT COMMUNICATION
Agminated Spitzoid Naevi after Remission of Langerhans Cell Histiocytosis: First Italian Case and
Literature Review
Roberta ROTUNNO 1 , Andrea DIOCIAIUTI 1 , Rita DE VITO 2 , Stefania GASPARI 3 , Claudia CARNEVALE 1 , Simona
GIANCRISTOFORO 1 and May EL HACHEM 1
1
Dermatology Unit, 2 Department of Pathology, and 3 Department of Paediatric Haematology-Oncology, Bambino Gesù Children’s Hospital,
Piazza Sant’Onofrio, 4, IT-00165 Rome, Italy. E-mail: [email protected]
Accepted Feb 26, 2019; E-published Feb 27, 2019
Langerhans cell histiocytosis (LCH) is a rare childhood
disease of the monocyte-macrophage system characterized
by a clonal, uncontrolled proliferation and accumulation
of CD1a + / CD207 + dendritic cells (DCs). LCH is stratified
into single system disease (unifocal or multifocal), multi
organ disease without organ dysfunction, and multiorgan
disease with organ dysfunction. The clinical presentation
ranges from isolated, self-healing skin and bone lesions,
to life-threatening multi-system disease, and the sympto
matology is extremely wide. Thus, the course, treatment
and prognosis vary according to LCH type. Skin lesions
represent the second most-common clinical manifestation
of LCH (30–60%). The eruption may involve the scalp,
intertriginous area, face, trunk and buttocks. Cutaneous
presentations are polymorphic, from erythematous, yellow
scaly or crusted papules, to macerated patches, pustules,
vesicles, or petechiae and purpura (1, 2). with fever, hepatosplenomegaly and anaemia, and presented with
diffuse papular and purpuric lesions on the trunk and inguinal and
axillary folds (Fig. 1 A–C), and crusted yellow papules on the
scalp. No bone marrow involvement was detected.
She was treated with vinblastine (6 mg/m 2 i.v.), oral prednisone
(40 mg/m 2 daily) and methotrexate (500 mg/m 2 24 h-infusion with
folinic acid rescue), because of skin, liver and spleen involvement
with complete regression within two years. At 5 years of age, she
began to develop freckling on the neck and bilateral axillary and
inguinal regions. Physical examination revealed several clustered
brown macules, 2 to 4 mm in diameter, on the neck, bilateral ax
illary and inguinal folds, and external genitalia (Fig. 1 D, E). The
differential diagnosis included lentigines, naevi or postinflamma
tory melanosis. On dermoscopy these pigmented lesions showed
a reticular pattern with sporadic globules. Finally, a biopsy of
two macules was performed and histologic examination revealed
junctional spitzoid naevi consisting of nests of melanocytes with
epithelioid morphology at the dermo–epidermal junction; immu
nohistochemical staining for B-RAF V600E mutant protein was
negative in naevus cells (Fig. S1 C, D 1 ).
CASE REPORT DISCUSSION
We report here the case of a girl, in remission from LCH, with
subsequent appearance of agminated spitzoid naevi on the neck,
and in the axillae, inguinal folds and vulva.
This 6-year-old Caucasian girl, followed for LCH in remission,
was referred to the Dermatology Department for the annual skin
check and for evaluation of her flexure freckling.
LCH had been diagnosed by skin biopsy when the child was
2 months old (Fig. S1 A, B 1 ). On the first admission, she arrived To our knowledge, a correlation between LCH and erup
tive agminated naevi has been described in 4 other child
ren (3–5). Our case is the first Italian patient reported in
the literature. There are several similarities between the
cases suggesting a potential causal and not coincidental
association between LCH and eruptive naevi. LCH skin
involvement was described in 4 of 5 patients; however, all
patients presented pigmented lesions in the folds, especi
ally the groin and axillae. None of the patients developed
naevi on the scalp, the area mostly involved in LCH (3–5).
In 3 cases the lesions
were diagnosed as Spitz
naevi or junctional nae
vi with spitzoid features
(3, 4); Feldstein et al.
(5) reported a diagnosis
consistent with agmi
nated junctional nae
vi. In another case no
biopsy was performed
due to clinical features
Fig. 1. Numerous diffuse crusted
of benign melanocytic
or scaly papules and purpuric
lesions (4). Our his
lesions on the trunk (A), axillary
folds (B) and inguinal (C). Several
tological examination
clustered brown macules, 2 to 4 mm
revealed features of
in diameter, were present on the neck
junctional naevi, with
(D) and bilaterally on axillary (E) and
spitzoid morphology.
inguinal folds (not shown).
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3156
1
doi: 10.2340/00015555-3156
Acta Derm Venereol 2019; 99: 824–825
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.