Journal of Zoonosis Subdivision

Type 2 Leprosy Reaction with Sweets

Syndrome Like Presentation

Francielle Chiaveli Chiaratti Antonielle Borges Faria Neumann

Egon Luiz Rodrigues Daxbacher Thiago Jeunon
Department of Dermatology, Hospital Federal de Bonsuceso-Rio de Jenerio, Brazil
(2016) 175:345-9

Read by: Astri Melistri

1

Introduction

Leprosy is a chronic, infectious disease

caused by Mycobacterium Leprae acid
fast bacilli and characterized by neural
and cutaneous manifestations
The chronic course of the disease can
be stopped by acute phenomena called
reactions.
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Introduction

Introduction
Characterized by painful erythematous edematous
plaque

Accompanied by fever

Leukocytosis with neutrophilia and infiltrate rich in

neutrophils in the dermis

Case Report

Female patient
38 years old
residing in Rio de Jenerio,
with history of asthma
1 year before the medical consultation,
intermittent erythematous lesions on the
lower members had appeared, which
improved with the used oral
corticosteroid for asthma attack

Case Report

In the previous month, skin lesions became

diffuse and associated with fever myalgia
nausea vomiting without neurological
symptoms

Case Report
On examination, papules and erythematous
edematous plaque were observed, some with a
pseudovesicular aspect and others with central
pallor, disseminated over the body, sparing
mucosa, palms and soles.

Neurological examination presented no changes.

10

Case Report
The notification was performed and the degree
of disability and neural function was assessed,
showing no changes
Evaluation of household contacts did not
present any signs and symptom of leprosy, and
they were referred to take the second dose of
the bacillus calmette guerine (BCG Vaccine)

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Case Report
Blood count showed 11.000 leukocytes, with
90% neutrophils, and biochemical laboratory
test showed no changes.
Histopatological examination of the skin
revealed marked edema of the papilary with
infiltration of intact and fragmented
neutrophils, in association with groups of
vacuolized histiocytes in the reticular dermis,
arranged along neurovascular bundle.

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Case Report
The patient treatment started with
multibacillary multidrug therapy and oral
prednisone at a dose of 1 mg/kg/day.
Later, pentoxyfilline at a dose 400 mg/day was
associated, with gradual increase up to a dose
of 400 mg every 8 hours.

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Discussion

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Discussion
The evolution of the lesions, there may be the
central clearing, resulting the target aspect
similar to erythema multiforme
The most commonly face,neck,chest,back
and upper extremities
Histopathology Infiltrate of intact and
fragmented neutrophils in the superficial and
middle dermis and marked edema in the
pappilary skin layer

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Discussion
Lepra are classified into two major type 1
and type 2
Type 1 reaction, or reversal, occurs in
borderline patients as a result of variations in
cellular immunity. Its characterized by
reactivation of preexisting lesion or by the
appearance of new lesion, which are
erythematous and infiltrative plaque with
swollen appearance

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Discussion
Type 2 reaction is mediated by immune
complex, occurring in multibacillary patients and
presenting typically as ENL

Its characterized by inflammatory nodule,

erythematous and painful which can progress to
necrosis

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Discussion
There are atypical forms of type 2 reaction, called
erythema multiforme like and sweets syndrome
like reactions.
Erythema multiforme like is characterized by
macule and purplish eritematous plaque, with
formation of vesicles and blisters progressing to
necrosis.
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Discussion

The clinical diagnosis of type 2 reaction variant

is difficult when no exist nodular lesions
characterized of ENL, predominating lesions in
edematous plaques that are frequently
misinterpreted as a type 1 reaction.

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Discussion
In the histopatological study of type 1 reaction, a
large influx of lymphosites and confluence of
tuberculoid granuloma in the dermis are observed,
which can be dissected by edema or show central
fibrinoid necrosis. Also blurring of the
dermoepidermal junction.

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Discussion
Histopathology of classical type 2 leprosy reaction
(ENL) is characterized by a large influx of
neutrophils in the dermis, which is permeated by
group vacuolated histiocytes containing bacilli.

Neutrophil predominantly in the reticular

portion of the dermis, in the dermo-hypodermic
junction and in subcutan tissue
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Discussion

In the case of sweets syndrome like type 2

reaction, the infiltration of neutrophil occurs
predominantly in the papillary portion with
pronounced edema.

22

Discussion
In Brazil, the treatment of choice for type 2
leprosy reaction is performed with thalidomide.

Pentoxyfilline is one of the alternatives in the

case of contraindications of thalidomide, as well
as corticosteroids.

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Conclusion
In tropical countries, where infectious disease like leprosy
is endemic.
It is important to consider that cases similar to sweets
syndrome may represent reaction presentation. A high
diagnostic suspicion and through clinical and laboratory
evaluation are essential for the correct diagnosis. In the
case of sweet syndrome like leprosy reaction, the
identification of vacuolated histiocyte containing bacilli in
the midst of an infiltrate rich in neutrophil is of
fundamental importance.

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Thank You
25

Lepra

Bryceson hal 1
26

Mycobacterium Leprae
Pada pemeriksaan
mikroskop electron
tampak gambar
berikut ini:

Bryceson hal 5
27

Mycobacterium Leprae
Patologi Klinis :
Bersifat subklinis
Multiplikasi lambat
Infeksi melalui penetrasi via kulit atau
mukosa hidung)
Respon imun manusia terhadap bakteri M.
Leprae adalah infeksi seluler dan humoral

Bryceson hal 11
28

Leprae
Lepra Tuberkuloid (TT)
Sistem imun seluler baik lepra
tuberkuloid. Histologi tuberkuloid

Bryceson hal2915

Leprae
Lepra Lepromatosa (TT)
Sistem imun seluler gagal lepra
lepromatosa. Histologi leproma

Bryceson hal 16
30

Leprae
Lepra Borderline (BB)
Di antara kedua kutub yang
ekstrem, terdapat bentuk
penyakit dari spectrum penyakit
leprakeseimbangan di antara
multiplikasi bakteri dan system
imun seluler pada pasien

31

Bryceson hal 20
32

Bryceson hal 20
33

Keluhan dan Tanda Klinis

Masa Inkubasi : berkisar 2-4 tahun, walaupun pernah

dilaporkan masa inkubasi 3 bulan hingga 40 tahun.

Pemunculan
Lesi kulit : mungkin tunggal atau beberapa, satu lesi
menetap sebelum lesi lainnya muncul, atau lesi inisial
hilang secara spontan beberapa bulan atau tahun

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Bryceson hal 22

Keluhan dan Tanda Klinis

Mati Rasa
Kelemahan : perlahan atau mendadak
Nyeri
Mata : Nyeri, fotofobia, pandangan kabur
Hidung : tersumbat, secret, darah
lepromatosa
Keluhan sistemik demam, malaise, nyeri
persendian
Gatal

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Diagnosis
Tanda Kardinal lepra
1. Anestesi : distribusi pada saraf
perifer besar, pada TT, keterlibatan
saraf halus pada LL
2. Penebalan saraf
3. Lesi kulit : macula atau infiltrat
hipopigmentasi atau merah
4. Didapatkan BTA pada slit skin smear
pasien lepromatosa dan lesi
Bryceson hal 38
borderline

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Gambar saraf tepi

37
Bryceson hal 50

Terapi
Lepra tipe PB
Rifampisin 600 mg sekali sebulan
Dapson 100 mg sekali sehari
Lepra MB
Rifampisin 600 mg sekali sebulan
Dapson 100 mg sekali sehari
Clofazimine 50 mg setiap hari
Clofazimine 300 mg setiap bulan
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Bryceson hal 60-61

Komplikasi Imunologis : Reaksi

Terminologi dari
reaksimenggambarkan timbulnya
tanda dan gejala dari suatu inflamasi akut
di lesi pada pasien lepra.
Reaksi tipe 1 adalah hipersensitivitas
seluler dan sering tapi tidak selalu. Reaksi
tipe ini terjadi pada pasien dengan
borderline (BL,BB,BT). Terbagi : reaksi
reversal dan upgrading.
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Komplikasi Imunologis : Reaksi

Reaksi Kedua (tipe 2) :

hipersensitivitas humoral. Terjadi
karena reaksi antigen-antibody.
Terjadi pasien lepromatosa (tipe
LL, lebih jarang tipe BL)

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Bryceson hal 81

Reaksi tipe 2
Gejala Klinis : Reaksi tipe 2 biasa
muncul pada tipe BL,LL
Reaksi ditandai ENL
Muncul lesi kulit dengan nodul eritem
yang nyeri, berbentuk kubah dengan
batas tidak tegas, mengkilap,nyeri
tekan.
Lesi mengalami ulserasi, pus kuning
kental, mengandung BTA polimorfik
Bryceson hal 84
dan degenerasi

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Reaksi tipe 2
Lesi biasanya muncul pada bagian
ekstensor
Lesi bertahan beberapa hari, kulit
terasa menebal
ENL kronik indurasi

42
Bryceson hal 84

Reaksi tipe 2

43
Bryceson hal 84

Terapi

Reaksi Ringan :
Aspirin 600-1200 mg diberikan
tiap 4 jam
Klorokuin : 150 mg basa
klorokuin diberi
3 kali sehari
Antimonial
Talidomid : 400 mg perhari
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Bryceson hal 87

Terapi
Reaksi berat :
Kortikosteroid
Dapson
Clofazimine

45
Bryceson hal 88