POSTER MINI

Bullous Pemphigoid
Created by : Ainil Fatima Zainodin
: Muhammad Syazwan Khali Aziz

(C111 10 878)
(C111 10 852)

PATIENTS IDENTITY
Name
Sex
Medical Record
Age
Address
Marital Status
Date of birth
Occupation
Religion
Date admitted

: Nn. E
: Female
: 663225
: 20 years old
: Jln Pampang Aswip 2 Block DD 1
: Single
: 16 August 1994
: Students
: Christian
: 13 Mei 2014

HISTORY TAKING
Anamnesis
: Autoanamnesis.
Chief complaint
: Skin Blister occur on whole body
Further anamnesis
:
The blister feel itchy and pain since 1 week ago. Firstly on January, a small blister
appear at patients chin. Then, the blister burst . One week later, new blister formed at the
neck with the size of a coin. The patient then seek treatment at Puskesmas and given cream
and also Acylclovir 5x1 for medication. However, the blister even spread to the whole body.
Then, the patient seek treatment at RS Pelamonia and given drug combination. The treatment
was a success. But the blister seems to reappear after 1 month (on February) because the
patient fail to maintain the medication. The patient then seek treatment at RS Awal Bros with
the same complaint like before and recover well. However the blister still reappear few
months later with bigger size of blisters on whole body, and the patient seek treatment at RS
Wahidin.
History of using cream medication (+),
History of other diseases (-),
History of the same disease (+) recurrent 3times since January 2014,
History of family with the same disease (-),
History of allergic (-)

GENERAL STATUS
Present condition
: Moderate illness
Nutritional status
: Normal
Consciousness
: Compos mentis
Vital Signs
- Blood pressure : 110/ 80 mmHg
- Pulse rate
: 70 x/minutes, regular
- Respiratory rate : 18 x/minutes
- Body temperature : 36C

DERMATOLOGY STATUS
Location
: Regio Generalisata
Lesion size
: Nummular
Efloresensi
: Tense bulla, Erosion,
hiperpigmentasi,

Macula

PHYSICAL EXAMINATION
Head

: Sclera
: Icterus (-)
: Conjunctiva
: Anemia (-)
: Labial
: Cyanosis (-)

Cor/pulmonal
: In normal state
Abdomen
: peristaltic (+) in normal state
Extremities
: In normal state
Lymph node
: Lymphadenopathy (-)

ADDITIONAL EXAMINATION
Test

Results

Interpretation

WBC

11.8 u/L

LED 1

45 mm

LYMPH

5.8 %

MONO

1.4 x 10 u/L

NEUT

91.6 x10 u/L

Ureum

8 mg/dl

GDS

124 mg/dl

PHOTO 1

PATIENTS PHOTO

c
a

Location
: Regio Extremitas Inferior
Effeloresensi
: (a) Eritem, (b) Crusta, (c) Macula Hiperpigmentasi
Date of picture taken
: 15 Mei 2014 (After bulla aspiration )

PHOTO 2

PATIENTS PHOTO
a

b
c

Location
: Regio Extremitas Superior
Effeloresensi
: (a) Eritem, (b) Crusta, (c) Macula Hiperpigmentasi
Date of picture taken
: 15 Mei 2014 (After bulla aspiration)

PHOTO 3

PATIENTS PHOTO

Location
: Regio Truchus Anterior
Effeloresensi
: (a) Eritem, (b) Macula Hiperpigmentasi
Date of picture taken
: 15 Mei 2014 (After bulla aspiration)

PHOTO 4

PATIENTS PHOTO

Location
: Regio Frontalis
Effeloresensi
: (a) Bulla,
Date of picture taken
: 20 Mei 2014 (New bulla lesion)

DIAGNOSIS
Bullous Pemphigoid

TREATMENT
SYSTEMIC

Inj. Ceftriaxone 1gr/ 12hours / i.v

Inj. Dexamethasone 1amp / 12hours / i.v
Inj. Neurobion 1amp / 12 hours / i.v
Interhistine Tab 2x1

TOPICAL
Fuson cr
Gentamycine 10 gr + Metronidazole
500 mg
Compres PK 1/10,000

DISCUSSION Bullous Pemphigoid

DEFINITION
Bullous pemphigoid :

An autoimmune blistering disease of elderly people.

The blisters are subepidermal & intact epidermis forms the roof.

Often starts with :

- Pruritus
- Urticated
- Erythematous lesions

Later they form :

-Large tense blisters (on erythematous & normal skin)

EPIDEMIOLOGY
Typically occur at age > 60 years old ( Peak incidence in the 70s).
There are several reports in infant & children although this is rare.
There is not known ethic, racial and sexual predilection for developing
bullous pemphigoid.
More common in Jews and people from middle east.

Incidence of Bullous Pemphigoid

United Kingdom

11%
25%

Scotland
64%

ETIOLOGY

Autoimmune inflammatory disease

PATHOGENESIS
IMMUNOPATHOLOGY
Presence of Bullous Pemphigoid Antigen
(Anti-BP 180 & Anti-BP 230 ) on Hemidesmosome.
Cause presence of Autoantibodies (IgG)
against the antigen.
Disrupt mechanical stability & adhesion
integrity between epidermis and dermis
Produce skin fragility and blistering
Fuction Hemidesmosome : Anchoring the basal cell to the underlying basement membrane

CLINICAL MANIFESTATION
CUTANEOUS LESIONS
Starts : Itching
: Urticaria
: Erytematous Lesion
Later form blister
: Large tense blister with dome shape
: Numerous in size
: Location flexural aspect of limbs
central abdomen
: Their contents : clear serious exudates
: sometimes bloodstains
Those that ruptured blister
: May heal spontaneously without scarring
: Leave hyperpigmented patches

ADDITIONAL EXAMINATION
1. LABORATORY TEST
Often laboratory studies play a small supporting role. Approximately half
patient will have elevated total serum IgE level.

of

2. HISTOPATHOLOGY
Biopsy of early small vesicle histology reveal subepidermal blister
superficial dermal infiltrate consist of
eosinophils
- neutrophils lymphocytes
- monocytes/ macrophage
3. ELECTRON MICROSCOPY
Ultrastructural studies Early blister formation occurs in lamina lucida.
On blister area there is loss of
- Anchoring filaments
- Hemidesmosomes.
Degranulation of eosinophils, neutrophils and MC
in the lesional/perilesional skin also been observed.
4. SPECIAL TEST (ELISA) : useful in detecting IgG and IgE antibodies.

DIFFERENTIAL DIAGNOSE
Bullous
Pemphigoid

Lesions

Patients

Cutaneous
distribution

Isotype

Urticated plaques
Tense Blister

Elderly (few
infant &
children)

Trunk, Limbs,
flexures

IgG
(Few IgA)

Adults &
children

Generalized,
variable

IgG

Adults

Generalized,
variable

IgG, IgA

Young adults
(some children &
elderly)

Symmetrical
elbow

IgA

Epidermolysis Urticated plaques

bullosa acquisita Tense Blister
(EBA)
Milia scarring
Bullous SLE

Dermatitis
Herpetiformis

Urticated plaques
Tense Blister

Papulovesicle

TREATMENT
Treatments
Corticosteroids

Descriptions
High potency topical steroids
Prednisone

Other Immunosuppressive Agents

Azathiprine
Mycophenolate Mofetil
Others; Methotrexate

Modulators Of Antibody Levels

Intravenous -globulin
Plasmapheresis

Other

Tetracycline or erythromycin and

micotinamide
Dapsone
Topical Tacrolimus

PROGNOSIS
BP is a chronic disease characterized by spontaneous exacerbations and remissions.
Because of - intractable pruritus,
- presence of bullous, eroded or impetiginized lesions
Impact on the quality of life.
Prognosis depend on :
- Age
- Treatment
- General Health
- Absence of anti-BP antibodies

REFERENCE
1. Calton DA, Liu Z, Diaz LA. Bullous Pemphigoid. In: Goldsmith LA, Katz SI, Gilchrest
BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick's Dermatology In General
Medicine. United States of America: McGraw-Hill Companies,; 2012. p. 861-70.
2. F. Wojnarowska VAVSMB. Immunobullous Disease. In: Burns T, Breathnach S, Cox N,
Griffiths C, editors. Rooks Textbook of Dermatology. 4. 7 ed. Garsington Road, Oxford
United Kingdom: Blackwell Science; 2004. p. 41.23-41.25.
3. Vesiculobullous diseases In: Yancey KB, Allen DM, editors. Dermatology. 2 ed. United
States: Elsevier Inc.
4. Harahap PDM. Penyakit Vesiko-Bulosa In: Sastrodripodjo dS, editor. Ilmu Penyakit Kulit
1. Jakarta, Indonesia: Hipokrates; 2000. p. 134-8.