Bullous Pemphigoid

Pemfigoid Bullousa

Bullous PemphigoidCreated by: Ainil Fatima Zainodin (C111 10 878) : Muhammad Syazwan Khali Aziz (C111 10 852)

POSTER MINIPATIENTS IDENTITY Name : Nn. ESex : FemaleMedical Record : 663225Age : 20 years oldAddress : Jln Pampang Aswip 2 Block DD 1 Marital Status: SingleDate of birth : 16 August 1994 Occupation: StudentsReligion : ChristianDate admitted : 13 Mei 2014

HISTORY TAKING Anamnesis : Autoanamnesis.Chief complaint : Skin Blister occur on whole bodyFurther 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 STATUSPresent condition: Moderate illnessNutritional status: NormalConsciousness: Compos mentisVital Signs - Blood pressure : 110/ 80 mmHg- Pulse rate: 70 x/minutes, regular- Respiratory rate: 18 x/minutes - Body temperature: 36C

Location: Regio GeneralisataLesion size: NummularEfloresensi: Tense bulla, Erosion, Macula hiperpigmentasi, DERMATOLOGY STATUSPHYSICAL EXAMINATIONHead : Sclera : Icterus (-) : Conjunctiva: Anemia(-) : Labial : Cyanosis (-)

Cor/pulmonal : In normal stateAbdomen : peristaltic (+) in normal stateExtremities : In normal stateLymph node : Lymphadenopathy (-)

ADDITIONAL EXAMINATIONTestResultsInterpretationWBC11.8 u/LLED 145 mmLYMPH 5.8 %MONO1.4 x 10 u/LNEUT91.6 x10 u/L Ureum8 mg/dlGDS124 mg/dlPATIENTS PHOTO

Location : Regio Extremitas Inferior Effeloresensi : (a) Eritem, (b) Crusta, (c) Macula HiperpigmentasiDate of picture taken : 15 Mei 2014 (After bulla aspiration )PHOTO 1acbPATIENTS PHOTOLocation : Regio Extremitas SuperiorEffeloresensi : (a) Eritem, (b) Crusta, (c) Macula HiperpigmentasiDate of picture taken : 15 Mei 2014 (After bulla aspiration)

PHOTO 2cabPATIENTS PHOTOLocation : Regio Truchus AnteriorEffeloresensi : (a) Eritem, (b) Macula HiperpigmentasiDate of picture taken : 15 Mei 2014 (After bulla aspiration)

PHOTO 3baPATIENTS PHOTOLocation : Regio FrontalisEffeloresensi : (a) Bulla, Date of picture taken : 20 Mei 2014 (New bulla lesion)PHOTO 4

DIAGNOSISSYSTEMICBullous Pemphigoid TREATMENTTOPICALInj. Ceftriaxone 1gr/ 12hours / i.vInj. Dexamethasone 1amp / 12hours / i.v Inj. Neurobion 1amp / 12 hours / i.vInterhistine Tab 2x1Fuson cr Gentamycine 10 gr + Metronidazole 500 mgCompres PK 1/10,000

DISCUSSION Bullous PemphigoidDEFINITIONBullous 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)

EPIDEMIOLOGYTypically 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.

ETIOLOGY Autoimmune inflammatory disease PATHOGENESISIMMUNOPATHOLOGY

Cause presence of Autoantibodies (IgG) against the antigen.Presence of Bullous Pemphigoid Antigen (Anti-BP 180 & Anti-BP 230 ) on Hemidesmosome.Disrupt mechanical stability & adhesion integrity between epidermis and dermis Produce skin fragility and blisteringFuction Hemidesmosome : Anchoring the basal cell to the underlying basement membraneCLINICAL MANIFESTATIONCUTANEOUS 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 EXAMINATION1. LABORATORY TESTOften laboratory studies play a small supporting role. Approximately half of patient will have elevated total serum IgE level.

2. HISTOPATHOLOGYBiopsy of early small vesicle histology reveal subepidermal blister superficial dermal infiltrate consist of - eosinophils- neutrophils lymphocytes- monocytes/ macrophage 3. ELECTRON MICROSCOPYUltrastructural 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 DIAGNOSELesionsPatientsCutaneous distribution IsotypeBullous PemphigoidUrticated plaquesTense BlisterElderly (few infant & children)Trunk, Limbs, flexuresIgG (Few IgA)Epidermolysis bullosa acquisita(EBA) Urticated plaquesTense BlisterMilia scarringAdults & childrenGeneralized, variableIgGBullous SLEUrticated plaquesTense Blister

AdultsGeneralized, variableIgG, IgADermatitis HerpetiformisPapulovesicleYoung adults(some children & elderly)

SymmetricalelbowIgATREATMENTTreatmentsDescriptionsCorticosteroidsHigh potency topical steroidsPrednisone Other Immunosuppressive AgentsAzathiprineMycophenolate MofetilOthers; MethotrexateModulators Of Antibody LevelsIntravenous -globulinPlasmapheresisOtherTetracycline or erythromycin and micotinamideDapsone Topical TacrolimusPROGNOSISBP 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 :AgeTreatment General Health Absence of anti-BP antibodies

REFERENCECalton 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.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.Vesiculobullous diseases In: Yancey KB, Allen DM, editors. Dermatology. 2 ed. United States: Elsevier Inc. Harahap PDM. Penyakit Vesiko-Bulosa In: Sastrodripodjo dS, editor. Ilmu Penyakit Kulit 1. Jakarta, Indonesia: Hipokrates; 2000. p. 134-8.