Hadassah University Hospital Discharge Summery
Date:	7/27/98
Date of Admission and discharge:	7/6/98
Department:	Dermatology
Code:	106400
Surname:	Daniel
First Name:	Dror
Date of Birth:	1957
Address:	Jerusalem, Israel



Diagnosis: 	Acquired Epidermolysis Bullosa

	40 year old white male.  A sudden appearance of widespread urticarial plaques and 
	blisters took place about one year ago, when the patient was having a summer vacation
	on a Greek island.  At that time we noticed many tense blisters, in configurations 
	somewhat resembling Chinese Symbols, which appeared over healthy skin or over urticarial
	plaques. Gradually, predilected to the upper back, buttocks, hands, elbows, feet and 
	knees, a cigarette paper-like atrophic scars, miliae and loss of finger and toe nails 
	appeared. His mouth, throat and both eyes are also severely involved: both conjunctivas
	and corneas are eroded and scarred and  a pannus is developing on the right eye, 
	endangering his eyesight.

	Ever since the initial episode there are flares with short periods of spontaneous 
	remissions, with a constant worsening.  What seemed to be a spread at random of skin 
	lesion is now clearly predilected to pressure areas:  Scapulae, upper back, buttocks, 
	elbows, hands and feet. Wide erosions develop and heal with the formation of atrophic 
	scars, milia, loss of nails and webbing of adjacent finger.  Skin of involved areas 
	becomes atrophic with a quality of cigarette paper.

	Physical examination is unremarkable otherwise.

Laboratory findings: 
	WBC	6,200
	Hb	9.1
	MCV 	89
	MCH	29
	PLT	514,000
	Granulytes	56%
	Lymphocytes	32%
	Mono	10%
	EO	0%
	Liver and renal functions	--normal
	Total Protein	83
	G/L Albumin	28
	G/L Complement	-normal levels of C3, C4, CH 50
	ANA	+1 out of +4
	Protein and immono Electrophoresis------	no paraprotein
	Hyper IgG ----	3660 mg/dl
	Total body computerized tomography --	no pathological findings
	Bone Marrow biopsy---	normal population

Skin biopsy:
	Subepidermal bulla.  Mixed inflammatory infiltrate, containing a few eosinophiles and
	intercellular edema in the upper dermis.

Immunofluorescense studies:
	LH72 antibody	(anti collagen 7)  continuous stain along the basal membrane.
	GB3 antibody (anti laminin 5): continuous stain along the basal membrane.
	IGA:	continuous stain in a few segments along the dermal epidermal junction.

Course of disease:	
	The patient had received one month's course of Prednisone. There was a burst of 
	urticarial plaques and angioedema of palms and soles.  The eruption was attributed 
	to disease, but three months later on rechallenge the same lesions appeared and it 
	was obviously drug eruption due to Prednisone.  Dapsone administration resulted in 
	hemolysis.
	Cyclosporine in a dose of 3 mg/kg/day showed no improvement. Colchicine 0.5mgx2 was
	not effective. Short course of photopheresis was unyielding. 
	Eventually the patient is now receiving pulse therapy with IV ACTH 20 IV/day and will 
	continue with weekly IM dose. He refuses our suggestion to add an immunosuppressive 
	agent such as Cyclophophamide. There is resolution of the daily fever, and slight 
	improvement of skin and mucosal lesions.

In Summery:  	
	The patient has the clinical features that are best consistent with acquired
 	epidermolysis bullosa.  This diagnosis was not established by immunoelectron
	microscopy.  No treatment modality was proven effective, and only slight 
	improvement is achieved with ACTH.

Dr. Lotem
Dermatology