CLINICAL HISTORY:

Signs and symptoms:  We present a 50 y/o caucasian female with new onset of blisters on her thighs, arms and axilla. Pt has a known history of Churg-Strauss Syndrome and states that she developed these blisters while on a prednisone taper. Pt denies any previous history of skin disease. She does state that these blisters become very irritated and painful at times. Pt denies oral lesions and constitutional symptoms. She denies starting, changing dosages and frequency of any medications.

Previous Treatment:  none

Other information:  Pt has a known history of Churg-Strauss which is currently being managed with prednisone 2.5 mg and 5 mg alternating daily. Other past medical history significant for MVP and HTN. Pt denies tobacco, etoh, and recreational drug use. NKDA. Medications include Fluticasone/Salmeterol inhaled, Triamcinolone nasal spray, Ramapril, ASA, Carvedilol, Digoxin, Spironolactone, Alprazolam, and Prednisone. Unremarkable family history.

PHYSICAL EXAM:

Examination of the skin reveals multiple tense bullae with surrounding erythema on the volar wrists bilaterally and extensor forearms. There are scattered hyperpigmented patches noted on bilateral arms and thighs. There are no oral lesions present.

LABORATORY TESTS:

Laboratory Tests: CBC- WNL
CMP- WNL except slightly elevated potassium (5.5 mmol/L )
G6PD- WNL
TPMT- 14.6 U/mL RBC (range: 15.1-26.4 U/mL RBC)
IgA and IgG- WNL
ANA- negative

DERMATOHISTOPATHOLOGY:

Dermatohistopathology: Two 4 mm punch biopsies were performed. Both from the volar wrists.
H&E- subepidermal blister with increased polymorphonuclear leukocytes and eosinophils
Direct IF- thick meshy linear IgG and much weaker C3 deposition along the epidermal basement membrane zone. After salt splitting this specimen the thick linear IgG and Ce deposition was located only at the dermal side of artificially induced blister. There is no IgA, IgM, C5b-9, or fibrinogen deposition observed in this specimen.

DIFFERENTIAL DIAGNOSIS:

1.   Epidermolysis Bullosa Acquisita
2.   Bullous Pemphigoid
3.   Bullous Drug Eruption
4.   Linear IgA Disease
5.   Dermatitis Herpetiformis

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Epidermolysis Bullosa Acquisita

DISCUSSION:

Epidermolysis Bullosa Acquisita (EBA) is a rare, acquired, chronic, autoimmune blistering disease. EBA is clinically characterized by blisters, scars and milia primarily at trauma prone areas. Most common areas affected include extensor surfaces of elbows, knees, ankles and buttocks. This disease typically presents in the third to fifth decades. EBA has been associated with numerous autoimmune diseases including SLE, RA, IBDs, DM, MEN, cryoglobulinemia and thyroiditis. A few case reports of EBA have been reported in patients with multiple myeloma, amyloidosis, lymphoma and CLL. The exact pathogenesis of this disorder is not well understood. EBA patients have IgG antibodies against the basement membrane. The most convincing evidence demonstrates that the autoantibodies react with type VII collagen which is the major component of anchoring fibrils. Clinical and histopathologic correlation is required for proper diagnosis of EBA. H & E is nonspecific for EBA. Direct IF demonstrates a linear deposition of IgG along the epidermal basement membrane zone. Salt-split skin will show immune deposits along the dermal side. This disease is rarely life threatening but usually chronic in nature and frequently recalcitrant to treatment.

TREATMENT:

Our patient was treated with a higher dose of prednisone and started on mycophenolate mofetil with improvement of her symptoms. The eventual goal for our patient was to taper her off the prednisone and ameliorate symptoms using steroid sparing medications.

REFERENCES:

Engineer L, Ahmed AR.Emerging treatment for epidermolysis bullosa acquisita. J Am Acad Dermatol. 2001 May;44(5):818-28.

Busch JO, Sticherling M.Epidermolysis bullosa acquisita and neuroendocrine pancreatic cancer - Coincidence or patho-genetic relationship?J Dtsch Dermatol Ges. 2007 Oct;5(10):916-8.

Remington J, Chen M, Burnett J, Woodley DT.Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita. Curr Dir Autoimmun. 2008;10:195-205.

Bakar O, Demirçay Z, Ergun T.Epidermolysis bullosa acquisita associated with vitiligo, Graves' disease and nephrotic syndrome.Int J Dermatol. 2004 May;43(5):378-80.

Rogers S, Larkin C, McDonald GS, Mullaney J, Collins EP.Epidermolysis bullosa acquisita with pulmonary tuberculosis: a case report. Clin Exp Dermatol. 1983 May;8(3):311-8.

Additional Comment:

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