Boullous Pemphygoid
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Francois Donatien De SadeBoullous Pemphygoid
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Francois Donatien De SadeAnswer
Bullous pemphigoid versus bullous drug eruption: Bullous pemphigoid is a
chronic autoimmune disease that typically has an onset between 60 and 80
years of age, though it occasionally starts in childhood. Bullous pemphigoid is
characterized by tense bullae that usually develop on a prodromal eruption of
urticarial-type skin lesions. Pemphigoid has a predilection for the flexural areas,
including the groin, axillae, abdomen, and especially the lower legs and feet.
Lesions tend to be scattered and discreet, with shapes varying from arciform to
annular or serpiginous. The rash may be pruritic, but it is often asymptomatic.
The Nikolsky sign, ie, intraepidermal cleavage and slippage resulting from a
gentle applied shear force, is typical but not always absent. In one study, 4 of 10
patients with bullous pemphigoid had a positive Nikolsky sign at the edge of a
blister, but none had the sign on nonblistered skin. Most blisters eventually
rupture and form shallow erosions.
The differential diagnosis includes pemphigus vulgaris, which tends to start at
40-60 years of age and which manifests with flaccid bullae on normal skin with
a positive Nikolsky sign (in distinction to the tense bullae on urticarial skin seen
in bullous pemphigoid). Pemphigus vulgaris is less common but more serious of
the 2 conditions. Other blistering conditions that may need to be considered
include porphyria cutanea tarda, erythema multiforme, toxic epidermal
necrolysis, staphylococcus scalded skin syndrome, and dermatitis
herpetiformis.
The cause of bullous pemphigoid is an unknown autoimmune mechanism
wherein autoantibodies attack glycoproteins in the dermal basement
membrane. In rare cases, certain drugs may precipitate true disease or may
cause a bullous drug eruption, which is a temporary condition that, unlike true
pemphigoid, eventually resolves once the offending agent is discontinued.
Furosemide and penicillamine are the classic examples or precipitating agents,
but sulfa-based drugs, penicillins, angiotensin-converting enzyme inhibitors, and
quinolones have all been implicated. If a pemphigoidlike illness develops soon
after exposure to a suspected agent, the drug should be stopped or replaced
and a steroid taper initiated. If lesions recur after this, true bullous pemphigoid is
likely and consultation with a dermatologist is warranted.
The diagnosis of bullous pemphigoid should be confirmed by means of skin
biopsy because treatment involves chronic immunosuppression, often for years.
Oral steroids are the initial treatment of choice, but other immunosuppressants
may help minimize adverse effects of therapy. A typical starting dosage is
prednisone 0.5-1 mg/kg/day. Dosages lower than this are used for mild disease.
Topical steroids tend to be as effective as oral agents, but they minimize
systemic adverse effects, especially in severe disease. However, oral
involvement, increased cost, and difficulty with self-application may limit the use
of topical agents in some patients. After the disease is well controlled, a gradual
taper to the lowest effective dosing is advisable.
This patient's rash resolved with steroids and discontinuation of levofloxacin.
The rash continued to resolve after steroids were withdrawn; therefore, she was
presumed to have a bullous drug reaction rather that true bullous pemphigoid.
For more information about bullous pemphigoid, see the eMedicine articles
Bullous Pemphigoid and Oral Manifestations of Autoimmune Blistering Diseases
(within the Dermatology specialty).
BACKGROUND
A bedridden 85-year-old woman with advanced multi-infarct dementia is sent in
from a nursing home for evaluation of a rash on her abdomen with a possible
skin infection at her gastrostomy tube (G-tube) site. The nursing-home staff first
noticed the rash 2 days ago. It apparently began after levofloxacin therapy was
started for a urinary tract infection. Because skin creases and the umbilicus
were involved, a topical antifungal was started, but no improvement was noted.
The patient has no fever or rigors.
On physical examination, the patient smiles when spoken to and has good
ocular tracking, but she cannot meaningfully verbalize and follows only the
simplest of commands. Her vital signs are within normal limits, and she appears
to be in no distress. Other than baseline right-hemiparesis, examination yields
unremarkable results except for the skin findings.
Although the scalp, palms, and mucous membranes appear to be spared, the
patient is noted to have a generalized, patchy, erythematous rash involving less
than 5% of her body surface. The rash is present around the side of her neck
and her upper abdomen, but it seems to be worst around the G-tube and in the
umbilicus (see Images 1-2). The distribution seems to suggest a fungal etiology
because of the predilection for moist areas. However, once the foam booties
used to prevent pressure sores are removed from her feet, impressive, tense
blisters on an erythematous base are noted (see Image).
What is the diagnosis?
Hint
The nature and location of the rash is key.
Authors: D. Brady Pregerson, MD,
Attending Physician,
Department of Emergency
Medicine,
Cedars Sinai Medical Center;
Author,
Quick Essentials: Emergency
Medicine and Pharm Animals
Pharmacopoeia
(www.ERpocketbooks.com)
eMedicine
Editor: Rick G. Kulkarni, MD,
Assistant Professor,
Yale School of Medicine,
Section of Emergency Medicine,
Department of Surgery,
Attending Physician,
Medical Director,
Department of Emergency
Services,
Yale-New Haven Hospital, Conn
