Multiple blisters on the hands and abdomen - Clinical Advisor

Multiple blisters on the hands and abdomen

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  • CA0811DermClin_1

A disoriented man aged 82 years was brought in by his wife after she noticed several nickel-sized blisters on his abdomen and smaller blisters on his hands. She reported that the blisters appeared suddenly one week earlier. Some had since ruptured and become itchy.

The man had not started any new medications or suffered trauma to the affected area. On exam, several tense clear bullae were noted on his upper abdomen, flanks, and dorsal hands. The underlying skin appeared normal with no erythema, induration, or inflammation appreciated. Scattered erosions were also noted on the patient’s lower back.
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Bullous pemphigoid (BP) is an uncommon chronic autoimmune inflammatory skin disease. BP is characterized by the subacute or acute appearance of large, tense vesicles with a predilection for the groin, axillae, trunk, thighs, and flexor surfaces of the forearms. After...

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Bullous pemphigoid (BP) is an uncommon chronic autoimmune inflammatory skin disease. BP is characterized by the subacute or acute appearance of large, tense vesicles with a predilection for the groin, axillae, trunk, thighs, and flexor surfaces of the forearms.

After the bullae rupture, large denuded areas remain. These areas heal spontaneously without scarring or milia formation. In some patients, there are scattered erythematous patches and urticarial plaques. Significant pruritus is frequently present.

Several distinct clinical forms of BP exist. In the most frequent generalized bullous form, tense bullae arise on normal-appearing or erythematous skin anywhere on the body. Oral and mucosal involvement, if present, is minimal. In the vesicular form, small tense vesicles are present on urticarial or inflamed skin. The vegetative verrucous subtype is uncommon, and the disease is manifested in the intertriginous areas. Persistent urticarial lesions with or without conversion to bullous eruptions characterize the urticarial form. Rarely, BP may be manifested as a generalized erythroderma, a nodular eruption or a disease limited to the acral skin only.

BP has no racial or sexual predilection. It generally affects older adults in the fifth through seventh decades of life, with an average age at onset of 65 years. There are case reports of childhood-onset BP.

Untreated, BP persists over a five- to six-year period, often with spontaneous remissions and exacerbations. If treated, the disease usually remits within 18 months to five years, although relapses may occur.

Immunoglobulin (Ig)G autoantibodies attach to the basement membrane zone of the skin. This activates complement via the classic and alternate pathways, attracting inflammatory mediators. Released proteases and reactive oxygen species from infiltrating neutrophils degrade local proteins and lead to blister formation. The layer of the basement membrane zone where IgG binding occurs has been localized to the lamina lucida, with accentuation near hemidesmosomes.1

It is thought that the antibodies directed at BP230 (BPAg1) and BP180 (BPAg2) — two hemidesmosomal bullous pemphigoid antigens — are the primary pathogenic factors in BP. In addition, it is postulated that infiltrating T-helper lymphocytes may play a role in blister formation.

BP has been reported to be precipitated by and localized to areas of radiation, burns, or psoriasis. Medications associated with BP include furosemide (Lasix), nonsteroidal anti-inflammatory drugs, captopril (Capoten), penicillamine, neuroleptics, and antibiotics. BP has been reported to develop shortly after vaccination, particularly in children.

Diagnosis of BP is established with histopathologic analysis of skin from the edge of a blister as well as direct immunofluorescence (DIF) studies on normal-appearing perilesional skin.

Histologic analysis reveals a subepidermal blister. Superficial dermal infiltrate contains many eosinophils, although mast cells and basophils may predominate early in the disease course. There is absence of acantholysis.

DIF is positive for IgG and/or complement C3 deposition in a linear band at the dermal-epidermal junction. Because this pattern may be seen in pemphigus and epidermolysis bullosa acquisita, a repeat DIF study utilizing a salt-split skin preparation should be performed. DIF on salt-split skin localizes IgG to the blister roof (epidermal side) in patients with BP, while in the other bullous disorders, the IgG localizes to the blister floor.

Experimental diagnostic procedures, including direct and indirect immunoelectron microscopy, immunoblotting, immunoprecipitation, and enzyme-linked immunosorbent assay, are available in research laboratories.

The goal of treatment is to decrease blister formation with the most minimal dose of therapy necessary. In addition, avoiding trauma to the skin is beneficial.

Although there are many treatment options available, relatively few controlled studies have been performed.2 The two most common approaches include the use of anti-inflammatory agents and immunosuppressants. Such anti-inflammatory agents as corticosteroids, tetracyclines, and dapsone obstruct the inflammatory process by inhibiting specific cytokine production and vascular permeability. Such immunosuppressants as azathioprine (Azasan, Imuran), methotrexate (Rheumatrex, Trexall), mycophenolate mofetil (Cellcept), and cyclophosphamide (Cytoxan) are useful adjuvants.

For localized or even extensive disease, high-potency topical steroids may be sufficient and should be initiated first.3 If needed, a systemic anti-inflammatory may be added, but consider the risks of severe complications carefully. More often, higher disease severity requires the use of immunosuppressives.

There is a high risk for morbidity and even death in patients with aggressive or widespread disease, those with other comorbidites, and in those who require high doses of medication. The highest risk for death is present in persons with high levels of circulating anti-BP180 antibodies, increased age, low serum albumin, and higher dosage of corticosteroids.4 In addition, sepsis, pain, and limited mobility are side effects of the disease. Side effects of disease therapy include peptic ulcer disease, agranulocytosis, malignancy and diabetes.

After close collaboration with the patient’s primary-care provider, the patient was started on topical clobetasol cream applied b.i.d to the affected skin. His caretaker and spouse were educated regarding gentle wound care and covering open sores to prevent infection. The disease was satisfactorily managed with topical steroids on an as-needed basis to control emerging lesions.

Esther Stern, NP-C, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J. The author has no relationships to disclose relating to the content of this article.


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References

1. James WD, Berger TG, Elston DM. Chronic blistering dermatoses. In: Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa.: Saunders-Elsevier; 2006:467.

2. Khumalo NP, Murrell DF, Wojnarowska F, Kirtschig G. A systematic review of treatments for bullous pemphigoid. Arch Dermatol. 2002;138:385-389.

3. Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. 2002;346:321-327.

4. Rzany B, Partscht K, Jung M, et al. Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol. 2002;138:903-908. 

All electronic documents accessed July 15, 2011


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