Senear-Usher Syndrome

At a glance

Combination of localized pemphigus-like syndrome and lupus erythematous that could be drug-induced and is frequently associated with autoimmune diseases. Penicillamine is the medication most commonly implicated.

Synonyms

Pemphigus Seborrheic; Pemphigus Erythematosus.

History

Described by E. Senear, an American dermatologist, and B. Usher, a Canadian dermatologist.

Incidence

0.5 to 3.2:100,000 in general population.

Genetic inheritance

Unknown.

Pathophysiology

Frequency increases with HLA A10 DRW6 A26. In pemphigus vulgaris and pemphigus erythematosus, acantholytic cells and perilesional cells exhibit normal dotted pattern along the cell periphery. Autoantigen desmoglein 1, a desmosomal adhesion protein in keratinocytes has been implicated.

Diagnosis

Occurs in persons 17 to 84 years of age but has been reported in children as young as age 6 years. Pemphigus erythematosus can often be distinguished on clinical grounds by the restriction of the lesions to the seborrheic areas of the face and trunk. The presence of antinuclear antibodies is also suggestive of pemphigus erythematosus. The diagnosis can often be confirmed by demonstration of immunoglobulin and complement at the dermal-epidermal junction.

Clinical aspects

Facial lesions have a predilection for the malar region and truncal lesions are often confined to the V area of the chest and interscapular region. There are isolated flaccid bullae or erythematous plaques surfaced with vesicles, erosions, or scale. They can be photoactivated. Electrolyte imbalance and loss of temperature control can occur. Treatment with steroids and immunosuppression.

Precautions before anesthesia

Pemphigus erythematosus has been seen in association with autoimmune diseases, including Lupus Erythematosus, Myasthenia Gravis, and Thymoma; look for this association. Laboratory investigation should include anti-DNA antibodies and electrolytes; evaluate adrenal function (treatment usually includes prednisone) and skin subinfection.

Anesthetic considerations

Cardiac monitoring can be difficult in cases of extensive thorax lesions. Central venous access even in healthy skin can be difficult. In presence of Myasthenia Gravis and Thymoma, the anesthetic considerations are those associated with these disorders. Photosensitivity may also be a problem before induction.

Pharmacological implications

Pemphigus erythematosus may be drug induced. Penicillamine is the medication most commonly implicated. In case of long-time prednisone treatment, hydrocortisone should be given before anesthesia (twice usual dose).

Other conditions to be considered

• Hailey-Hailey Disease (Familial Benign Chronic Pemphigus): Hailey-Hailey disease is a severe genetic blistering disease of intertriginous skin locations that can lead to poor quality of life and increased morbidities. A family history usually is present. The onset of age is usually in the fourth and fifth decade. It is characterized by ...