Senear-Usher Syndrome

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

Pemphigus Seborrheic; Pemphigus Erythematosus.

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

0.5 to 3.2:100,000 in general population.

Unknown.

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.

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.

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.

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.

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.

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).

Hailey-Hailey Disease (Familial Benign Chronic Pemphigus [Autosomal Dominant]): A family history usually is present. The onset of age is usually in the fourth and fifth decade. It is characterized by the presence of vesicles and erythemateous plaques with overlying crusts typically in the genital areas, also seen in the chest, neck and axillary regions. Burning and itching most often accompany the eruption, and a malodorous drainage occurs as a result of secondary infection. Symptoms related to staphylococcal and candidal overgrowth are common. The characteristic clinical appearance, as well as biopsy, readily confirms the diagnosis. It has been suggested that Hailey-Hailey disease results from a genetic defect in a calcium pump protein. The gene is localized on chromosome 3. The dotted pattern is lost in acantholysed and perilesional areas and antidesmoplakin I + II-positive proteins are observed diffusely in the cytoplasm.

Pemphigoid Bullous Pachydermoperiostosis (Touraine-Solente-Golé Syndrome): This disorder is characterized by cutis verticis gyrata (corrugated overgrowth of the scalp, or “bull-dog scalp” lesion).