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
Pemphigus Seborrheic; Pemphigus Erythematosus.
Described by E. Senear, an American dermatologist, and B. Usher, a
0.5 to 3.2:100,000 in general population.
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
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
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
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
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 ...