Quiz 39: What is your diagnosis?

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Diagnosis: Quiz 39

Quiz 39

Answer:  Pemphigus vulgaris

Criteria for diagnosis histopathologically: In addition to a sparse superficial perivascular and interstitial infiltrate of lymphocytes and eosinophils, acantholytic cells, mostly in the lower half of an epidermis that displays in a locus a suprabasal blister, the roof of which is covered largely by a normal cornified layer with a basket-woven configuration, are findings of pemphigus vulgaris.

Differential diagnosis histopathologically: The possibility of Hailey-Hailey disease also must be considered and in this instance is exceedingly difficult to exclude on the basis of changes histopathologic solely. In favor of pemphigus vulgaris and against Hailey-Hailey disease, however, is the absence of any dyskeratotic acantholytic cells, the absence of any hyperplasia of the epidermis, and the presence of eosinophils in the infiltrate of inflammatory cells.

Criteria for diagnosis clinically: Vesicles on an erythematous base accompanied by erosions and crusts are in synchrony with pemphigus vulgaris.

Differential diagnosis clinically: This is not Hailey-Hailey disease because of the sparing of the axillae and that part of the neck visible in the first clinical photograph, and it is not pemphigus foliaceus because the blisters are too tense.

Clinicopathologic correlation: The surface of the blisters is smooth because the cornified layer is normal, the blister is tense because much of it is situated in a spinous zone that, in vivo  was filled with serum, and the redness is a consequence of red blood cells that, in vivo, were jammed in widely dilated venules of the superficial plexus.

Options for therapy predicated on knowledge of histopathologic findings: This process, acantholytic basically and a result of an aberration immunologic pertinent to keratocytes, can be interrupted by administration systemic of corticosteroid in high dose.

1) The findings histopathologic reveal how difficult it may be at times to determine the exact nature of an inflammatory process at a very early stage of it. In this instance, it is challenging in the extreme to decide whether the changes in the section of tissue pictured are those of pemphigus vulgaris or of Hailey-Hailey disease. Although the findings favor pemphigus vulgaris, the possibility of Hailey-Hailey disease cannot be excluded on grounds histopathologic alone.

2) Even the features clinical do not come to the rescue of histopathologist seeking to arrive at a diagnosis with specificity. Conflicts in criteria for diagnosis clinical abound here. The blisters in this patient are more tense than usual for pemphigus vulgaris. Moreover, the distribution of the lesions is more characteristic of pemphigus foliaceus (erythematosus) than it is of pemphigus vulgaris, which tends to involve mucous membranes, (they, at least at this juncture, not being affected in this patient). The distribution of the lesions, however, is more in favor of pemphigus vulgaris than of Hailey-Hailey disease, which has a predilection for intertriginous sites. The flexural creases shown of this patient, however, can be seen to be involved.

3) In the analysis ultimate, results of studies by ELISA saved the histopathologist, the clinician, and the patient; anti-desmoglein 1 and 3 are diagnostic of pemphigus vulgaris.

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