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Case Number : CT0112 Adam_Bates

Please read the clinical history and view the images by clicking on them before you proffer your diagnosis.
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61-year-old female with biopsy from elbow.


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Adam_Bates

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DH

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Adam_Bates

Posted

Dermatitis herpetiformis.
 

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Adam_Bates

Posted

Agree with Dermatitis Herpetiformis (Duhring Disease)
 

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Adam_Bates

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Subepidermal blisters with neutrophils
Dermatitis herpetiformis
Papulovesicles have a characteristic herpetiform grouping and a predilection for extensor surfaces such as the elbows, usually in symmetrical distribution. Excoriations are almost always present. Direct immunofluorescence is expected to show granular deposits of IgA in the dermal papillae of perilesional and uninvolved skin, and the deposition is not uniform (IgA deposits haphazard in papilla).

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Adam_Bates

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Vesicular subepidermal dermatitis with predominance of neutrophils. The differential includes dermatitis herpetiformis, linear IgA dermatosis, acquired epidermolysis bullosa and systemic lupus erythematosus. I can't see dermal mucin deposits to think in the last diagnosis. Although clinical information and, principally, direct immunofluorescence testing, are absolutely necessary, my prefered diagnosis is IgA linear dermatosis.
 

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Adam_Bates

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The histopathological distinction between dermatitis herpetiformis and linear IgA disease is almost impossible in most cases. Direct immunofluorescence is essential for diagnosis.
 

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Adam_Bates

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Hopping there´s moderate to intense pruritus, my diagnosis is DH.
 

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Adam_Bates

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I favor dermatitis herpetiformis over linear IgA dermatosis just because the patient's age and the affected site.

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Adam_Bates

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I agree with the above comprehensive differentials for a neutrophil-predominant subepidermal bullous dermatosis. Most likely DH or Linear IgA disease (assuming that the photos represent a non-solitary lesion).

The presence of very sparse eosinophils in the infiltrate in some photos is non-specific; however, I believe it warrants addition of bullous pemphigoid, cicatricial pemphigoid, or epidermolysis bullosa acquisitia to the differential dx. These can uncommonly be neutrophil-predominant instead of eosinophil-predominant. (This looks like a recent lesion, with no significant upper epidermal changes, so I doubt this is the case).

Direct immunofluorescence and/or indirect immunofluorescence studies should pare down the long differential to one or two entities, if clinical presentation is not helpful. 

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Adam_Bates

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It may well be dermatitis herpetiformis but age does favor the diagnosis of linear IgA dermatosis over that of dermatitis herpetiformis. According to McKee's book dermatitis herpetiformis occurs in all ages, "but particularly people in their second to fourth decades" and about linear IgA dermatosis of adults: "there are peaks in teenagers and young adults and in patients in their sixties".
 

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Adam_Bates

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Dermatitis herpetiformis , but the age of the pt will make think in bullous pemphigoid - neutrophilic infiltrate , epidermolysis bullosa aquisita or bullous lupus

immunofloresence will tell the difference

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Adam_Bates

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Dermatitis herpetiformis. This was confirmed with immunofluorescence.
 

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