59: Dermatitis Herpetiformis Flashcards
What is the typical age range for the onset of Dermatitis Herpetiformis?
The typical age range for the onset of Dermatitis Herpetiformis is during the 2nd to 4th decades of life.
What are the common clinical features of Dermatitis Herpetiformis?
Common clinical features include:
- Erythematous papule, urticaria-like plaque, or a vesicle
- Herpetiform grouping of lesions
- Localized stinging, burning, and itching that may precede eruption by 8-12 hours
- Symmetric lesions on elbows, knees, buttocks, shoulders, and sacral areas.
What is the most reliable criterion for the diagnosis of Dermatitis Herpetiformis?
The most reliable criterion for the diagnosis of Dermatitis Herpetiformis is the presence of granular IgA deposits in normal appearing skin.
What are the treatment options for Dermatitis Herpetiformis?
Treatment options include:
1. Sulfones:
- Dapsone (100-150 mg/day)
- Sulfoxone
- Sulfapyridine (1-1.5 g daily if intolerant of dapsone)
2. Dietary changes:
- Gluten-free diet
- Elemental diet
- Atkins diet
What is the prognosis for patients with Dermatitis Herpetiformis?
The prognosis for patients with Dermatitis Herpetiformis includes:
- Symptoms may persist indefinitely.
- The condition may have a waxing and waning course, with some patients being free of symptoms for up to a year without treatment.
- NSAIDs can exacerbate the condition.
What is the most likely diagnosis for a patient with symmetric erythematous papules and vesicles on their elbows and knees?
The most likely diagnosis is Dermatitis Herpetiformis (DH).
Genetic markers to test include HLA-DQ(a10501, B102) or HLA-DQ(a103, b10302), which are identical to those in celiac disease.
What diagnostic technique was used to find granular IgA deposits in normal-appearing skin?
The diagnostic technique used is Direct Immunofluorescence (DIF).
It is significant because granular IgA deposits in normal-appearing skin are the most reliable criterion for diagnosing DH.
What alternative treatment options are available for a patient with Dermatitis Herpetiformis who is intolerant to dapsone?
Alternative treatments include Sulfapyridine (1-1.5 g daily), a gluten-free diet, an elemental diet, or the Atkins diet.
What additional health risks should be monitored for a patient with Dermatitis Herpetiformis and a history of untreated celiac disease?
Monitor for increased frequency of bone loss, atrophic gastritis, pernicious anemia, malignancies (e.g., NHL, GI lymphoma), thyroid disease, insulin-dependent diabetes, lupus, Sjögren’s syndrome, vitiligo, and neurologic diseases such as epilepsy and ataxia.
What histological findings would you expect in older lesions of Dermatitis Herpetiformis?
In older lesions, you would expect subepidermal vesicles.
What is the likely reason for exacerbation of Dermatitis Herpetiformis symptoms after taking NSAIDs?
NSAIDs can exacerbate Dermatitis Herpetiformis, likely due to their effect on immune or inflammatory pathways.
What is the typical dosage of dapsone for a patient with Dermatitis Herpetiformis?
The typical dosage of dapsone is 100-150 mg/day.
Potential side effects include hemolysis, methemoglobinemia, and hypersensitivity reactions.
What additional dietary interventions could be considered for a patient with Dermatitis Herpetiformis who has a gluten-free diet but continues to experience symptoms?
Additional dietary interventions include an elemental diet or the Atkins diet.
What diagnostic technique confirms blister formation above the lamina densa in Dermatitis Herpetiformis?
Indirect Immunofluorescence (IIF) confirms blister formation above the lamina densa, indicating that the lamina lucida is the most vulnerable component of the dermoepidermal junction (DEJ).
What diagnostic test likely used detects transglutaminase antibodies in a patient with Dermatitis Herpetiformis?
The diagnostic test likely used was ELISA, which detects transglutaminase antibodies.
This finding suggests gluten sensitivity and supports the diagnosis of DH.
What underlying condition might explain neurologic symptoms such as ataxia and dementia in a patient with Dermatitis Herpetiformis?
The neurologic symptoms such as ataxia and dementia might be explained by untreated celiac disease, which is associated with DH.
What other autoimmune conditions should be screened for in a patient with Dermatitis Herpetiformis and a history of pernicious anemia?
Screen for thyroid disease, insulin-dependent diabetes, lupus, Sjögren’s syndrome, and vitiligo.
What genetic predispositions should be considered for a patient with Dermatitis Herpetiformis and a family history of celiac disease?
Consider genetic predispositions such as HLA-DQ(a10501, B102) or HLA-DQ(a103, b10302), which are identical to those in celiac disease.
What does a distinct fibrillary pattern of IgA deposits indicate in a patient with Dermatitis Herpetiformis?
A distinct fibrillary pattern of IgA deposits indicates a variant presentation of DH, confirmed by Direct Immunofluorescence (DIF).
What other common sites should be examined for lesions in a patient with Dermatitis Herpetiformis who has symmetric lesions on their buttocks and sacral area?
Examine the elbows, knees, and shoulders, as these are common sites for DH lesions.
What is the typical course and prognosis of Dermatitis Herpetiformis?
The typical course of DH is waxing and waning, and it may persist indefinitely. Patients may be symptom-free for up to a year without treatment.
What other gastrointestinal conditions are commonly associated with Dermatitis Herpetiformis?
Other gastrointestinal conditions commonly associated with DH include celiac disease and pernicious anemia.
What is the likely connection between Dermatitis Herpetiformis and non-Hodgkin lymphoma (NHL)?
The connection is that DH is associated with an increased risk of malignancies, including non-Hodgkin lymphoma (NHL) and gastrointestinal lymphoma.
What other neurologic conditions might be associated with Dermatitis Herpetiformis in a patient with a history of epilepsy?
Other neurologic conditions associated with DH include ataxia, opsoclonus-myoclonus, and dementia.
What histological technique was used to identify eosinophils and fibrin in the dermal papillae of a patient with Dermatitis Herpetiformis?
The histological technique used was Hematoxylin and Eosin (H&E) staining.
What is the typical dosage of sulfapyridine for a patient with Dermatitis Herpetiformis, and in what scenarios is this medication preferred?
The typical dosage of sulfapyridine is 1-1.5 g daily.
It is preferred in patients intolerant to dapsone, elderly patients, or those with cardiopulmonary problems.
What is the likely explanation for bone loss in a patient with Dermatitis Herpetiformis who has a gluten-free diet?
The likely explanation is untreated celiac disease, which is associated with an increased frequency of bone loss.
What is the characteristic grouping of lesions in Dermatitis Herpetiformis?
The characteristic grouping of these lesions is herpetiform.
What is the significance of localized stinging, burning, and itching that occurs 8 hours before a skin eruption in a patient with Dermatitis Herpetiformis?
This symptom indicates localized stinging, burning, and itching, which are common prodromal symptoms of DH, occurring 8-12 hours before the eruption.
What other histological findings are characteristic of Dermatitis Herpetiformis besides subepidermal vesicles?
Other histological findings include dermal papillary collections of neutrophils (microabscesses), neutrophilic fragments, eosinophils, and fibrin.
What is the most reliable diagnostic criterion for Dermatitis Herpetiformis?
The most reliable diagnostic criterion for DH is granular IgA deposits in normal-appearing skin, identified by Direct Immunofluorescence (DIF).
What does blister formation within the lamina lucida suggest about the dermoepidermal junction in a patient with Dermatitis Herpetiformis?
This finding suggests that the lamina lucida is the most vulnerable component of the dermoepidermal junction (DEJ).
What is the rationale behind prescribing a gluten-free diet for a patient with Dermatitis Herpetiformis?
The rationale is that DH is associated with gluten-sensitive enteropathy (celiac disease), and a gluten-free diet helps manage symptoms and prevent complications.
What other autoimmune conditions should be considered in a patient with Dermatitis Herpetiformis and a history of thyroid disease?
Consider insulin-dependent diabetes, lupus, Sjögren’s syndrome, and vitiligo.
What cells are typically involved in microabscesses found in the dermal papillae of a patient with Dermatitis Herpetiformis?
The cells typically involved in these microabscesses are neutrophils.
What other histological features are commonly seen in Dermatitis Herpetiformis besides eosinophils in the dermal papillae?
Other histological features include dermal papillary collections of neutrophils (microabscesses), neutrophilic fragments, fibrin, and subepidermal vesicles.
What is the significance of finding granular IgA deposits in the skin of a patient with Dermatitis Herpetiformis?
This finding is the most reliable diagnostic criterion for DH, identified by Direct Immunofluorescence (DIF).
What diagnostic technique is used to confirm blister formation above the lamina densa in a patient with Dermatitis Herpetiformis?
Indirect Immunofluorescence (IIF) is used to confirm blister formation above the lamina densa.
What alternative medications can be considered for a patient with Dermatitis Herpetiformis who experiences side effects from dapsone?
Alternative medications include Sulfapyridine (1-1.5 g daily) and Sulfoxone (diazone).
What underlying condition might explain neurologic symptoms such as opsoclonus-myoclonus in a patient with Dermatitis Herpetiformis?
The underlying condition might be untreated celiac disease, which is associated with DH and neurologic symptoms.
What other gastrointestinal conditions are commonly associated with Dermatitis Herpetiformis besides atrophic gastritis?
Other gastrointestinal conditions commonly associated with DH include celiac disease and pernicious anemia.
What is the likely connection between Dermatitis Herpetiformis and gastrointestinal lymphoma?
The connection is that DH is associated with an increased risk of malignancies, including gastrointestinal lymphoma.
