dermatitis herpetiformis; Duhring-Brocq disease
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Introduction
A chronic symmetric, itching vesiculobullous dermatitis.
Etiology
- ingestion of iodides may precipitate symptoms
- gluten-sensitive enteropathy (celiac sprue) in nearly all patients (generally asymptomatic)
- small bowel malabsorption occurs in 10-20%
Epidemiology
- onset 20-60 years, most commonly 30-40, but may occur in children
- male:female ratio 2:1
- 10-11 cases/100,000/year
Pathology
- microabscesses at the tips of the dermal papillae
- neutrophils
- eosinophils
- results in subepidermal separation[5]
- fibrin accumulation & necrosis
- dermal infiltration of neutrophils & eosinophils
- subepidermal vesicle
- IgA deposits in affected & normal-appearing skin
- granular in tips of papillae
- correlates with small bowel disease
- found in majority of patients
- complement also deposited
- linear, bandlike along dermal-epidermal junction
- other bullous diseases may have IgG in band-like pattern beneath the epidermal basement membrane
- pattern not associated with small bowel disease
- pattern in minority of patients
- IgA associated with microfibrils
- IgA & complement mediate cascade of events leading to tissue injury
- IgA antibodies to transglutaminase (TG3)[6]
- granular in tips of papillae
* histopathology images[11]
Genetics
- IL31 is up-regulated in lesional biopsies of patients with allergic contact dermatitis
Clinical manifestations
- intense, episodic pruritus
- erythematous papules, vesicles, bullae (occasionally)
- urticaria-like wheal
- scratching results in excoriations with crusting; may obscure visualization of vesicles or bullae[5]
- post-inflammatory hyperpigmentation at sites of healed lesions
- lesions arranged in groups (crops), symmetrically distributed
- sites of predilection:
- does not involve oral mucosa[5]
Laboratory
- eosinophilia
- HLA typing: association with HLA-B8, HLA-DR3 & HLA-DQ2
- serology:
- circulating antibodies to basement membrane generally not detectable
- IgG & IgA anti-reticulin antibodies may be present
- antimicrosomal antibodies may be present
- antinuclear antibodies may be present
- circulating immune complexes in 20-100%
- IgA anti-endomysial antibodies
- bind to intermyofibril substance of smooth muscle
- present in the majority of patients
- correlate with severity of intestinal disease
- IgA antibodies to transglutaminase (TG2, TG3)[6]
- malabsorption studies
- steatorrhea (20-30%)
- abnormal D-xylose absorption (10-73%)
- anemia secondary to iron or folate deficiency
- erythrocyte glucose-6-phosphate dehydrogenase (erythrocyte G6PD) level prior to treatment with sulfones
- skin biopsy
- best from early erythematous papule
- immunofluorescence of normal-appearing skin
- see ARUP consult[7]
Diagnostic procedures
- upper GI endoscopy
- blunting & flattening of the villi (80-90%)
- small bowel biopsy
Complications
Differential diagnosis
- allergic contact dermatitis
- atopic dermatitis
- scabies
- neurotic excoriations
- papular urticaria
- bullous pemphigoid
- herpes gestationis
Management
- dapsone 100-200 mg QD with taper to 25-50 mg QD
- measure serum G6PD prior to treatment with dapsone
- relieves pruritus & clears skin lesions in 24-48 hours by blocking IgA-mediated neutrophil chemotaxis
- follow CBC weekly for 1st month, then every 6-8 weeks
- sulfapyridine 1.0-1.5 g QD with fluids, if dapsone contraindicated or not tolerated
- gluten-free diet (1st line)[5]
- may completely suppress symptoms or allow reduction of dapsone or sulfapyridine
- response to gluten-free diet is slow
- prognosis
More general terms
Additional terms
- atopic dermatitis (atopic eczema)
- bullous pemphigoid; parapemphigus
- celiac sprue (gluten-sensitive enteropathy)
- contact dermatitis (exogenous eczema)
- scabies
- urticaria (hives)
References
- ↑ Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 878
- ↑ Color Atlas and Synopsis of Clinical Dermatology, Common and Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 325-27
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 168
- ↑ 5.0 5.1 5.2 5.3 5.4 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2021.
- ↑ 6.0 6.1 6.2 Hull CM et al. Elevation of IgA anti-epidermal transglutaminase antibodies in dermatitis herpetiformis. Br J Dermatol 2008 Jul; 159:120. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18503599
- ↑ 7.0 7.1 ARUP Consult: Dermatitis Herpetiformis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/dermatitis-herpetiformis
- ↑ Zone JJ. Skin manifestations of celiac disease. Gastroenterology. 2005 Apr;128(4 Suppl 1):S87-91. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15825132
- ↑ 9.0 9.1 Hervonen K, Vornanen M, Kautiainen H, Collin P, Reunala T Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives. Br J Dermatol. 2005 Jan;152(1):82-6. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15656805
- ↑ Karpati S Dermatitis herpetiformis. Clin Dermatol. 2012 Jan-Feb;30(1):56-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22137227
- ↑ 11.0 11.1 11.2 Miller JL, Elston DM (images) Medscape: Dermatitis Herpetiformis http://emedicine.medscape.com/article/1062640-overview
- ↑ 12.0 12.1 DermNet NZ. Dermatitis herpetiformis (images) http://dermnetnz.org/immune/dermatitis-herpetiformis.html
- ↑ Jakes AD, Bradley S, Donlevy L. Dermatitis herpetiformis. BMJ. 2014 Apr 16;348:g2557. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24740905
- ↑ Reunala T, Hervonen K, Salmi T Dermatitis Herpetiformis: An Update on Diagnosis and Management Am J Clin Dermatol. 2021 May;22(3):329-338 PMID: https://www.ncbi.nlm.nih.gov/pubmed/33432477 PMCID: PMC8068693 Free PMC article