Dermatitis Herpetiformis
What percentage of patients with dermatitis herpetiformis have GI symptoms at time of diagnosis?
Bonus: Where is the ideal spot to biopsy for DIF?
20%- although >90% have gluten sensitive enteropathy
Bonus: 1 cm away from blister
A salt-split skin is performed on a biopsy from a patient with suspected LABD. What side of the split most commonly stains on IIF?
Epidermal side
How long does it take for pruritus to improve after initiating dapsone treatment?
Symptoms will abate in 48-72 hours
A 2 day old neonate develops blotchy erythematous macules with a small central pustules over the trunk and extremities.
What is the diagnosis? What anatomic site is most commonly spared?
Bonus: T/F- most neonates are premature
Erythema toxic neonatorum
Palms and soles
False - rarely occurs in premies or infants <2500g
In erythema toxic neonatorum, a giemsa or wright stain of fluid from a pustule will demonstrate increased numbers of...?
Bonus: what will it show for transient neonatal pustular melanosis?
Eosinophils!
Neutrophils!
What is the DIF finding in dermatitis herpetiformis? How can you differentiate it from LABD?
DIF: granular IgA deposits in the dermal papillae (90%); granular IgA deposition along the BMZ (10%)
LABD: LINEAR deposits!
What is the most serious SE of Dapsone therapy? When does it occur? Explain the clinical symptoms and treatment.
Agranulocytosis
Sx: fever, pharyngitis, sepsis
Tx: Stop dapsone
A 52-year-old male with history of DH on dapsone presents to clinic with a 3-day history of cough, sore throat, sinus congestion, and O2 saturation of 87% on RA. On exam, the patient exhibits a mild bluish hue to his skin. What diagnostic test would be most useful in this patient?
A. Repeat pules ox as this like is a transient finding
B. Co-oximetry
C. Chest xray
D. Sputum culture
E. CT chest
What are the 4 stages of incontinentia pigmenti?
1. Vesicular
2. Verrucous
3. Hyperpigmented
4. Hypopigmented Atrophic
What is the inheritance pattern for incontinentia pigmenti (IP)?
Bonus: Gene?
X-linked dominant;
Bonus: IKBKG formerly NEMO – prevents apoptosis
Over 97% of dermatitis herpetiformis and celiac disease patients have one or both of the following HLA II alleles?
HLA-DQ2 (90%) and HLA-DQ8 (7%)
Name the top 5 most common drugs associated with LABD?
1. Vancomycin
2. Penicillins
3. Cephalosporins
4. Captopril > other ACEi
5. NSAIDS
Your patient is recently diagnosed with DH and is very reluctant to use any forms of systemic medication. You both agree to a strict gluten free diet. You make sure the patient understands that he should only expect to have results after several months. Which of the following grains is he allowed to include in his gluten free diet?
A. Kamut
B. Wheat
C. Oats
D. Barley
E. Rye
C. Oats
See photo - Slide #1
What is the diagnosis? Name the other 2 types. What is the cause? Where is the obstruction?
Miliaria crystallina
2 other types - Rubra, profunda
obstruction of eccrine sweat ducts - associated with overheating, over swaddling, fever, etc.
Crystallina- stratum corner, Rubra-spinosum, profunda - dermis
A 1 year old african american male presents to clinic for evaluation of an itchy rash on his palms and soles. Mother reports rash started when he was 3 months old. The rash appears in "clusters" every month or so. She notes he scratches frequently.
Slide #2 PIC
Acropustulosis of infancy
-idiopathic, onset 3-6 months
-More common in african American males
-post-scabetic variant
-appears in crops every 3-5 weeks, resolves 3 y.o.
-Need to r/o scabies
Tx: anti-Hi, TCS, dapsone
What is the most common autoimmune disease associated with dermatitis herpetiformis?
Hashimoto thyroiditis
In LABD, IgA ab is directed against ____ and ____ on ____ (BPXXX)
In LABD, IgA ab is directed against LAD-1 and LABD97 on BPAG2 (BP180)
What is the antibody found in celiac disease? In dermatitis herpetiformis?
CD: IgA antibodies against tissue transglutaminase 2
DH: IgA antibodies against epidermal transglutaminase 3
What are the three separate morphologies/stages that occur in transient neonatal pustular melanosis?
1. Vesiculopsutules
2. Hyperpigmented macule with collarette of seale
3. PIH
How do you differentiate between neonatal cephalic pustulosis and neonatal acne?
Neonatal cephalic pustulosis: earlier onset and shorter duration - onset 5 days to 3 weeks, resolves in weeks to months. NO COMEDONES. Distributed in areas other than the cheeks.
Infantile acne: Less common. Appears in 6 weeks to 1 year. Resolves in 6-18 months. Mainly located on the cheeks. Open and closed comedones. Can scar and requires treatment.
Patients with dermatitis herpetiformis are at risk for what malignancies?
GI lymphoma - mucosa-associated lymphoid tissue (MALT) also nonhodgkins lymphoma
What are the two sites of IgA deposition in LABD? Which is most common?
1. Lamina lucida (MC)
2. Sublamina densa
Ingestion of what can lead to worsening of DH?
What are the extracutaneous manifestations of incontentia pigmenti?
•CNS - seizures, delayed psychomotor development, spastic paralysis
•Ocular – Blindness, retinal vascular abnormalities
•Teeth- Hypodontia, conical teeth
Referral for periodic neurodevelopmental and dental evaluations
You diagnose an infant with LCH. Do you need to evaluate this patient for systemic involvement? If so, what do you order?
Yes! Physical exam, chemistry panel including LFTs, CBC w/ diff, urine osmolality, abdominal US, skeletal survey, chest X-ray. Referral to oncology for possible BM aspiration/bx.