Neut and Eos Derm Book Club I

Sweet's Syndrome I

100

Name 3 conditions associated with Classical Sweets Syndrome.

1. Infections (URI, GI) 2. Inflammatory Bowel Disease 3. Pregnancy

200

Which malignancy is most commonly associated with Sweets Syndrome?

AML (Acute Myelogenous Leukemia)

300

Which drug is most commonly associated with Drug Induced Sweets Syndrome?

G-CSF (Granulocyte Colony Stimulating Factor)

400

Which cytokines are implicated in the pathogenesis of Sweets Syndrome?

G-CSF, GM-CSF, IFNg, IL-1, IL-3, IL-6, and IL-8.

500

What else can you find on physical exam in patients with Sweets Syndrome and underlying hematologic disorder?

Mucosal ulcers.

Pyoderma Gangrenosum I

100

What systemic diseases are pyoderma gangrenosum associated with?

Inflammatory Bowel Disease – Ulcerative Colitis and Crohn’s disease, Arthritis, Hematologic disorder (IgA monoclonal gammopathy, AML, myelodysplasia), Malignancy

200

Which inflammatory bowel disease is PG more often associated with?

UC! Ulcerative Colitis (2%), Crohns (0.9%)

300

Name 4 types of PG and what systemic illness they are most commonly associated with.

Ulcerative – all Bullous – hematologic dyscrasia/malignancy Pustular – IBD Vegetative (superficial granulomatous) – CKD.

400

Where on the body do you most commonly see bullous PG and what systemic condition is it asst with?

Dorsal hands! Also face and upper extremities. It is also known as superficial bullous variant of Sweet’s. Occurs commonly with AML.

500

What is PG of mucosal surfaces called, what does it look like (buzz word), and what systemic condition is it asst with?

Pyostomatitis vegetans “snail tracks, snail trails” asst with IBD (UC>Crohns)

Granuloma Faciale

100

What is a classic finding on path for Granuloma Faciale, and in what other conditions can you see it?

Grenz zone can be seen in Granuloma faciale, Tumor stage MF, DF, Lepromatous leprosy, leukemia cutis, Merkel cell

200

What are the clinical findings of granuloma faciale?

Slight male predominance. Mean age – 52yo. Reddish brown papules/plaques on face. Soft, smooth, well circumscribed red/brown plaques with FOLLICULAR ACCENTUATION and telangiectasia.

300

What are the findings on path for granuloma faciale? LCV or no LCV?

Normal epi, Grenz zone, dense infil of lymphs, plasma cells, eos, and neuts, with LCV and perivascular fibrin deposition.

400

What is the primary histologic ddx for granuloma faciale?

EED – clinically has multiple lesions on extensor extremities, with sparing of trunk and face. On path, EED does not have a grenz zone and has rare eos or plasma cells. Has more neuts and also has a chronic fibrosing vasculitis.

500

Which laser and which systemic medication are considered first line treatments for granuloma faciale?

Pulsed dye laser (585-595nm) Dapsone Other first line = topical steroids, cryo, intralesional steroid. 2nd line = topical tacrolimus and excision

Subcorneal Pustular Dermatosis

100

Describe Sneddon’s syndrome?

Sneddon’s syndrome is different from Sneddon-Wilkinson Disease Sneddon’s syndrome is idiopathic livedo reticularis with hypertension and cerebrovascular accidents.

200

What systemic condition is SCPD most commonly associated with?

IgA monoclonal gammopathy.

300

What is the target antigen of SPD-type IgA pemphigus?

Desmocollin I

400

Describe clinical exam in pt with Sneddon Wilkinson and what is a classic feature?

Crops of flaccid, coalescing pustules in an annular or serpiginous pattern, distributed symmetrically in axillae, groin, submammary, flexor limbs, and abdomen. More common in women > 40yo. Classic feature is the HYPOPYON – pus accumulating in lower half of pustule due to gravity.

500

What is SAPHO syndrome, and what is the most common site of involvement?

A syndrome of painful osteoarticular lesions that can occur with any neutrophilic dermatosis (aseptic pustular dermatosis). SAPHO = Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis Most common sites are Anterior chest / Axial skeleton.

Not for Noobs!

100

What type of arthritis is characteristic in Behcet’s? Symmetric? Erosive?

Asymmetric non-erosive polyarthritis

200

Name 1 major and 2 minor criteria for Behcet’s disease.

Major: Recurrent aphthous ulcers (at least 3x/year) Minor: Recurrent genital ulcers Eye lesions (post uveitis, retinal vasculitis) Cutaneous lesions (E nodosum-like, papulopustular lesions, pseudofolliculitis, acneiform nodules) Pathergy test (inject saline and see papule/pustule 24-48h)

300

What HLA type is associated with Behcet’s disease?

HLA-B51

400

Would you want to be BAD-AS? Why not?

BADAS – Bowel Associated Dermatosis Arthritis Syndrome Seen in patients with intestinal bypass surgery or blind loops of bowel. Serum sickness like constitutional symptoms, Diarrhea/malabsorption, Arthritis, Skin lesions. Skin lesions – vesiculopustules, tender nodules on upper arms/trunk, or E nodosum on lower legs (all with neutrophilic infiltrates on path).

500

If you suspect SAPHO in an infant, what two autosomal recessive genodermatoses would you look for?

Majeed Syndrome (congenital dyserythripoietic anemia, LPIN2 mutation) DIRA syndrome (Deficiency of IL-1 Receptor Antagonist, IR1RN mutation)