Neutrophilic Dermatoses II

Sweetsie Pie

100

In a patient with history of malignancy associated Sweets Syndrome, what does re-appearance of the dermatosis mean?

Possible recurrence of malignancy.

200

Name 3 systemic treatments for Sweets Syndrome.

Systemic corticosteroids, potassium iodide, dapsone, and colchicine.

300

What is the recurrence rate of Sweet’s syndrome?

30% (with or w/o tx). 50% in immunocompromised.

400

Name 2 Major criteria and 2 Minor Criteria for Sweets Syndrome.

Major: (1) Abrupt onset of painful juicy plaques, (2) Histopath showing dense neutrophilic infiltrate without LCV. Minor: (3) Fever, (4) Association with malignancy (AML, GU, breast, GI), IBD, Pregnancy, Infection (URI, GI), or vaccination. (5) Rapid response to systemic steroid or KI, (6) Abnormal labs – ESR, CRP, WBC, neutrophilia.

500

In histiocytoid sweets, what do the histiocytoid cells stain with?

MPO (myeloperoxidase)

You down with Pyo-G, yea you know me!

100

What is PAPA syndrome and what is its gene?

Pyogenic Arthritis, Pyoderma gangrenosum, Acne PSTPIP1 gene that encodes CD2 binding protein 1. Auto Dominant.

200

What type of cell is found at the edge of the PG ulcer?

T cells.

300

In a patient with cutaneous PG, where else can you get sterile neutrophilic abscesses?

Lung, joints, CNS, CVS, intra-abdominal viscera, eye

400

What type of monoclonal gammopathy are neutrophilic dermatoses most commonly asst with?

IgA monoclonal gammopathy

500

Name several entities associated with an IgA gammopathy.

Sneddon Wilkinson, Sweet’s, PG, EED, IgA Pemphigus, POEMS.