Eosinophilic Dermatoses

Question 1

What is the presentation of granuloma faciale?

Answer 1

Multiple red-brown papules, plaques or nodules. Usually on the face, especially the central face (nose, malar prominence, forehead and ear)

Can also have a follicular prominence, telangiectasias or a “peau d’ orange” look to it

Question 2

What is the histopathology of granuloma faciale?

Answer 2

Remember that this looks like EED (some think it is the same/similar entity)

• Look for LCV (may be difficult to see… smoldering)
• Look for mixed dermal infiltrate of eos, neuts, and lymphs w/ plasma cells and fibrosis

Question 3

What is the treatment for granuloma faciale?

Answer 3

Treatment is limited, consider intralesional triamcinolone as the first-line therapy

• Can also try cryotherapy, topical steroids, topical calcineurin inhibitors
• Systemic: can consider dapsone, colchicine, or hydroxychloroquine

Question 4

What is the presentation of Well’s syndrome (eosinophilic cellulitis)?

Answer 4

Recurrent burning, pruritic or painful pink to red edematous plaques that favor extremities –> become more indurated and brown or slate-gray overtime

Can have eosinophilia

Question 5

What things can trigger Well’s syndrome or be associated with it?

Answer 5

Arthropod, parasitic infection, (?) myeloproliferative dz, eosinophilic granulomatosis with polyangiitis

Question 6

What is the histopathology of Well’s syndrome?

Answer 6

Massive and diffuse dermal eosinophilic infiltrate, some degranulated, which leads to flame figures

Question 7

What is the treatment for Well’s syndrome?

Answer 7

-Systemic steroids lead to rapid resolution (eosinophilic conditions are sensitive to steroids)

Question 8

Who is papuloerythroderma of Ofuji most common in?

Answer 8

Elderly men, like to test on Japanese ancestry

Question 9

Clinical presentation of papuloerythroderma of Ofuji?

Answer 9

Generalized pruritic red-brown papules –> erythroderma sparing the skin folds (gives the “deck chair sign”)

Question 10

What are the lab abnormalities associated with Papuloerythroderma of Ofuji?

Answer 10

Eosinophilia, lymphopenia, elevated IgE, lymphadenopathy is common

Question 11

What diseases can be associated with Papuloerythroderma of Ofuji?

Answer 11

Malignancy: gastric carcinoma, B-cell lymphoma and T-cell lymphomas

Question 12

Treatment for Papuloerythroderma of Ofuji?

Answer 12

Responds to systemic steroids, PUVA, or oral retinoids

Tends to be a chronic condition until it remits. Also, don’t forget to work up for underlying malignancy.

Question 13

What should be considered if you have a patient with particularly exaggerated responses to insect bites (pruritic, erythematous, edematous papulonodules, and vesicobullae)?

Answer 13

CLL or less commonly other myeloproliferative disorders

Question 14

What tumor may be associated with exuberant bite reactions to mosquito bites?

Answer 14

Chronic lymphocytic lymphoma (CLL) is most often tested, can also consider underlying EBV infection/activation as well

Question 15

What is the definition of hypereosinophilic syndrome?

Answer 15

Eosinophilia (>1500 eosinophils/ul) x 6 months or <6 months if a/w end-organ damage

Question 16

What are the clinical presentations of hypereosinophilic syndrome?

Answer 16

• Skin lesions in 50%
• Most common = itchy red papules and nodules, urticaria and angioedema
• Ulcers on mucosal surfaces

Question 17

What should be expected if mucosal ulcers are seen with hypereosinophilic syndrome?

Answer 17

Myeloproliferative dz and more aggressive course

Question 18

What are the systemic symptoms of hypereosinophilic syndrome?

Answer 18

• Fever, cough, malaise, and myalgias are common.
• Can also have cutaneous and mucocutaneous ulcers that are polymorphic in nature.

Question 19

What is the most common cause of death in hypereosinophilic syndrome?

Answer 19

Congestive heart failure (5-year survival = ~80%)

Question 20

How does eosinophil count correlate with disease in hypereosinophilic syndrome?

Answer 20

Decreasing cell counts correspond to treatment effect

Question 21

What are the two major subtypes of hypereosinophilic syndrome?

Answer 21

Myeloproliferative and lymphocytic

Question 22

What is the most common gene mutation in myeloproliferative hypereosinophilic syndrome?

Answer 22

A fusion of FIP1L1-PDGFRA genes leading to constitutively activated tyrosine kinase

Question 23

What is the cause of the lymphocytic hypereosinophilic syndrome?

Answer 23

Clonal T-cell proliferation with T-cell gene rearrangement –> increased Th2 cytokine production, especially IL-5 –> eosinophil activation

Question 24

What treatments are available for hypereosinophilic syndrome?

Answer 24

• Myeloproliferative form with FIP1L1-PDGFRA mutation use imatinib or other TKIs
• Lymphoproliferative form use systemic steroids +/- mepolizumab (anti-IL-5 mAb)

*Prednisone first line for both as well

• Other treatments include hydroxyurea, interferon

Question 25

What findings are seen in myeloproliferative hypereosinophilic syndrome?

Answer 25

Most common in males and a/w endomyocardial fibrosis/cardiomyopathy, hepatosplenomegaly

• CD25 (+) mast cells from BM biopsy
• Elevated serum tryptase and vitamin B12 (not always present)

Question 26

What is the clinical presentation of lymphocytic hypereosinophilic syndrome?

Answer 26

Itch/eczema/erythroderma/angioedema/urticaria w/ increased IgE, eosinophilia, and lymphadneopathy

Question 27

What is the difference in prognosis between the myeloproliferative and lymphocytic hypereosinophilic syndromes?

Answer 27

Lymphocytic is generally more benign, can still have cardiac complications and there is increased risk of T-cell lymphoma