Eosinophilic Dermatoses Flashcards
What is the presentation of granuloma faciale?
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
What is the histopathology of granuloma faciale?
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
What is the treatment for granuloma faciale?
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
What is the presentation of Well’s syndrome (eosinophilic cellulitis)?
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
What things can trigger Well’s syndrome or be associated with it?
Arthropod, parasitic infection, (?) myeloproliferative dz, eosinophilic granulomatosis with polyangiitis
What is the histopathology of Well’s syndrome?
Massive and diffuse dermal eosinophilic infiltrate, some degranulated, which leads to flame figures
What is the treatment for Well’s syndrome?
-Systemic steroids lead to rapid resolution (eosinophilic conditions are sensitive to steroids)
Who is papuloerythroderma of Ofuji most common in?
Elderly men, like to test on Japanese ancestry
Clinical presentation of papuloerythroderma of Ofuji?
Generalized pruritic red-brown papules –> erythroderma sparing the skin folds (gives the “deck chair sign”)
What are the lab abnormalities associated with Papuloerythroderma of Ofuji?
Eosinophilia, lymphopenia, elevated IgE, lymphadenopathy is common
What diseases can be associated with Papuloerythroderma of Ofuji?
Malignancy: gastric carcinoma, B-cell lymphoma and T-cell lymphomas
Treatment for Papuloerythroderma of Ofuji?
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.
What should be considered if you have a patient with particularly exaggerated responses to insect bites (pruritic, erythematous, edematous papulonodules, and vesicobullae)?
CLL or less commonly other myeloproliferative disorders
What tumor may be associated with exuberant bite reactions to mosquito bites?
Chronic lymphocytic lymphoma (CLL) is most often tested, can also consider underlying EBV infection/activation as well
What is the definition of hypereosinophilic syndrome?
Eosinophilia (>1500 eosinophils/ul) x 6 months or <6 months if a/w end-organ damage
What are the clinical presentations of hypereosinophilic syndrome?
- Skin lesions in 50%
- Most common = itchy red papules and nodules, urticaria and angioedema
- Ulcers on mucosal surfaces
What should be expected if mucosal ulcers are seen with hypereosinophilic syndrome?
Myeloproliferative dz and more aggressive course
What are the systemic symptoms of hypereosinophilic syndrome?
- Fever, cough, malaise, and myalgias are common.
- Can also have cutaneous and mucocutaneous ulcers that are polymorphic in nature.
What is the most common cause of death in hypereosinophilic syndrome?
Congestive heart failure (5-year survival = ~80%)
How does eosinophil count correlate with disease in hypereosinophilic syndrome?
Decreasing cell counts correspond to treatment effect
What are the two major subtypes of hypereosinophilic syndrome?
Myeloproliferative and lymphocytic
What is the most common gene mutation in myeloproliferative hypereosinophilic syndrome?
A fusion of FIP1L1-PDGFRA genes leading to constitutively activated tyrosine kinase
What is the cause of the lymphocytic hypereosinophilic syndrome?
Clonal T-cell proliferation with T-cell gene rearrangement –> increased Th2 cytokine production, especially IL-5 –> eosinophil activation
What treatments are available for hypereosinophilic syndrome?
- 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
What findings are seen in myeloproliferative hypereosinophilic syndrome?
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)
What is the clinical presentation of lymphocytic hypereosinophilic syndrome?
Itch/eczema/erythroderma/angioedema/urticaria w/ increased IgE, eosinophilia, and lymphadneopathy
What is the difference in prognosis between the myeloproliferative and lymphocytic hypereosinophilic syndromes?
Lymphocytic is generally more benign, can still have cardiac complications and there is increased risk of T-cell lymphoma
