Test 1.2 Flashcards

Question

What type of publication is StatPearls?

Answer 1

An online medical resource ## Footnote It provides updated medical information and reviews.

Answer 2

* Morren MA * Legius E * Giuliano F * Hadj-Rabia S * Hohl D * Bodemer C ## Footnote This article discusses new therapies in the field.

Answer 3

Abnormal sweating ## Footnote This syndrome includes several dermatological abnormalities.

Answer 4

Reticulate hyperpigmentation, non cicatricial alopecia, onychodystrophy ## Footnote These features help in the diagnosis of the condition.

Answer 5

An autoimmune blistering dermatosis occurring in elderly patients characterized by oral and cutaneous bullae; high mortality rate. ## Footnote Affects mainly individuals aged 50-60 years, with a higher prevalence in Jewish and Mediterranean populations.

Answer 6

HLA DR4 & 6; Drugs: captopril, penacillamine, rifampicin, NSAIDs, cephalosporins Environmental factors, such as diet, stress, viral infections, medications, ultraviolet radiation, ionizing radiation therapy, pesticides, and allergens, may all induce immune dysregulation leading to a flare of PV. ## Footnote These factors can trigger the autoimmune response leading to the condition

Answer 7

Anti-DSG3 > DSG1 antibodies. ## Footnote DSG refers to desmoglein, a protein that plays a crucial role in cell adhesion within the skin.

Answer 8

Flaccid bullae > ulcers > enlarge > crusted > slow healing > no scarring. Mucosal PV can be found in the conjunctiva, nasal mucosa, larynx, pharynx, esophagus, penis, vagina, and anus. Cutaneous lesions are commonly found on the face, trunk, groin, scalp, and armpits. PV usually spares the palms and soles.[57] Blisters can heal without scarring but may result in changes in the pigment. Alopecia may be observed when PV affects the scalp.[58] Rarely, PV will involve nails when the disease is severe.[19][59] Another rare manifestation of PV is pemphigus herpetiformis, where PV can present with urticarial plaques and cutaneous vesicles arranged in a herpetiform or annular arrangement. Go ## Footnote Commonly affects the oral cavity (60% of cases) as well as the face, neck, flexures, trunk, and limbs.

Answer 9

These Early lesions of pemphigus vulgaris show suprabasal epidermal acantholysis, clefting and blister formation. The blister cavity - eosinophils and rounded acantholytic cells with intensely eosinophilic cytoplasm and a perinuclear halo. The floor of the blister may be lined with intact keratinocytes, the “tombstone pattern” (figures 1, 2). Acantholysis can also affect adnexae. Dermal changes include perivascular inflammatory infiltrate particularly with eosinophils. Direct immunofluorescence may be positive in perilesional skin with intercellular deposits of IgG and/or C3 in the epidermis (figures 3, 4). Antigen deposition can be seen in hair follicles (follicular outer root sheath and germinal matrix), meaning direct immunofluorescence may be positive when performed on plucked hair

Answer 10

Steroids (1mg/kg/day); AZA; MMF; Cyclophosphamide; Cyclosporine; Rituximab. ## Footnote Treatment may involve pulse therapy with dexamethasone or methylprednisolone plus cyclophosphamide.

Answer 11

Pemphigus vegetans is rare variant of pemphigus vulgaris with the same target antigen (desmoglein 3). While pemphigus vegetans also features flaccid bullae and oral ulceration, these lesions develop into chronic vegetative plaques that tend to form on intertriginous skin. Further, patients presenting with pemphigus vegetans can progress to pemphigus vulgaris or vice versa ## Footnote It has two subtypes: Neumann type (classic) and Hallopeau type (localized).

Answer 12

Anti-DSG1 antibodies leading to acantholysis in the upper epidermis. ## Footnote This condition is characterized by superficial blisters that rapidly break.

Answer 13

Flaccid bullae > superficial ulcers > flaking > cornflake crust > exfoliative erythroderma. ## Footnote Patients typically present with burning, pain, and pruritus.

Answer 14

A form of endemic pemphigus foliaceous presenting as superficial crusting on face and neck in young patients. ## Footnote It is often seen in Brazil and Colombia and is triggered by genetic and arthropod factors.

Answer 15

It is a photo-aggravated form affecting mostly head and neck with anti-DSG1 antibodies. ## Footnote Clinical features include bullae and crusting in sun-exposed areas.

Answer 16

Blistering dermatosis associated with underlying hematological malignancies. ## Footnote Common neoplasms include Castleman’s disease, NHL, CLL, and thymomas.

Answer 17

Steroids AND/OR Dapsone > MMF. ## Footnote IgA pemphigus presents with pustules or vesicles in a serpiginous pattern.

Answer 18

DIF, ELISA, histological examination. ## Footnote DIF shows IgG deposits, while ELISA can detect specific antibodies like Anti-DSG1 and Anti-DSG3.

Answer 19

True. ## Footnote It presents with a herpetiform pattern of small grouped vesicles.

Answer 20

Topical steroids, Dapsone, nicotinamide, doxycycline. ## Footnote These treatments can vary in severity of the condition.

Answer 21

A rare subtype of pemphigus vulgaris characterized by vegetative plaques composed of excessive granulating tissue and crusting.

Answer 22

1-2% of all pemphigus cases.

Answer 23

At intertriginous sites, face, and scalp.

Answer 24

Hyperkeratosis, pseudoepitheliomatous hyperplasia, and papillomatosis with acantholysis creating a suprabasal cleft.

Answer 25

Pemphigus vegetans of Neumann (vegetative plaques arising from pemphigus vulgaris lesions) and Pemphigus vegetans of Hallopeau (plaques not preceded by bullae).

Answer 26

A rare subtype of pemphigus vulgaris characterized by urticarial plaques and vesiculobullous eruption in a herpetiform or annular pattern.

Answer 27

Plaques are associated with severe pruritus and rarely involve the mucosa.

Answer 28

Eosinophilic spongiosis, mostly without acantholysis.

Answer 29

Extensive, life-threatening erosions, secondary bacterial infection, fungal infection (especially candida), viral infection (especially herpes simplex), nutritional deficiencies due to difficulty eating, and complications of systemic steroids (especially infections and osteoporosis).

Answer 30

Group of diseases characterized histologically by presence of eosinophils in inflammatory infiltrate and/or evidence of eosinophilic degranulation ## Footnote Eosinophilic diseases often relate to allergic reactions, infections, and other inflammatory processes.

Answer 31

* Arthropod bites * Drug eruptions * Allergic contact dermatitis * Atopic dermatitis * Auto-immune blistering disorders * Parasitic infections * Urticarial vasculitis * Churg-Strauss syndrome * Urticarial allergic eruption * Granuloma faciale ## Footnote These causes encompass a range of environmental, allergic, and infectious triggers.

Answer 32

Middle aged Caucasian men ## Footnote This demographic is most commonly affected, but eosinophilic diseases can occur in various populations.

Answer 33

* Unknown * Immune complexes * IMF: IgG, IgA, IgM, C3 in blood vessel wall * Role for Interferon gamma (pro-inflammatory mediator) * IL-5 production ## Footnote These factors contribute to the development and persistence of eosinophilic inflammation.

Answer 34

* Solitary + asymptomatic * Smooth, red-brown to violaceous plaque on face * Prominent follicular openings * Multiple papules and plaques possible * Lesions predominantly on face * Occasionally extrafacial sites: trunk, extremities, sinosoidal variant eosinophilic angiocentric fibrosis

Answer 35

* Epidermis usually not affected * Dermis shows dense, nodular, diffuse polymorphous infiltrate of lymphocytes, neutrophils, plasma cells * Numerous eosinophils (characteristic) * Prominent Grenz zone ## Footnote The pathology often reveals a characteristic eosinophilic infiltrate in the dermis.

Answer 36

* Lymphoma * Pseudolymphoma * Sarcoidosis * Tumid lesions of lupus erythematosus * Fixed drug eruption * Rhinophyma * Other granulomatous diseases (leprosy, foreign body granuloma) ## Footnote These conditions can mimic eosinophilic diseases and require careful evaluation.

Answer 37

IL corticosteroids ## Footnote While corticosteroids are the mainstay, treatment may vary based on individual patient response.

Answer 38

* Dapsone * Clofazimine * Topical calcineurin inhibitors * PUVA ## Footnote These alternatives may be considered in cases resistant to standard therapies.

Answer 39

Widespread, symmetrical, pruritic eruption – flat topped, red-brown papules on trunk + extremities Spare skin folds - deck chair sign ## Footnote This condition is particularly noted in elderly Japanese men.

Answer 40

Diffuse dermal infiltrate of eosinophils and characteristic flame figures ## Footnote Flame figures are formed by eosinophil granule proteins coating collagen fibers.

Answer 41

* Hypereosinophilia (>1.5 x 10^9 eosinophils on FBC on 2 examinations >1 month apart) * Eosinophils in bone marrow >20% of all nucleated cells * Extensive tissue infiltration by eosinophils * Evidence of organ or tissue damage attributable to tissue hypereosinophilia * Exclusion of other disorders as major reasons for organ damage ## Footnote These criteria help differentiate HES from other eosinophilic disorders.

Answer 42

* Primary/Neoplastic (HESN) * Myeloid variants (M-HES) * T-cell lymphocytic variants (L-HES) * Secondary/Reactive (HESR) * Familial HES (HESFA) * Idiopathic (IHES) ## Footnote Each variant has distinct underlying causes and implications for treatment.

Answer 43

* Eczema * Erythroderma * Skin lichenification * Dermographism * Urticaria * Pruritic erythematous macules, papules, plaques, nodules on trunk + extremities ## Footnote Dermatologic signs are often prominent in patients with HES.

Answer 44

* Signs of heart failure (dyspnea, chest pain, cough, palpitations) * Pericarditis * Myocardial ischemia * Intracardiac thrombus *endomyocardial fibrosis (most common cause of death) ## Footnote Cardiac involvement can lead to serious complications, including restrictive cardiomyopathy.

Answer 45

* FBC with differential * Comprehensive blood chemistries * Liver enzymes * Renal function tests * Creatine kinase * Troponin levels history, physical examination, and blood work to evaluate for organ involvement and exclude conditions that cause eosinophilia, including infection, asthma, malignancy, atopy, rheumatologic conditions, and medications. If diagnosis of HES is suspected, evaluation of the heart, lungs, and hematologic system, such as peripheral blood T-lymphocyte phenotyping using flow cytometry and T-cell receptor rearrangement studies, should be performed. Bone marrow should be assessed for cellularity, dysplasia, CD34 expression, reticulin fibrosis, mast cells, and karyotype. Molecular studies for FGFR1, PDGFRA, and PDGFRB fusion genes—BCR-ABL1, JAK2 V617F, KIT, D816V—and clonal T-cell receptor rearrangements can be performed ## Footnote These tests help assess the extent of eosinophilia and any potential organ damage.

Answer 46

FBC with differential, comprehensive blood chemistries, liver enzymes, renal function tests, creatine kinase, and troponin levels ## Footnote Additional studies include electrocardiogram, echocardiogram, pulmonary function tests, chest radiography, CT of the chest and abdomen, and tissue biopsies if indicated.

Answer 47

To investigate the underlying cause of HES ## Footnote This includes tests like FISH for CHIC2 deletion and RT-PCR for FIP1L1-PDGFRA mutation.

Answer 48

* FISH for CHIC2 deletion * RT-PCR for FIP1L1-PDGFRA mutation * Flow-cytometry for T-lymphocyte phenotypes * Bone marrow aspiration and biopsy * Molecular studies for fusion genes and mutations ## Footnote Tests for specific T cell clonality and karyotyping may also be included.

Answer 49

Increased serum B12 and FIP1L1-PDGFRA fusion genes ## Footnote Other findings may include anemia, thrombocytopenia, elevated serum tryptase, and hepatosplenomegaly.

Answer 50

Dermatologic symptoms may be present, with potential involvement of other organs ## Footnote Molecular testing may reveal abnormal T cell markers.

Answer 51

Eosinophilic granulomatosis with polyangiitis (EGPA) ## Footnote The key distinguishing factor is the presence of vasculitis in EGPA.

Answer 52

* Reducing eosinophil count * Improving disease symptoms * Preventing disease progression ## Footnote Treatment depends on the underlying cause and disease severity.

Answer 53

An absolute eosinophil count of 100 x 10^9/L (>100,000 cells/µL) with signs of hyperleukocytosis ## Footnote Symptoms may involve the nervous and pulmonary systems.

Answer 54

High-dose intravenous prednisone 1 mg/kg/day or 1 g/day of methylprednisolone ## Footnote Strongyloides serology should be tested before initiating treatment.

Answer 55

Potential intracranial hemorrhage ## Footnote This risk may persist up to a week after treatment due to reperfusion injury.

Answer 56

Systemic glucocorticoids ## Footnote A multicenter analysis showed 85% achieved partial or complete symptom remission within 1 month.

Answer 57

Re-evaluate and initiate treatment with imatinib ## Footnote Treatment for secondary HES should target the underlying cause.

Answer 58

Inactivation of downstream phosphorylation by occupying the kinase pocket ## Footnote This affects genes such as abl, c-kit, PDGFRA, and PDGFRB.

Answer 59

Hydroxyurea ## Footnote It targets eosinophil development and can be used as monotherapy or in combination with prednisone.

Answer 60

Interferon-α (IFN-α) ## Footnote It has a broad side effect profile which can complicate treatment.

Answer 61

* Leukemias * Irreversible heart failure * Endocarditis * Severe restrictive cardiomyopathy ## Footnote These complications arise from the type and degree of end-organ involvement.

Answer 62

Advancements in earlier detection of complications and enhanced surgical management ## Footnote A broader array of therapeutic modalities targeting the underlying cause of HES also contributes.