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A child with fever had multiple skin lesions, an on microscopic examination the skin lesions are seen to have neutrophilic and histiocytic infiltration in the dermis. What is the diagnosis? (AIIMS Nov 2014)

A Sweet syndrome

B Erythema nodosum leprosum

C Erythema elevatum diumitinum

D Rosai Dorfman syndrome

Ans. A
Sweet’s Syndrome:

1). A constellation of symptoms and findings: the acute onset of fever, neutrophilia, erythematous, and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils.

2). Cardiovascular, central nervous system, gastrointestinal, hepatic, musculoskeletal, ocular, oral, otic, pulmonary, renal, and splenic organs can be involved.

3). Classic Sweet syndrome may be associated with infection of the upper respiratory and/or gastrointestinal tract, inflammatory bowel disease, and pregnancy.

4). Malignancy-associated Sweet syndrome occurs in individuals with previously undiagnosed or relapsing hematologic malignancies and solid tumors; in these patients, Sweet syndrome appears as a cutaneous paraneoplastic syndrome.

5). Drug-induced Sweet syndrome describes the onset of the dermatosis in patients after the initiation of certain medications—most commonly granulocyte-colony stimulating factor (G-CSF).

6). Cytokines may have an important role in the pathogenesis.

7). The first-line oral systemic agents for treating Sweet syndrome are corticosteroids, potassium iodide, and colchicine.

8). Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions.

9). Histopathology:

a. Typically, a dense perivascular infiltrate composed largely of neutrophils is seen assuming a band-like distribution throughout the papillary dermis.

b. Some of the neutrophils may show nuclear fragmentation (leukocytoclasis).

c. In addition, the infiltrate may contain scattered lymphocytes and histiocytes and occasional eosinophils.

d. The density of the infiltrate varies and may be limited in a small proportion of cases.

e. Vasodilation and swelling of endothelium with moderate erythrocyte extravasation, as well as prominent edema of the upper dermis, are characteristic.

f. In later stages, lymphocytes and histiocytes may predominate.

Erythema elevatum diutinum:
1). Rare disease; unknown incidence.

2). A chronic leukocytoclastic vasculitis typified by a distinctive clinical pattern.

3). Symmetric, erythematous, violaceous, or yellow-brown papules/nodules/plaques.

4). Most common sites of involvement are the extensor surfaces of the hands, fingers, elbows, knees, legs, and Achilles tendon. Trunk is usually spared.

5). Co-occurring diseases include monoclonal paraproteinemias, lymphoproliferative disorders, chronic infection, autoimmune conditions, and connective tissue diseases.

6). In the early stage of erythema elevatum diutinum (EED), nonspecific leucocytoclastic vasculitis is observed. In later stages, granulation tissue and fibrosis form with a diffuse mixed-cell infiltrate showing a predominance of neutrophils. The capillaries may still show deposits of fibrinoid material or merely fibrous thickening.

7). Dapsone- and sulfone-based therapies are considered first-line treatment choices for EED

Erythema Nodosum Leprosum:
1). Erythema nodosum leprosum (ENL) occurs most commonly in LL leprosy and less frequently in borderline lepromatous (BL) leprosy.

2). It may be observed not only in patients under treatment but also in untreated patients.

3). On the skin one observes tender, red plaques and nodules together with areas of erythema and occasionally also purpura and vesicles. Ulceration, however, is rare.

4). The eruption is widespread and is accompanied by fever, malaise, arthralgia, and leukocytosis.

5). New lesions appear for only a few days in some cases but for weeks and even years in others.

6). This is the only type of reactional leprosy that responds to treatment with thalidomide

7). Histopathology:

a. In erythema nodosum leprosum, the lesions are foci of acute inflammation superimposed on chronic multibacillary leprosy.

b. Polymorph neutrophils may be scanty or so abundant as to form a dermal abscess with ulceration.

c. Whereas foamy macrophages containing fragmented bacilli are usual, in some patients no bacilli remain and macrophages have a granular pink hue on Wade-Fite staining, indicating mycobacterial debris.

d. An antimycobacterial immunocytochemical stain (e.g., anti-BCG) will indicate abundant antigen.

e. A necrotizing vasculitis affecting arterioles, venules, and capillaries occurs in some cases of ENL; these patients may have superficial ulceration.

Rosai Dorfman disease:
1). Massive cervical lymphadenopathy, usually bilateral and painless, is the most common manifestation.

2). In most patients the disease resolves spontaneously, others have persistent problems, and very few die.

3). About 10% of patients with nodal disease have cutaneous involvement.

4). The lesions are typically papules or nodules. The skin and subcutaneous soft tissue are also the most common sites of involvement when patients present with extranodal disease.

5). Histopathology:

a. The skin lesions contain a polymorphous infiltrate in which lymphocytes and macrophages with clear cytoplasm are most prominent.

b. Occasionally they may be multinucleated or have a foamy cytoplasm.

c. The hallmark histologic feature is emperipolesis of lymphocytes. Emperipolesis differs slightly from phagocytosis in that the lymphocytes are taken up but seem not to be attacked and digested by enzymes. Thus, they appear intact. On occasion, red cells can also be taken up.

Vasculitis Flashcard List

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