CPC. Chutika Srisuttiyakorn, M.D. Kobkul Aunhachoke, M.D. Phramongkutklao Hospital Bangkok, Thailand

Transcription

1 CPC Chutika Srisuttiyakorn, M.D. Kobkul Aunhachoke, M.D. Phramongkutklao Hospital Bangkok, Thailand

2 A 53 year-old woman with fever, facial swelling and rashes on face, trunk and upper extremities for 3 days.

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4 BT 38 0 C Physical Examination No lymphadenopathy No liver and spleen enlargement Others - unremarkable

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7 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

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13 Histiocytes with crescentic nuclei

14 Histiocytes with crescentic nuclei

15 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

16 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

17 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

18 Confluent parakeratosis with neutrophilic collection PLEVA Interface vacuolization

19 Our Case No parakeratosis No neutrophilic collection

20 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

21 Erythema Multiforme Target lesion Interface vacuolation, lymphocytic infiltration No karyorrhexis

22 Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection

23 Our Case Dense lymphohistiocytes infiltrate with nuclear debris or karyorrhexis

24 Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma

25 Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma

26 Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma

27 Further investigations CBC BUN/Cr LFT LDH 172 Normal

28 Further Investigations ANA Anti-Ro/La Anti-Sm Negative

29 Further investigations Hemo c/s Urine c/s Negative CMV IgM negative EBV IgM negative EBV IgG positive Past infection

30 Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma

31 Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma

32 Our Case Numerous CD68- positive cells Lymphohistiocytes, karyorrhexis, crescentic histiocytes

33 Our Case Numerous CD68- positive cells Lymphohistiocytes, karyorrhexis, crescentic histiocytes Kikuchi s disease

34 Kikuchi-Fujimoto disease (KFD) Histiocytic necrotizing lymphadenitis Rare benign disorder Women < 40 year old Characteristic features Fever Painful lymphadenopathy (cervical)

35 Kikuchi-Fujimoto disease (KFD) Etiology Unknown Autoimmune mechanism Abnormal responses to infectious agents (eg. EBV, parvovirus, HIV, HHV6, HHV8, Yersinia enterocolitica, Bartobella, Brucella, Tocoplasma) Ruiz Beguerie J, et al. Dermatol Online J. 2012;18(9):8 Atwater AR, et al. J Am Acad Dermatol 2008;59:130-6.

36 Kikuchi-Fujimoto disease (KFD) The skin involved in 17-40%; mostly in Asian Rash Erythematous maculopapular eruptions Erythematous plaques Kim JH, et al. Human Pathology. 2010;41: Atwater AR, et al. J Am Acad Dermatol 2008;59:130-6.

37 Erythematous maculopapular eruptions Kim JH, et al. Human Pathology. 2010;41:

38 Facial swelling Erythematous papules and vesicles on trunk Kim JH, et al. Human Pathology. 2010;41:

39 Non-neutrophilic karyorrhexis Kim JH, et al. Human Pathology. 2010;41:

40 CD68 CD3 Kim JH, et al. Human Pathology. 2010;41: CD4 CD8

41 Kikuchi disease-like inflammatory pattern (KLIP) Cutaneous lesion with histology similar to KFD No lymph nodes involvement Thai LH, et al. Medicine (Baltimore) Nov;94(46):e2065.

42 Kikuchi disease-like inflammatory pattern (KLIP) Mainly female; mean age 49 (14-83 year-old) Dermatologic findings Papules **, nodules, papulovesicles Infiltrative lesions Atrophic lesions Annular erythema Lichenoid lesions Ulcers Folliculitis/ pseudofolliculitis Thai LH, et al. Medicine (Baltimore) Nov;94(46):e2065.

43 Kikuchi disease-like inflammatory pattern (KLIP) Associated diseases Connective tissue diseases** SLE, DM, Behcet s disease Malignant hemopathies Drug eruptions Acute viral infection Thai LH, et al. Medicine (Baltimore) Nov;94(46):e2065.

44 Kikuchi disease-like inflammatory pattern (KLIP) Treatments Treat underlying diseases Topical/ systemic corticosteroids Hydroxychloroquine Thai LH, et al. Medicine (Baltimore) Nov;94(46):e2065.

45 Kikuchi disease-like inflammatory pattern (KLIP) Clinical course Most of the patients respond to treatment or resolved spontaneously No recurrent was found Thai LH, et al. Medicine (Baltimore) Nov;94(46):e2065.

46 Topical steroid + supportive Rx All lesions revolved with hyperpigmentation in 2 weeks.

47 Conclusion Skin rash with histologic findings of Kikuchi s disease without lymphadenopathy = Kikuchi disease-like inflammatory pattern (KLIP) Histopathologic clues in diagnosis = Karyorrhetic debris without neutrophil and presence of CD68-positive cells

48 Thank you

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