CPC Chutika Srisuttiyakorn, M.D. Kobkul Aunhachoke, M.D. Phramongkutklao Hospital Bangkok, Thailand
A 53 year-old woman with fever, facial swelling and rashes on face, trunk and upper extremities for 3 days.
BT 38 0 C Physical Examination No lymphadenopathy No liver and spleen enlargement Others - unremarkable
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Histiocytes with crescentic nuclei
Histiocytes with crescentic nuclei
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Confluent parakeratosis with neutrophilic collection PLEVA Interface vacuolization
Our Case No parakeratosis No neutrophilic collection
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Erythema Multiforme Target lesion Interface vacuolation, lymphocytic infiltration No karyorrhexis
Subepidermal vesicle with necrosis Inflammatory diseases Erythema multiforme Lupus erythematosus Pityriasis lichenoides et varioliformis acuta (PLEVA) Small vessel vasculitis Infection Herpes infection
Our Case Dense lymphohistiocytes infiltrate with nuclear debris or karyorrhexis
Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma
Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma
Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma
Further investigations CBC BUN/Cr LFT LDH 172 Normal
Further Investigations ANA Anti-Ro/La Anti-Sm Negative
Further investigations Hemo c/s Urine c/s Negative CMV IgM negative EBV IgM negative EBV IgG positive Past infection
Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma
Lymphohistiocytes with karyorrhexis Inflammatory diseases Lupus erythematosus Lymphocytic vasculitis Kikuchi s disease Hematologic malignancies, eg. NK-T cell lymphoma
Our Case Numerous CD68- positive cells Lymphohistiocytes, karyorrhexis, crescentic histiocytes
Our Case Numerous CD68- positive cells Lymphohistiocytes, karyorrhexis, crescentic histiocytes Kikuchi s disease
Kikuchi-Fujimoto disease (KFD) Histiocytic necrotizing lymphadenitis Rare benign disorder Women < 40 year old Characteristic features Fever Painful lymphadenopathy (cervical)
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.
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;41:1245-1254 Atwater AR, et al. J Am Acad Dermatol 2008;59:130-6.
Erythematous maculopapular eruptions Kim JH, et al. Human Pathology. 2010;41:1245-1254
Facial swelling Erythematous papules and vesicles on trunk Kim JH, et al. Human Pathology. 2010;41:1245-1254
Non-neutrophilic karyorrhexis Kim JH, et al. Human Pathology. 2010;41:1245-1254
CD68 CD3 Kim JH, et al. Human Pathology. 2010;41:1245-1254 CD4 CD8
Kikuchi disease-like inflammatory pattern (KLIP) Cutaneous lesion with histology similar to KFD No lymph nodes involvement Thai LH, et al. Medicine (Baltimore). 2015 Nov;94(46):e2065.
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). 2015 Nov;94(46):e2065.
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). 2015 Nov;94(46):e2065.
Kikuchi disease-like inflammatory pattern (KLIP) Treatments Treat underlying diseases Topical/ systemic corticosteroids Hydroxychloroquine Thai LH, et al. Medicine (Baltimore). 2015 Nov;94(46):e2065.
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). 2015 Nov;94(46):e2065.
Topical steroid + supportive Rx All lesions revolved with hyperpigmentation in 2 weeks.
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
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