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2015 ; 94
(17
): e787
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The pathologic findings of skin, lymph node, liver, and bone marrow in patients
with adult-onset still disease: a comprehensive analysis of 40 cases
#MMPMID25929927
Kim HA
; Kwon JE
; Yim H
; Suh CH
; Jung JY
; Han JH
Medicine (Baltimore)
2015[May]; 94
(17
): e787
PMID25929927
show ga
Adult-onset Still disease (AOSD) is characterized by fever, skin rash, and
lymphadenopathy with leukocytosis and anemia as common laboratory findings. We
investigated the characteristic pathologic findings of skin, lymph node, liver,
and bone marrow to assist in proper diagnosis of AOSD.Forty AOSD patients were
included in the study. The skin (26 patients), lymph node (8 patients), liver (8
patients), or bone marrow biopsies (22 patients) between 1998 and 2013 were
retrospectively analyzed. AOSD patients were diagnosed according to the Yamaguchi
criteria after excluding common infections, hematological and autoimmune
diseases. Immunohistochemistry, immunofluorescence, and Epstein-Barr
virus-encoded RNA (EBER) in situ hybridization were performed.Most skin biopsies
revealed mild lymphocytic or histiocytic infiltration in the upper dermis.
Nuclear debris was frequently found in the dermis in 14 cases (53.8%). More than
half of the cases (n?=?14, 53.8%) showed interstitial mucin deposition. Some
cases showed interface dermatitis with keratinocyte necrosis or basal
vacuolization (n?=?10; 38.5%). The lymph node biopsies showed a paracortical or
diffuse hyperplasia pattern with immunoblastic and vascular proliferation. The
liver biopsies showed sparse portal and sinusoidal inflammatory cell
infiltration. All cases showed various degrees of Kupffer cell hyperplasia. The
cellularity of bone marrow varied from 20% to 80%. Myeloid cell hyperplasia was
found in 14 out of the 22 cases (63.6%). On immunohistochemistry, the number of
CD8-positive lymphocytes was greater than that of CD4-positive lymphocytes in the
skin, liver, and bone marrow, but the number of CD4-positive lymphocytes was
greater than that of CD8-positive lymphocytes in the lymph nodes.The relatively
specific findings with respect to the cutaneous manifestation of AOSD were mild
inflammatory cell infiltration in the upper dermis, basal vacuolization,
keratinocyte necrosis, presence of karyorrhexis, and mucin in the dermis. In all
cases, pathologic findings in the lymph nodes included paracortical hyperplasia
with vascular and immunoblastic proliferation. Skin and lymph node pathology in
addition to clinical findings can aid in the diagnosis of AOSD.