PMC8068553_01
(a) Tumor localized to the cheek of the patient. (b) Painless, nonitching maculopapular rash on the patient's truncus. (c) Normalization of the skin after BRAF-inhibitor treatment.
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A 69-year-old man was admitted to our department after skin biopsies from the ear and right cheek (Figure 1(a)) had revealed a histopathological picture morphologically and immunohistochemically consistent with LCH (Figure 2). Due to a painless, nonitching maculo-papular rash on face, scalp, and truncus (Figure 1(b)), he was treated with phototherapy (i.e., UVB) and topical steroids by a local dermatologist for approximately one year. This considerably improved his skin alterations (Figure 1(c)).
Skin sample at the time of diagnosis showing histopathological and immunohistochemical features consistent with Langerhans cell histiocytosis. (a) Haematoxylin and eosin staining (100x), and immunohistochemical staining for (b) S100 (100x) and (c) CD1a (100x).
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A 69-year-old man was admitted to our department after skin biopsies from the ear and right cheek (Figure 1(a)) had revealed a histopathological picture morphologically and immunohistochemically consistent with LCH (Figure 2). Immunohistochemically, a biopsy from the skin and ear showed a tumor cell population with a strong expression of S100 and CD1a (CD207) and moderate expression of CD68 (Figures 2(b) and 2(c)).
Giemsa staining of the bone marrow sample showing histopathological features consistent with primary myelofibrosis (200x).
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A trephine biopsy showed no evidence of LCH in the bone marrow, but the histomorphology was consistent with a chronic myeloproliferative neoplasia of PMF type (Figure 3). The CT scan revealed an edematous appearance of both kidneys surrounded by adipose tissue similar to the appearance described in patients with Erdheim-Chester disease (Figure 3(c)).
Elevated 18F-FDG uptake localized corresponding to (a) the right cheek, cutaneous portion of the back head (occipital) and in one lymph node at the neck and (b) several foci in the pelvic bones, and a soft tissue process ahead of right os pubis showing increased 18F-FDG activities. (c) The CT scan showing pathological edematous appearance of the kidney surrounded by adipose tissue.
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It revealed increased focal 18F-FDG uptake localized to the right cheek lesion, a cutaneous lesion in the occipital area, a cervical lymph node, the soft tissue anterior to the pubic bone (Figure 4(a)), and several foci in pelvic bones (Figure 4(b)).