Acquired Gaucher Cells Located in Dermis near a Malignant Hidradenoma
Jules J. Berman, PhD, MD and Oscar A. Iseri, MD
Ultrastructural Pathology, 12:245-246, 1988
Gaucher cells are histiocytes containing accumulated cerebroside.
These cells are typically large (20-80 microns) and engorged with a
faintly fibrillar material. On electron microscopic examination
the cytoplasm is filled with membrane-bounded sheaths of long
tubular elements. In Gaucher's disease, a metabolic deficiency
of betaglucocerebrosidase results in Gaucher cells appearing in
many organs. Acquired Gaucher cells appear in the RES in conditions
that generate, through rapid cell turnover, such large amounts of
cerebroside that local macrophages become engorged.
For the most part, this phenomenon is limited to conditions of
rapid bone marrow turnover, as blood cells, particularly neutrophils,
generate large levels of ceramide lactoside. Acquired Gaucher cells
are seen in chronic myelogenous leukemia, thalassemia, congenital
dyserythropoietic anemia, and the so-called sea-blue histiocyte
In addition, Gaucher cells have also been encountered
in lobo mycosis owing to breakdown of fungal cell walls (2) and in
granular cell tumors (3).
As an incidental finding in a case of
recurrent malignant hidradenoma, we noticed plump histiocytes
surrounding a dermal Pacinian corpuscle (Fig. 1). This structure was
located several millimeters from a tumor diagnosed in our department
as malignant hidradenoma. The tumor was poorly differentiated with a
high mitotic rate. Many cells had abundant clear cytoplasm.
No other similar
hystiocytes were seen in related dermal structures or in the
tumor itself. The tissue containing the Pacinian corpuscle was
cut from the paraffin block and processed for electron microscopic
FIG. 1. Dermal Pacinian corpuscle surrounded by histiocyte. Higher power on right. H&E.
Around the Pacinian corpuscle were large cells filled
with sheaths of rod like material (Fig. 2). These rodlike structures,
seen at higher power, were smooth, rather straight tubes (Fig. 3).
Delimiting membranes were apparently lost in paraffin processing.
The patient expired several months later with widespread metastases.
No other Gaucher cells were noted at autopsy with careful examination
of the spleen, bone marrow, and liver.
FIG. 2. Histiocyte filled with sheaths of thin tubular rods, EM x 5000.
FIG. 3. Oval packet of rods showing tubelike structure, EM x 33,200.
We presume that hidradenoma-derived cell lipid drained to the space
around the Pacinian corpuscle to be engorged by local phagocytic cells.
With the exception of granular cell tumor, this is, to our knowledge,
the only report of Gaucher cells associated with a nonhematologic
1. Wintrobe MM: Clinical Hematology, 8th Ed., pp.
1340-1344. Philadelphia: Lea and Febiger, 1981.
2. Bhawan J, Bain RW, Purtilo DT, et al.: Lobomycosis. An electron
microscopic, histochemical and immunologic study. J Cutan Pathol 3:5-16,
3. Bhawan J, Malhotca R, Neik DR: Gaucher-like cells in a granular
cell tumor. Hum Pathol 14:730-733, 1983.
Author note [jb]. This work was prepared by the authors as a part of their
work activites for the U.S. Government. Therefore, text and images cannot
be copyrighted and belong to the public domain.
Key words. Gaucher disease, Gaucher's disease, electron microscopy,
surgical pathology, dermatopathology, histopathology, photomicrograph,
histology, public domain images
Last modified: Jan 12, 2013