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• W2337805931 abstract "To the Editor: Generalized eruptive histiocytosis/histiocytoma (GEH) is an extraordinary rare condition affecting mainly adults of either sex, which was first reported by Winkelmann and Muller.1 Patients present with multiple disseminated red-brown papules or nodules on the trunk and extremities. Skin lesions develop rapidly within several weeks and may persist over years. Spontaneous healing can occur,2 and the condition has been reported to respond to PUVA treatment.3 Because of the lack of a comprehensive large series of GEH, it is difficult to determine whether reports of underlying malignancies, such as cutaneous T-cell lymphoma,3 association with autoimmune diseases and immunosuppressive drugs,4 are coincidental. Interestingly, transition or association with other members of the non-Langerhans cell histiocytoses can occur.3,5 Histopathologically, this is reflected by the predominance of so-called “vacuolated” macrophages,5 which are the predominant cell type not only in early GEH but also in benign cephalic histiocytosis and indeterminate cell histiocytosis. Later on, clinicopathologic signs of multiple adult xanthogranulomas, xanthoma disseminatum, or multicentric reticulohistiocytosis may evolve.3,5 Therefore, in all these conditions, clinicopathologic correlation is mandatory to establish the diagnosis. By contrast, multinucleate cell angiohistiocytoma (MCAH)—only described in 1985 by Smith and Wilson Jones6—typically is a localized disease with the gradual development of agminated, grouped, violaceous erythematous papules predominantly located on the upper and lower limbs of middle-aged women. Generalized lesions,7,8 oral mucosal presentation,9 and regression6,8 seem to be the exception. In times when Morbus Kaposi and bacillary angiomatosis tended to complicate HIV infection, MCAH became an important differential diagnosis. MCAH has recently been reviewed in a retrospective study of 142 cases by Frew.10 The author comes to the conclusion that MCAH may be inflammatory and vascular in the initial origin, whereas fibrosis and atrophy play a vital role in the pathogenesis as well. He also found an inverse correlation between positive immunohistochemical staining for CD68 on multinucleate cells and the development of multiple lesions and dermal fibrosis. This may in part explain the divergent results of CD68 staining of multinucleate cells in previous studies.6–8,10 Epidermal hyperplasia and full-thickness dermal fibrosis also point toward a reactive process as previously suggested by one of us (B.Z.).11 The role of estrogen receptor alpha overexpression in MCAH has not been finally investigated,10,12 and similar to GEH, no statistically significant relationship between neoplasia and MCAH has been found.10 Notably, MCAH-like lesions have also been observed at the periphery of basal cell and squamous cell carcinomas.13 Thus, MCAH is now increasingly considered to represent a variant of dermatofibroma/fibrous histiocytoma with an agminated distribution of lesions,11,14 a clinical pattern, which may also occur in melanocytic nevi or pyogenic granulomas. In the following, we report a 64-year-old man with features of both GEH and MCAH. The patient presented with a rapid onset of multiple brownish papules on the trunk and extremities lasting for several years—each single lesion with the typical clinical presentation of a dermatofibroma/fibrous histiocytoma (Fig. 1). There was no evidence of an underlying systemic disease, and the family history was unremarkable. Routine investigation including blood count, chest x-ray, and abdominal ultrasound did not reveal any abnormalities. Biopsies taken from the left abdomen, right forearm, and left upper arm show identical features. Histopathologically, there is moderate acanthosis, papillomatosis, and an inflammatory process affecting the mid and deep dermis. The infiltrate is composed predominantly of not only macrophages with scant cytoplasm but also lymphocytes in a nodular perivascular distribution. In addition, multinucleate cells with angulated cytoplasm are present. There also is a proliferation of thin-walled capillaries with regular endothelial cells. These findings are accompanied by fibrosis extending to the deep dermis. Immunohistologically, there is prominent positivity of the inflammatory infiltrate for CD68, whereas the multinucleate cells are negative for this marker (Fig. 2).FIGURE 1: Multiple disseminated brownish papules located on the trunk and extremities in a 64-year-old-man with focal agminated distribution on the right lateral abdomen and inner side of the left arm.FIGURE 2: Biopsy taken from the left abdomen with acanthosis, papillomatosis, and orthohyperkeratosis in a slightly oblique cut. In the mid and deep dermis, there is a mixed nodular and interstitiell inflammatory infiltrate composed of lymphocytes and macrophages accompanied by multinucleate cells with angulated cytoplasm (inset) and a proliferation of dilated capillary vessels and fibrosis (A). Immunohistologically, the infiltrate is mostly positive for CD68, whereas the multinucleate cells (arrows) are negative (B).Dermatofibroma/fibrous histiocytoma is a fairly common lesion in dermatopathology, and the diagnosis is usually straightforward. However, because plaque-like, giant, multiple, and agminated forms, as in MCAH, can occur, the clinical presentation may be sometimes challenging. Similarly, the histopathology varies considerably depending on architectural, cellular, and/or stromal alterations and has led to the description of several new entities, such as palisading fibrous histiocytoma, epithelioid cell histiocytoma, myofibroblastic dermatofibroma, dermatofibroma with eosinophilic globules,15 or MCAH.6 Cellular atypia, often representing a degenerative phenomenon,16 or induction of sebaceous, follicular, smooth muscle, or neural elements11,14 may sometimes impede rapid diagnosis as well. In addition, cases with prominent hemosiderin deposition may imitate angiomatoid fibrous histiocytoma.17 Because of the lack of specific markers, even immunohistochemistry may be confusing when, for example, desmin or S100 protein is positive and the differential diagnosis of a muscular/neural lesion has to be considered. In cases of recurrence, which is frequently observed in dermatofibroma, type cellular benign fibrous histiocytoma,18 the histopathologic differentiation from fibrosarcoma may be difficult and complete excision and clinical follow-up therefore advisable. Cytology becomes important when dermatofibromas that lack typical architectural and/or stromal features have to be differentiated from non-Langerhans cell histiocytoses.3,5 As cases of eruptive histiocytomas reveal, even then there may be clinicopathologic overlap. This is also demonstrated by the present case that shows the clinical and histological overlap of GEH and MCAH, which seems to be a rare event. Both conditions seem to be reactive in nature with a varying degree of fibroblasts, macrophages, and the presence of multinucleate giant cells. The macrophages found in multiple biopsies in our patient are monomorphous with scant cytoplasm, and the accompanying fibrosis is consistent with long-standing disease, which may explain the negativity for CD68 on multinucleate cells. Vascular hyperplasia is also observed in different other conditions, even scars, and therefore may also be interpreted as a reactive phenomenon. In conclusion, GEH remains a puzzling entity within the spectrum of non-Langerhans cell histiocytoses/fibrous histiocytomas with clinicopathologic overlap to MCAH. Clinical follow-up is advisable in these patients, especially in light of early cases evolving to xanthoma disseminatum or multicentric reticulohistiocytosis and then possible underlying systemic disease. ACKNOWLEDGMENTS The authors thank P. Kind (Offenbach, Germany) for kindly providing case material and clinical details." @default.
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• W2337805931 title "Generalized Eruptive Histiocytosis With Features of Multinucleate Cell Angiohistiocytoma" @default.
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