ABSTRACT
Three cases of medallion-like dermal dendrocyte hamartoma are reported.The patients included two men and one woman,and were aged from 2 to 38 years.All the patients presented with medallion-shaped,welldefined,slightly atrophic and asymptomatic congenital patches with prominently dilated superficial veins that had grown proportionately with the body length.Histological examination revealed similar changes in all of these patients,including epidermal atrophy and dermal proliferation of spindle cells,which stained positive for CD34,factor ⅩⅢa and vimentin,but negative for S100,CD68,CDla,CD31 and smooth muscle actin (SMA).None of the patients received special therapy,and the lesions enlarged slightly with body growth within 3-4 years of follow-up after the diagnosis.
ABSTRACT
Reticulohistiocytoma, a rare form of non-Langerhans cell histiocytoses, usually presents it-self as a single cutaneous nodule mainly on the head and neck of young men without associated arthritis. We describe an 18-year-old male patient with a red dome-shaped nodule of 2 months' duration on the scalp. Histologic examination of the excised mass revealed numerous large, mononucleated or multinucleated histiocytes with abundant eosinophilic, finely granular cytoplasm having a ground-glass appearance. Immunohistochemical staining was positive for vimentin, lysozyme, and factor XIIIa, supporting a dermal dendrocyte lineage. Ultrastructurally, large mononuclear or multinucleated cells exhibiting numerous peripheral villi contained pleomorphic cytoplasmic inclusions, fatty droplets and dense bodies. Our clinicopathologic findings support the concept that reticulohistiocytoma is a variant of adult xanthogranuloma.
Subject(s)
Adolescent , Adult , Humans , Male , Arthritis , Cytoplasm , Eosinophils , Factor XIIIa , Head , Histiocytes , Histiocytosis , Histiocytosis, Non-Langerhans-Cell , Inclusion Bodies , Muramidase , Neck , Scalp , VimentinABSTRACT
BACKGROUND: The relationship and differentiation among various dendritic cells of the dermis are unclear. Recently it has hecome possible to identify different subpopulat,ions of dermal dendritic cells using anti-CD34 and anti-factor XIIIa antibodies. OBJECTIVE: To elucidate which cell types the fibrous dermal turnors consist of we compared the staining patterns of these antibodies as well as of anti-Mac 387 antibody which are labeled as inflammatory cells of the monocyte-macrophage lineage. METHODS: Tumors studied included dermatofibrosarcoma protuberans(DFSP, n=2), dermato-fibroma(n=22), neurofibroma, n=27), acrochordon(n=15), keloid, hypertrophic scar(n=10), juvenile xanthogranuloma(n=1, and malignant fibrous histiocytoma (MFH, n=1). We performed immunoperoxidase staining(AUSC technique) with polyclonal anti FXIIIa antibody, monoclonal anti-CD34 antibody, and monoclonal anti-Mac 387 antibody on the formalin-fixed-paraffin-embedded tissue specimens of these fibrous tumors. The intensity of staining was graded as negative, weakly staining, or strongly stainiring. RESULTS: FXIIIa reactivity was strongly present in dendritic and spindle-shaped cells of all dermatofibromas and some nurofibromas(11 of 27 specimens), but absent from the other fibrous tumors. Among these tumors, one of the two DFSPs was uniquely expressed CD34. Dendritic and spindle-shaped cells within tiese tumors were MAC 387 negative. In inflammatory conditions, variable numbers of MAC 38 positive cells were observed, corresponding to histiocytes and mac-rophages, but the labeling of ipithelioid cells and multinucleated foreign body giant cells were variable. CONCLUSION: The findings of significant numbers of FXIIIa positive cells in dermal fibrous tumors studied suggest that thet may be diagnostic utility associated with the use of this antit)ody. In addition, CD 34 expression by the tumor cells can be an extremely useful marker in establishing a definitive diagnosis of IFSP.