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Plasmacytoid monocytes in Jessner's lymphocytic infiltration of the skin. A valuable clue for the diagnosis.

Plasmacytoid monocytes (PCMs) are mononuclear cells of monocyte/macrophage origin with peculiar plasmacytoid features on electron microscopy. This cell type is largely unknown in dermatopathology, probably due to standard fixation procedures. The authors provide a detailed histopathologic, immunohistochemical, and electron-microscopic study of PCMs in Jessner's lymphocytic infiltration of the skin (LIS). PCMs occurred in 58% of all biopsy specimens from 75 patients with LIS in single or multiple clusters within the dermal infiltrates. Recognition of this cell type was important for differential diagnostic reasons because PCMs did not occur in polymorphic light eruption. In discoid lupus erythematosus, PCMs were present in only 7.3% of 110 biopsy specimens. PCMs are probably involved in the activation, proliferation, and differentiation of T-lymphocytes. Their origin and function are discussed and a special role in the homing of lymphocytes into the skin is suggested.

Diagnosis, Differential↗

Atypical lymphoid infiltrates arising in cutaneous lesions of connective tissue disease.

Atypical lymphoid infiltrates occurring in the setting of connective-tissue disease (CTD) comprise malignant neoplasms of B-cell or T-cell phenotypes and various reactive lymphoid hyperplasias, such as myoepithelial sialadenitis, lymphocytic thyroiditis, and lymphocytic interstitial pneumonitis. We describe 17 patients with atypical lymphoid infiltrates arising in cutaneous lesions of CTD, the spectrum of which included lupus erythematosus, dermatomyositis, relapsing polychondritis, and lichen sclerosus et atrophicus. There were two principal categories, pseudolymphoma and malignant lymphoma, the former representing 15 of the 17 cases. The clinical and histologic features and possible pathogenetic mechanisms are discussed.

Adult↗

Progressive hemifacial atrophy in a patient with lupus erythematosus.

This is a single case report describing a young woman with progressive facial atrophy that began at age 15. After she underwent commissuroplasty and collagen injection at age 29, her symptoms continued to progress, and she was ultimately shown to have serologic evidence of systemic lupus. She had a "remission of symptoms" after commencement of medical treatment (chloroquine and topical steroids) for her lupus. The association of progressive hemifacial atrophy and lupus is not new. This case and other cases associated with different autoimmune diseases suggest an autoimmune collagen-vascular origin for some cases of progressive facial atrophy. Furthermore, there is a need to modify the surgical approach in light of concurrent disease.

Adult↗

The human C3b receptor: function and role in human diseases.

The human C3b receptor (CR1) is a polymorphic glycoprotein which functions regulating the complement system by inhibiting the activation of C3 and C5, through its effect on their convertases, and serving as cofactor for factor I in mediating the degradation of C3b to its inactive fragment C3bi and further to C3d-g. The latter are then ligands for their respective receptors on leukocytes, CR3 and CR2. Additionally, CR1 on erythrocytes endows these cells with the capacity to deliver immune complexes (IC) to the reticuloendothelial system, resulting in their clearance from the circulation. On phagocytes, this receptor participates in the process of endocytosis of foreign particles. There is a wide inherited variation of CR1 expression on erythrocytes (CR1/E) of different individuals. Patients with diseases which feature elevated levels of IC, such as systemic lupus erythematosus, leprosy, and AIDS, have a marked decrease of CR1/E, which may result in an altered clearance. This reduction appears to be related to disease activity, and the most probable site for CR1/E loss is during the transfer of IC to macrophages. Healthy neutrophils increase tenfold their expression of CR1 in response to the effect of chemoattractant peptides. Neutrophils from patients with AIDS display an altered response to stimulation. This defect may be of relevance in the process of endocytosis.

Acquired Immunodeficiency Syndrome↗