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Lymphomatoid papulosis.

In this review, we discuss the clinical and histologic features of lymphomatoid papulosis, a cutaneous disorder characterized by recurrent eruptions of self-healing papules and small nodules with histologic findings suggestive of malignant lymphoma. Possible causes of this peculiar disease are discussed in the light of recent investigation. Recognition is important to avoid misdiagnosis and overly aggressive therapy, but it should be remembered that a small number of cases have been reported to progress to malignant lymphoma.

Adult↗

Unusual chronic maculopapular rash associated with human immunodeficiency virus infection.

A 21-year-old black man with sickle cell disease who has received blood transfusions for 11 years because of recurrent stroke has had a decline in numbers of circulating lymphocytes and T4 cells over the past four years. Two and a half years ago persistent generalized lymphadenopathy developed, associated with a positive test for human immunodeficiency virus (HIV) antibody. For the past seven months he has had a pruritic maculopapular rash, primarily on the extensor surfaces of the extremities. Multiple skin biopsies revealed only nonspecific perivascular infiltrates of mononuclear cells. This rash appears to be an unusual manifestation of HIV infection, and may be an indicator of impending AIDS.

Adult↗

Unusual cutaneous adverse reaction to warfarin therapy.

We have described a patient who had an extensive maculopapular pruritic rash after a single dose of warfarin. Although dermatologic reactions have been reported with this drug, the pathogenesis of these reactions remains unknown.

Adult↗

Circulating Sézary cells in the diagnosis of Sézary syndrome (quantitative and morphometric analyses).

Plastic-embedded circulating Sézary cells were examined in semithin and thin sections (assisted by the nuclear contour index-NCI). Eight cases of Sézary syndrome were analyzed as well as 11 controls (3 cases of widespread eczemas and 8 cases of erythroderma), 7 cases of mycosis fungoides, and 3 healthy individuals. Discriminating criteria between Sézary syndrome and benign diseases were sought: in addition to Sézary cells (NCI greater than 6.5) intermediate lymphocytes (5.0 less than NCI less than or equal to 6.5) proved to be helpful. Cases with Sézary syndrome were clearly differentiated when the following 3 ultrastructural criteria were fulfilled: (1) Sézary cells (SC) greater than 9%; (2) intermediate lymphocytes (IL) greater than 20%; (3) the sum of SC and IL greater than 37%. A good correlation between thin and semithin sections was obtained (correlation coefficient for Sézary cells r = 0.82). Usually the values of SC were slightly higher on thin sections. The diagnosis of SS can be made on semithin sections when the ultrastructural criteria are fulfilled. In this way 8 of 12 samples of Sézary syndrome were correctly classified. Therefore, semithin sections (studied by light microscopy) are recommended as a routine method in the diagnosis of cases suspected of Sézary syndrome, whereas thin sections (studied by electron microscopy) appeared to be necessary in problem cases only.

Adult↗

Langerhans cell histiocytosis associated with myelodysplastic syndrome in adults.

BACKGROUND: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders associated with dyplasia of myeloid elements that may have cutaneous manifestations including infections, vasculitis, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, and leukemia cutis. These cutaneous manifestations are attributed to the underlying bone marrow defect. Langerhans cell histiocytosis (LCH) is primarily a pediatric disease, and rarely LCH has been described in association with pediatric MDS. We are aware of only a single case report of LCH associated with MDS in an adult. METHODS: We report two new cases of LCH in elderly patients with underlying MDS. The specimens were examined by routine microscopy as well as immunohistochemical stains for S100 protein and CD1a. RESULTS: Both patients were elderly men with established diagnoses of MDS. One presented with a solitary pruritic papule while the other had a 2-year history of erythematous papules involving the trunk and extremities. Histologic examination revealed intraepidermal and dermal collections of mononuclear cells with reniform nuclei. The cells were strongly positive for S100 and CD1a, confirming their identity as Langerhans cells. CONCLUSION: Cutaneous LCH may be associated with underlying MDS in adults and should be considered in the differential diagnosis of cutaneous eruptions in patients with MDS.

Aged↗