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Multicentric reticulohistiocytosis (MR) is a syndrome characterized by papulonodular skin lesions, a debilitating polyarthritis, and characteristic histologic findings. It is a rare disorder with only 82 cases reported. Twenty-eight percent had an associated neoplasm. Hematologic malignancies (four) and neoplasms arising from the breast (four), cervix (three), stomach (three), ovary (two), colon (two), lung (one), pleura (one), and from an unknown source (two) were all seen associated with MR. Eleven cases (including our own) were available for review. The papulonodular skin involvement was the initial manifestation (alone or concurrent with the polyarthritis) in 90% of our cases. The diagnosis of MR preceded that of the associated neoplasm in 73% of the cases. A case report is presented illustrating the relapse of MR before the clinical relapse of the associated tumor, and the response of both the tumor and MR to combination chemotherapy with cyclophosphamide, methotrexate, 5-fluorouracil, and prednisone when prednisone and aspirin alone failed to relieve the symptoms of MR. Steroids were not beneficial in the patients reviewed. The best management of MR associated with a neoplasm is the best management of the primary neoplasm. Cytotoxic chemotherapy including cyclophosphamide should be considered if there is progression of the MR in order to control the symptoms of MR and prevent a progressive debilitating arthritis.

Thirty women who were seen at our institution between 1984 and 1988 for pruritic urticarial papules and plaques of pregnancy (PUPPP) were retrospectively evaluated and interviewed. We found a significantly increased maternal weight gain and newborn birth weight in patients with PUPPP, compared with age and parity-matched controls. The average weight gain during pregnancy was 18.1 +/- 0.9 (SEM) kg for the patients with PUPPP (excluding twin gestations) and 14.6 +/- 1.0 kg for the controls. The mean newborn birth weight was 3.6 +/- 0.09 kg for the PUPPP group and 3.3 +/- 0.08 kg for the control group. There were three twin pregnancies (10%), compared with the twin gestation rate at our institution of 1.6%. Therefore, based on our findings of an increased maternal weight gain and neonatal birth weight, an increased twin rate, and an abdominal eruption that occurs in primigravidas in their third trimester of pregnancy, we suggest that abdominal distention or a reaction to it may play a role in the development of PUPPP.

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