Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget's disease: response to topical 5-fluorouracil.

An 86-year-old woman presented with a 3-year history of an erythematous axillary lesion, which was histologically confirmed to be extramammary Paget's disease (EMPD) confined to the epidermis and adnexa. Surprisingly, spontaneous clinical regression occurred in the lesion, but Paget's cells persisted within the epidermis and adnexa on histologic examination. One year of intermittent topical chemotherapy with 5-fluorouracil resulted in ulcers that were interpreted as EMPD and completely excised. Histologic examination showed a complete absence of Paget's cells. To our knowledge, only one previous report investigated apparent spontaneous clinical resolution with histologic persistence of EMPD. We emphasize that topical 5-fluorouracil cannot be considered a safe treatment modality for EMPD, but it may be useful in certain cases in which the extent of the lesions, or the general condition of the patient, advise against surgery or radiotherapy.

Widespread cutaneous necrosis and antiphospholipid antibodies: two episodes related to surgical manipulation and urinary tract infection.

Widespread cutaneous necrosis (WCN) associated with antiphospholipid antibodies is rare. Its mechanisms have yet to be elucidated, and there are no well-established guidelines for its management. We describe a woman who had two episodes of WCN related to surgical manipulation for urinary tract obstruction and urinary tract infection. Lupus anticoagulant was always positive. In the second episode anticardiolipin antibodies were elevated, and protein C levels were temporarily decreased. We found only ten previously reported cases of WCN associated with antiphospholipid antibodies, none of which were related to surgical manipulation.

Fingertip tophi without gouty arthritis.

We report 2 patients without a history of acute gouty arthritis showing tophi located only on the fingertips, an unusual location. A review of the literature found a few similar reports; these patients might represent a subgroup of gout characterized by: (1) elderly patients, from the 7th to 9th decades; (2) hyperuricemia; (3) absence of a history of acute gouty arthritis; (4) tophi in the fingertips or fingerpads; (5) chronic renal failure, and (6) all taking drugs or suffering from a disease that affects urate renal excretion.

Multiple epidermoid cysts in a renal transplant recipient taking cyclosporine A.

A forty-two-year-old male recipient of a cadaver renal transplant showed multiple nodulocystic lesions on his back and lateral aspects of his neck four months after beginning immunosuppressive therapy with cyclosporine A and prednisone. Results of histopathologic studies revealed that they were epidermoid infundibular cysts. We note the relationship between this clinical appearance and cyclosporine A, as well as this agent's cutaneous side effects.

An unusual benign neural tumour with stellate-cell morphology.

We report a stellate-cell dermal tumour which was studied by immunohistochemistry and electron microscopy. The stellate morphology of the tumour cells appeared to be due to retraction of their cytoplasm resulting in the emergence of intercellular spaces. Results of the immunohistochemical investigations were consistent with a neural tumour, but we could not assign it to any particular category of the known neural tumours. Therefore we have designated this tumour as an unusual, benign, neural-tumour with stellate-cell morphology.

Epidermotropic metastases from internal carcinomas.

Four cases of epidermotropically metastatic carcinoma are reported. One of them originated from a breast adenocarcinoma; in two other cases, the primary tumor was located in the large intestine; and, in the fourth case, the primary malignancy was a laryngeal squamous cell carcinoma. Clinically, the cutaneous metastases were nodular lesions and histologically, in addition to the involvement of the dermis by malignant cells, in three cases there was intraepidermal involvement by glandular structures. In the fourth case, the cutaneous metastatic tumor cells showed striking "folliculotropism." We review the literature of epidermotropically metastatic carcinoma and discuss its differential diagnosis.

Hereditary epidermolytic palmo-plantar keratoderma (Vörner type)--report of a family and review of the literature.

We describe a patient in whose family 13 of 26 members have a palmo-plantar keratoderma. The histopathological findings in the proband, his brother and father were those of epidermolytic hyperkeratosis. As in other families reported, this disorder was found to be inherited as an autosomal-dominant trait. A review of the literature on the clinical, genetic and associated features of this genodermatosis is presented. We also review the cutaneous conditions that may exhibit the histopathological pattern of epidermolytic hyperkeratosis.

Multiple trichodiscomas: histogenetical observations.

A 23-year-old man with multiple trichodiscomas on his face is described. Four papules were histologically examinated, and all of them showed similar findings, a dermal fibrovascular proliferation with abundant ground substance between collagen bundles and an absence of elastic fibers. We found no evidence to establish a relationship between trichodiscomas and hair disk, and, in our opinion, these lesions should be included into the spectrum of hyperplasia of the adventicial dermis.

Multinodular hemosiderotic dermatofibroma.

We describe a patient who developed a pigmented plaque on the anterior aspect of his right knee clinically mimicking angiokeratoma circumscriptum or malignant melanoma. Histopathological examination demonstrated a multinodular hemosiderotic dermatofibroma in which the cellular tumor islands were separated by areas of hyalinized collagen. Multinucleated giant tumor cells were found in the lumen of a medium-size vein underlying the tumor. The differential diagnosis of angiomatoid malignant fibrous histiocytoma, a tumor with clinical and morphologic similarities, is discussed.