Follicular lupus erythematosus: a new cutaneous manifestation of systemic lupus erythematosus.

We report the clinical, histopathological and immunological features of follicular erythema and petechiae in a 30-year-old Japanese woman with systemic lupus erythematosus (SLE). Histology showed this eruption to constitute a cutaneous manifestation of SLE. To our knowledge, this is the first reported case of follicular erythema and petechiae in association with SLE. Accordingly, we propose that this rare eruption be termed 'follicular lupus erythematosus'.

PUVA-induced lichen planus pemphigoides.

A 72-year-old woman had suffered from parapsoriasis en plaque (large plaque type) controlled by topically applied psoralen ultraviolet A (PUVA) therapy. The parapsoriasis lesions gradually disappeared, but numerous tiny red papules with pruritus appeared over the forearms and lower legs 120 days after starting PUVA therapy. These papules developed to form violaceous plaques. Histological findings demonstrated the characteristics of lichen planus. Two months later, tense bullae developed on the plaques and on uninvolved skin of the limbs. These were subepidermal, with linear deposits of IgG and C3 along the basement membrane zone (BMZ) in immunofluorescence of peribullous skin, and immunodeposits of type IV collagen along the floor of the bullae. We therefore, diagnosed lichen planus pemphigoides (LPP). Using systemic and topical steroid therapy, the lesions rapidly resolved and there has been no recurrence. This case suggests that the combination of basal cell injuries caused by chronic inflammation and PUVA therapy could expose BMZ components to autoreactive lymphocytes and induce LPP.

Glomeruloid hemangioma in POEMS syndrome shows two different immunophenotypic endothelial cells.

The case of a Japanese woman with glomeruloid hemangioma, an initial marker for POEMS syndrome, is reported. Her cutaneous lesions were multiple and consisted of glomeruloid hemangiomas, cherry-type capillary hemangiomas, and a mixture of both. The specimens of glomeruloid hemangiomas were studied by paraffin section immunohistochemistry with a large panel of antibodies and electron microscopy, respectively. The lesions, whose size ranged from minute foci to large nodules, were composed of anastomosing vascular channels resembling renal glomeruli and had irregular lumina, often featuring capillaries and sinusoid-like spaces. The vascular channels were lined by a single layer of endothelial cells, which showed two types of cells. The capillary-type endothelium possessed large vesicular nuclei with open chromatin and large amount of cytoplasm. The sinusoidal endothelium possessed small basal nuclei with dense chromatin as well as scant amount of cytoplasm. The former cells had a characteristic CD31+/CD34+/UEA I+/CD68- phenotype. Some of these cells ultrastructurally showed intracytoplasmic lumen formation. The latter cells had a characteristic CD31+/CD34-/UEA I-/CD68+ phenotype. The present study shows that glomeruloid hemangioma has unique morphologic and immunologic features that differ from the traditional hemangiomas as well as littoral cell angioma of the spleen.

Apoptosis and p53 protein expression increase in the process of burn wound healing in guinea-pig skin.

Apoptosis and the expression of p53 protein, an apoptosis-related protein, in the process of healing of a full-thickness burn wound in guinea-pig skin were studied with the terminal deoxynucleotide transferase nick-end labelling method, electron microscopy, and immunohistochemistry, respectively. Apoptosis was detected in the peripheral zone of heat-injured skin from 12 h until day 10 after the burn, with the peak occurring on day 2. The peripheral zone of heat-injured skin showed p53 protein from 12 h through day 2, with the peak occurring on day 2. Apoptosis was also detected in tissues regenerated for covering skin defects. The peak of apoptosis in the regenerated epidermis occurred at days 7-10, when the epidermis was most acanthotic. p53 protein reactivity was also detected in the acanthotic regenerated epidermis, with a peak on day 7. The peak of apoptosis in the granulation and scar tissue took place from day 10 to 14, when the granulation tissue started diminishing, but p53 protein reactivity was not detected there. These findings suggest that apoptosis plays an important part in the elimination of dying and/or dead cells resulting from heat stress, the terminal differentiation of the regenerated epidermis, and the decrease in cellularity during remodelling. The apoptotic process during remodelling may be mediated by some p53-independent pathway.

Virus-associated haemophagocytic syndrome caused by rubella in an adult.

Haemophagocytic syndrome is a systemic clinicopathological entity characterized by systemic proliferation of benign haemophagocytic histiocytes, fever, cytopenia, abnormal liver function and, frequently, coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections, a wide spectrum of malignant neoplasms, autoimmune diseases and drugs. We report a case of rubella virus-associated haemophagocytic syndrome in a previously healthy 29-year-old woman. Blood tests showed cytopenia, especially severe thrombocytopenia, liver dysfunction, hyperferritinaemia and hypercytokinaemia. Bone marrow examination showed many mature histiocytes with active haemophagocytosis. A skin biopsy from the rash revealed perivascular lymphohistiocytic infiltrates with haemophagocytic histiocytes in the upper and mid-dermis. The patient was treated with antibiotics and immunoglobulin, and by supportive measures including platelet transfusion, and recovered completely.

Recurrent malignant proliferating trichilemmal tumour: local management with ethanol injection.

We report a 59-year-old woman who exhibited a recurrent malignant proliferating trichilemmal tumour on the scalp for 1 5 years. The tumour was recalcitrant to conventional treatments such as chemotherapy, radiation or hyperthermia and we performed intratumoral ethanol injection as an alternative means of reducing tumour mass and obtaining haemostasis. Biopsy specimens obtained after the ethanol injection revealed oedema, haemorrhage in the dermis and degeneration of the tumour cells, showing vacuolization with pyknotic nuclei. For cases of recurrent skin tumours and for patients in poor clinical condition, intratumoral ethanol injection is likely to be a therapeutic alternative to surgery or other conventional treatments.

Verruciform xanthoma in association with a vulval fibroepithelial polyp.

We report the clinical, light microscopic, immunohistochemical and ultrastructural features of a verruciform xanthoma that developed in association with a vulval fibroepithelial polyp. To our knowledge, this is the first time that this association has been reported. Immunohistochemical findings confirmed that the xanthoma cells were of a monocyte/macrophage lineage. In addition to typical histological characteristics, prominent vascular ectasias were detected in the deep dermis at the periphery of this lesion. The ectasias may play a part in pathogenesis.

Merkel cell carcinoma with partial spontaneous regression: an immunohistochemical, ultrastructural, and TUNEL labeling study.

We report a case of Merkel cell carcinoma that partially regressed after biopsy. A 76-year-old woman presented with an 1 month history of a rapidly enlarging nodule on her left cheek. After biopsy, the nodule reduced to almost half the size and was excised 1 month later. The excised specimen showed a dense cluster of lymphocytes and fibrosis around the tumor nests. In addition, lymphocytes showed apposition with tumor cells. An immunohistologically dense, even infiltration of CD4+ and CD8+ cells was found around the tumor nests, and more CD8+ cells than CD4+ cells were seen in the tumor nests. By electron microscopy (EM), apoptosis of tumor cells and lymphocytes was observed. Many apoptotic cells were also detected by in situ nick end-labeling (TUNEL) of DNA-breaks, especially in the marginal area of tumor nests surrounded by dense lymphocytic infiltrates. It seems likely that T-cell immunity, which induces apoptosis of tumor cells, may have been involved in tumor regression.