Histopathology of keratotic papules of the limbs in frontal fibrosing alopecia.

Frontal fibrosing alopecia (FFA) is a scarring type of alopecia that presents clinically as progressive frontotemporal hairline regression and eyebrow loss, mainly in postmenopausal women. An additional common finding is keratotic papules on the face or (less commonly) on the trunk and extremities. The histopathology of the facial keratotic papules associated with FFA is the same as that of lichen planopilaris. There are very few FFA cases with biopsies from extrafacial sites and no cases of folliculocentric keratotic papules with biopsies from extrafacial sites. In the current report, we describe the histopathology of one such keratotic papule from the arm of a 75-year-old woman with FFA and show that the morphology is that of lichen planopilaris and that the papules are identical to those found in Graham-Little-Piccardi syndrome.

Repigmentation of gray hair in lesions of annular elastolytic giant cell granuloma.

Hair pigmentation is a complex phenomenon that involves many hormones, neurotransmitters, cytokines, growth factors, eicosanoids, cyclic nucleotides, nutrients, and a physicochemical milieu. We report a case of repigmentation of gray hairs in lesions of annular elastolytic giant cell granuloma (AEGCG) on the scalp of a 67-year-old man.

The differential diagnosis between primary cutaneous large B-cell lymphoma and cutaneous follicular lymphoma: prognostic and therapeutic implications.

The differential diagnosis between primary cutaneous diffuse large B-cell lymphoma and cutaneous follicular lymphoma is one of the most difficult aspects of dermatopathology, even though morphological criteria are well established and a wide panel of antibodies is available to every laboratory. Such diagnosis is, however, not trivial because it has important prognostic and therapeutic implications. Nevertheless, when the literature is reviewed, there is a feeling that the diagnostic deficits from the past could perhaps be responsible for the differences observed in the therapeutic results with less aggressive treatments, such as rituximab. The current report briefly revises some cases of primary cutaneous diffuse large B-cell lymphoma treated with rituximab, which have been reported in the literature. It also presents an additional case emphasizing the current approach to the differential diagnosis.

Abundance of plasma cells in a case of lipodermatosclerosis.

Plasma cells have been considered as an important morphological clue in the diagnosis of some sclerosing cutaneous diseases such as morphea, so much so that humoral immunology has been suggested to have a role in the pathogenesis of such diseases. Nevertheless, they are hardly ever described as a prominent feature in lipodermatosclerosis in which granulocytes have been claimed as the main pathogenic cell. We report a case of lipodermatosclerosis in a 77-year-old woman, in which plasma cells were abundant in the thickened fibrous septa of the hypodermis. They were highlighted on immunohistochemical study with CD79a, CD138 and EMA, and showed polyclonal immunoexpression of kappa and lambda immunoglobulin light chains.

Morphological evidence of periodical exacerbation of hyperkeratosis lenticularis perstans.

We present a case of hyperkeratosis lenticularis perstans (Flegel's disease) in a 71-year-old-woman. Apart from all the typical morphological features of the disease, we evidenced mounds of parakeratosis that contained neutrophils, alternating with other strata of the horny layers in which no infiltrate was seen. Such a pattern has been described in association with diseases that show a periodic clinical pattern of presentation with periods of exacerbations and remissions. Our case also showed a lichenoid inflammatory chronic infiltrate with vacuolation of the basal layer and mild spongiosis. Both findings strongly support the hypothesis that the inflammatory infiltrate might play a primary role, and that the hyperkeratosis might be a secondary event.

Mitosis in dermatofibroma: a worrisome histopathologic sign that does not necessarily equal recurrence.

BACKGROUND: High mitotic rate is a worrying histopathologic feature that pathologists occasionally face when diagnosing benign fibrous histiocytoma (BFH), that otherwise seem to be benign. The clinical significance of this histologic feature has not been completely studied, because no studies on large series of BFH have been carried out, concerning mitotic rate as the only worrisome morphologic sign, in relation with recurrence. METHODS: In order to study how common a high mitotic rate is in BFH, which do not present any other worrisome morphological signs, we studied 200 cases from our archives and counted the number of mitosis per 10 high power fields (HPFs) in all of them. We selected those cases that had a mitotic number of 4 or more mitosis per 10 HPF. We also performed the immunohistochemical study with MIB-1 antibody (DakoCytomation, Denmark A/S). The clinical histories of the patients were also reviewed, in order to find out if there had been any recurrence. RESULTS: Four BFH presented four or more mitosis in ten HPF. None of them had presented any recurrence or metastasis, and the follow-up time was at least for 71 months. CONCLUSIONS: We conclude that common BFH can occasionally present a high mitotic rate and still show a benign behavior.

Metastatic eccrine porocarcinoma: a 5.6-year follow-up study of a patient treated with a combined therapeutic protocol.

BACKGROUND: Metastatic eccrine porocarcinoma (EP) is an uncommon, malignant, and potentially lethal neoplasm that arises from the eccrine sweat glands. OBJECTIVE: To present the 5.6-year follow-up study of a male patient with metastatic EP, highlighting the widespread cutaneous involvement and the response to the treatment carried out. METHODS: We describe the evolution of the tumor and the combined therapy carried out and review the treatments employed in previously reported cases, comparing them with ours. RESULTS: The patient developed multiple cutaneous and regional lymph node metastases 15 months after surgical excision of the primary tumor. He was treated with prophylactic lymphadenectomy, radiotherapy, and oral isotretinoin, subsequently substituted by tegafur. We have not found evidence of distant metastases after a 5.6-year follow-up. CONCLUSIONS: The optimum treatment for metastatic EP is not standardized, and the results obtained to date were generally poor. In this context, we consider it of interest to highlight the response of our patient to the therapeutic regime employed, which may be advantageous in future cases of this rare tumor.