Multicentric primary extramammary Paget disease: a Toker cell disorder?

Toker cells are epithelial clear cells found in the areolar and nipple areas of the breast, vulvar region, and other apocrine gland-bearing areas of the skin. Toker cells have been implicated in the pathogenesis of clear cell papulosis, cutaneous hamartoma with pagetoid cells, and rare cases of primary extramammary Paget disease (EMPD) but not in secondary EMPD with underlying adenocarcinoma. The pathogenesis of primary EMPD is not well defined. We report a case of multicentric primary EMPD with evidence of Toker cell proliferation and nonaggressive biologic behavior in a 63-year-old white man. A detailed description of the morphologic and biologic features of Toker cells and their possible carcinogenetic links also are discussed. Based on the observation and follow-up of our patient, we hypothesize that multicentric primary EMPD starts with Toker cell hyperplasia and can potentially evolve to carcinoma in the genital region.

The debate between gadolinium versus erythropoietin in a renal transplant patient with nephrogenic systemic fibrosis.

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a debilitating skin condition that causes fibrotic changes in the setting of renal failure. Gadolinium-based contrast agents (GBCA), erythropoietin (EPO), and vascular intervention are the most widely known associated factors in the pathogenesis. A 53-year-old female with chronic renal insufficiency secondary to fibrillary glomerulonephritis (FGN) presented with generalized hardening of skin 1 week after her renal transplant. Due to her numerous medical and surgical health problems, she had received six imaging procedures with GBCA with the last being eight months prior to the onset of her skin symptoms. She had also historically been treated with high doses of EPO. Histopathologic examination was consistent with NSF. In susceptible renal failure patients who develop NSF after GBCA exposure, the onset of symptoms is usually within a 2-3 month time frame, which undermines but not eliminates the proposed role of GBCA in our patient. It can be proposed that despite having various risk factors, while being exposed to high doses of EPO, vascular trauma during renal transplant facilitated the onset of her symptoms.

Skin biopsy for inflammatory and common neoplastic skin diseases: optimum time, best location and preferred techniques. A critical review.

The diagnosis of skin diseases, particularly inflammatory dermatoses, is based primarily on clinical information. Pathologic examination of the biopsied specimen often serves as a complementary or confirmative part of the diagnosis. However, the clinical diagnosis of skin diseases may be challenging, as the clinical information and appearance of skin lesions invariably overlap. Evidence for a correct diagnosis may be lacking without histopathologic examination of skin biopsies. It is well known that the histologic diagnosis of inflammatory and other skin diseases requires clinicopathologic correlation, and there is evolution of skin lesions into different stages as the diseases progress. Other factors important for accurate dermatopathologic diagnosis are optimum time, best location and preferred techniques of skin biopsy. In searching for available information concerning when, where and how to take skin biopsies, it is noted that there are only limited practical guidelines currently available. We present this review article in hopes that our collective dermatopathologic and dermatologic experience can provide a quick reference for accurate diagnosis and proper management of skin diseases.

Localized nephrogenic fibrosing dermopathy: Aberrant dermal repairing?

Nephrogenic fibrosing dermopathy (NFD) has emerged as a clinicopathologic entity since 2000 and was recently renamed nephrogenic systemic fibrosis. The cause and pathogenesis remain uncertain. The classic clinical presentation is diffuse thickening and hardening of the skin that occurs in patients with renal insufficiency, with or without systemic involvement. We report a patient with renal failure who presented to our dermatology clinic with a localized plaque on the left forearm along the vein that was traumatized during the infusion of erythropoietin. Histologic examination revealed a dermal proliferation of CD34(+) fibrocytes with collagen fibers and interstitial mucin accumulation, features characteristic for NFD. We conclude that NFD may present as a localized, scarlike plaque after trauma and exhibit overlapping histopathologic features resembling cicatrix and other dermal reparative/regenerative processes. NFD may, in fact, be a disorder of aberrant extracellular matrix remodeling in patients with renal insufficiency. This is a single case observation with discussion of literature and attempted hypothesis on pathogenesis. No experimental evidence is provided.

Umbilical metastasis from prostate carcinoma (Sister Mary Joseph's nodule): a case report and review of literature.

Sister Mary Joseph's nodule is referred to as metastatic lesion of the umbilicus. Most of the tumors are adenocarcinomas originating from gastroenteric and genital tracts. Only rarely were metastases from other locations reported. We describe here an unusual case of a Sister Mary Joseph's nodule that was metastasized from prostate carcinoma 3 years after radiation therapy. The lesion was the first sign of metastatic disease, and the diagnosis was made on skin biopsy. The patient died of extensive metastases of prostate carcinoma 4 months later. We report this case to extend the list of differential diagnosis for Sister Mary Joseph's nodule in male patients and emphasize the importance of Sister Mary Joseph's nodule as an ominous diagnostic sign.

Acne keloidalis: a novel presentation for tinea capitis.

Acne keloidalis is characterized by firm skin-colored papules on the surface of the neck and scalp. We report a case of tinea capitis in 60-year-old female which mimicked acne keloidalis. The lesions resolved following oral antifungal medication.

Interstitial granulomatous dermatitis associated with the use of tumor necrosis factor alpha inhibitors.

BACKGROUND: Tumor necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of numerous inflammatory and autoimmune disorders. Accordingly, TNF-alpha inhibitors, such as thalidomide, infliximab (Remicade), adalimumab (Humira), and etanercept (Enbrel), have been used with success in the treatment of autoimmune disorders, including psoriasis, rheumatoid arthritis, inflammatory bowel diseases, and lymphoproliferative disorders. Although anti-TNF-alpha therapy is safe and well tolerated, various adverse cutaneous reactions have been reported. OBSERVATIONS: We encountered 5 patients who developed erythematous annular plaques on the trunk and extremities while receiving 4 different medications with inhibitory activity against TNF-alpha. One patient was treated with lenalidomide (Revlimid) for multiple myeloma, 2 received infliximab, and 1 received etanercept for severe rheumatoid arthritis; the last patient was in a clinical trial of adalimumab for psoriatic arthritis. Skin biopsy specimens revealed diffuse interstitial granulomatous infiltrates of lymphocytes, histiocytes, and eosinophils, palisading degenerated collagen. Withdrawal of the medications led to complete resolution of the skin lesions. CONCLUSION: Interstitial granulomatous dermatitis should be considered in the differential diagnosis of skin lesions occurring in the setting of anti-TNF-alpha therapy.

Benign cutaneous pseudoglandular schwannoma: a case report.

Schwannoma is a common peripheral neural neoplasm that could present as a primary skin lesion. In addition to typical schwannoma with classic Antoni A and Antoni B areas, many variant types have been described, such as plexiform, cellular, epithelioid, and ancient schwannomas. Glandular schwannoma is a rare variant characterized by the presence of glands in an otherwise typical schwannoma. There are also a few reported cases in the literature of pseudoglandular schwannoma from central nervous system, eye, submandible, and shoulder, in which the gland-like structures were lined by Schwann cells. We report here a patient with a benign cutaneous schwannoma composed of predominantly gland-like spaces that contained mucinous material and were lined by Schwann cells confirmed by immunohistochemistry and ultrastructural studies. The tumor was well circumscribed and showed minimal cytologic atypia, indicating benignity. We report this unusual case of benign cutaneous pseudoglandular schwannoma to further awareness of the morphologic diversity of schwannoma.