Rapid Growth of Dermatofibrosarcoma Protuberans Associated with Bilateral Adrenalectomy for Cushing's Syndrome

We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl. It is known that glucocorticoids decrease the transcriptions of the COL1A1 gene and the PDGFB gene, which is under the direct control of the COL1A1 gene in most DFSP. Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP. To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome

Phaeohyphomycosis Caused by Phaeoacremonium rubrigenum in an Immunosuppressive Patient: A Case Report and Review of the Literature

Phaeohyphomycosis (PHM) is a rare, deep fungal infection of the skin and subcutaneous tissues caused by dematiaceous fungi. In this report, we describe a case of PHM caused by Phaeoacremonium rubrigenum, which is generally known to infect woody plants. We detected the gray-blackish villi by biopsy culture material, and slide culture revealed the conidia arising from slightly tapering phialides. Furthermore, we differentiated these fungi as P. rubrigenum by Basic Local Alignment Search Tool (BLAST) algorithm. We performed surgical debridement of disseminated nodules and administered oral itraconazole for a duration of 4 weeks. One year after stopping itraconazole, there was no sign of relapsing subcutaneous nodules. To our knowledge, this is the third case report of PHM developing from skin infection by P. rubrigenum in human

Successful Treatment of MMP-9-Expressing Angiosarcoma with Low-Dose Docetaxel and Bisphosphonate

We describe a 78-year-old Japanese patient with angiosarcoma on the scalp. Interestingly, immunohistochemical staining revealed this tumor as positive for matrix metalloproteinase 9 (MMP-9). After conventional therapy for angiosarcoma with surgical treatment and radiation therapy, we intravenously administered docetaxel at 40 mg/m2 body surface area together with oral administration of 17.5 mg sodium risedronate hydrate. One and a half years after the standard treatment, there was no evidence of local recurrence or metastasis

Keratoacanthoma Centrifugum Marginatum with Spontaneous Regression and Its Possible Differential Diagnosis

Keratoacanthoma centrifugum marignatum (KCM) is a rare variant of keratoacanthoma, which is characterized by the dense infiltration of inflammatory cells throughout the dermis, especially around the keratinocytic islands. Therefore, it is sometimes difficult to differentiate between KCM and cutaneous T-cell lymphomas. In this report, we describe a case of KCM with spontaneous regression that showed dense infiltration of CD3+CD8+ T cells. Our present case suggested the importance of investigating tumor-infiltrating lymphocytes to avoid the misdiagnosis of KCM as cutaneous T-cell lymphoma

Profiles of Cytotoxic T Lymphocytes in Cutaneous Lymphoid Hyperplasia of the Face

Cutaneous lymphoid hyperplasia (CLH) is difficult to differentiate from primary malignant cutaneous lymphomas that may present as solitary nodules, and sometimes it requires much time to achieve a final diagnosis. A recent report [Park et al.: Acta Haematol 2011;126:79-86] suggested that the expression of granulysin correlates with the prognosis of cancer patients, even in hematological disorders. In this report, we immunohistochemically examine the expression of cytotoxic molecules (e.g. granulysin, TIA-1 and perforin) in tumor-infiltrating lymphocytes of 10 patients with CLH and 3 patients with cutaneous diffuse large B cell lymphoma, not otherwise specified (CDLBCL-NOS) of the face. In the patients with CLH, the number of granulysin-bearing cells was higher than in the patients with CDLBCL-NOS. In contrast, there was no difference in the number of TIA-1+ or perforin+ cells. The present study attempts to explain the different biological behaviors of these two hematological disorders and suggests granulysin as a possible diagnostic tool for CLH and CDLBCL-NOS of the face

Granulysin-Bearing Cells in the Skin Lesions of Acute Graft-versus-Host Disease: Possible Mechanisms for Hypohidrosis in Graft-versus-Host Disease

Graft-versus-host disease (GVHD) is an important complication of bone marrow transplantation and is known to induce sweat gland abnormalities. We employed immunohistochemical staining for granulysin, dermcidin as well as IL-17 and Foxp3 in the lesional skin of 7 patients with acute GVHD. Granulysin-bearing cells were distributed in the epidermis, basal membrane zone of the dermis, and superficial perivascular lesion of the dermis. Interestingly, granulysin-bearing cells were also detected around the secretory portion of the eccrine glands, which might be related to the downregulation of the expression of dermcidin on the secretory portion of the eccrine glands. Differing from a mouse model, in human acute GVHD, IL-17-producing cells and Foxp3+ cells were not prominent. Our present observation sheds light on the contribution of granulysin-bearing cells to the decrease of the sweat in patients with acute GVHD. Though we did not assess the function of these infiltrating lymphocytes directly, further analysis of the mechanism of this phenomenon would offer fundamental insight into the establishment of acute GVHD

Immunohistochemical Similarities between Lichen Sclerosus et Atrophicus and Morphea: A Case Study

Both morphea and lichen sclerosus et atrophicus (LSA) are connective tissue diseases that mainly affect the skin. A recent report suggested that a substantial portion of morphea coexists with LSA. In this report, we describe a case of LSA on the abdomen accompanied by morphea; we employed immunohistochemical staining for periostin as well as MMP-7 and MMP-28, both of which are reported to facilitate fibrosis in the development of various organs, including skin. To our knowledge, this is first English language paper that demonstrates the immunohistochemical staining of periostin, MMP-7 and MMP-28 for morphea and LSA. Our present case might suggest possible mechanisms for the coexistence of two different sclerotic skin disorders

Bullous Pemphigoid Accompanied by Aplastic Anemia: The Induction of IL-17-Producing Cells in the Affected Areas of the Skin

Th17 cells, characterized by IL-17 production, play a critical role in the pathogenesis of autoimmune diseases, including autoimmune bullous disorders and aplastic anemia (AA). In this report, we describe a 58-year-old Japanese man with bullous pemphigoid (BP) accompanied by AA. Interestingly, immunohistochemical staining revealed the existence of IL-17-producing cells in the skin biopsy specimens from BP. Our findings might suggest relationships between IL-17 and the pathogenesis of these autoimmune diseases, and, to our knowledge, this is the first English report of BP accompanied by AA

Merkel Cell Carcinoma with Spontaneous Regression: A Case Report and Immunohistochemical Study

Merkel cell carcinoma (MCC) is an aggressive neuroendocrine carcinoma that only rarely regresses spontaneously. Since little is known about the immunological mechanisms involved in the spontaneous regression of MCC, we describe a case of MCC with spontaneous regression and employed immunohistochemical staining for cytotoxic and immunosuppressive molecules to investigate possible mechanisms involved in the spontaneous regression of MCC. Interestingly, compared to conventional MCC, tumor-infiltrating lymphocytes in MCC with spontaneous regression contained higher numbers of CD8+ cells and granulysin-bearing cells and lower numbers of CD206+ cells. Our present study suggests one of the possible reasons for the spontaneous regression of MCC