Necrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma.

A 51-year-old white woman with a past medical history significant for steroid-dependent ulcerative colitis, rheumatoid arthritis, and diabetes mellitus presented to the hospital with fever and painful, erythematous subcutaneous nodules on the medical aspects of both thighs. Histopathologic examination showed features suggestive of an abscess, but her condition failed to improve with intravenous broad-spectrum antibiotics. Molecular studies detected T cell receptor-ß gene rearrangements. The lesions later exhibited signs of necrosis, requiring multiple debridements as well as therapy with hyperbaric oxygen. She was later referred to the MD Anderson Cancer Center for evaluation for possible subcutaneous panniculitis-like T cell lymphoma.

Localized cutaneous argyria: two case reports and clinicopathologic review.

We report 2 cases of patients who presented with blue macules clinically suspicious for blue nevi. One patient had no documented history of trauma or silver exposure, and the other reported exposure to silver over 30 years ago. Microscopic examination revealed a dermal population of brown-black globules predominantly adhering to collagen fibers. In both cases, no melanocytic proliferation was identified by immunohistochemistry. Analysis of the skin biopsies with scanning electron microscopy and energy dispersive x-ray spectroscopy demonstrated the presence of silver and selenium. These findings were diagnostic of localized cutaneous argyria. Our case reports highlight the importance of including localized cutaneous argyria in the differential diagnosis of pigmented lesions.

Mycosis fungoides involving an acrochordon: a case report.

We present the case of a 77-year-old male undergoing treatment for mycosis fungoides (MF) who presented for removal of an acrochordon on his mid back. Histopathologic examination of the acrochordon revealed a dense, band-like lymphocytic inflammatory infiltrate in the dermis with epidermotropism of single lymphocytes and small nests of lymphocytes into the lower epidermis. Immunohistochemical staining characterized the dermal and epidermal lymphocytic population as CD3-positive T lymphocytes with a predominance of CD4-positive over CD8-positive lymphocytes. These findings were consistent with the patient's known MF and molecular identification of a clonal T-cell receptor gene rearrangement further supported the diagnosis. Our unusual case reports MF involving an acrochordon and provides evidence to support the importance of submitting acrochordons for histopathologic examination.

Prognostic factors for melanoma in children and adolescents: a clinicopathologic, single-center study of 137 Patients.

BACKGROUND: Cutaneous melanoma in childhood is rare; therefore, its prognostic factors and biologic behavior and the effectiveness of adjuvant diagnostic techniques in this group remain mostly unknown. METHODS: The authors conducted a retrospective, observational study on the prognostic significance of clinical and pathologic findings from 137 cutaneous and mucosal melanomas in patients aged <18 years that were reviewed by the pathology department of a large cancer center during the period from 1992 to 2006. RESULTS: Univariate analysis indicated that there was a significantly greater risk of metastases for patients who had previous nonmelanocytic malignancies, nodular histologic type, fusiform or spitzoid cytology, high Breslow thickness, vertical growth phase, high dermal mitotic activity, ulceration, and vascular invasion. Adjacent nevus and radial growth phase were associated with a better prognosis. Twelve patients (10.3%) died during follow-up. Decreased overall survival was related significantly to age >10 years, previous nonmelanocytic malignancy, high Breslow thickness, high Clark level, and the presence of metastases at diagnosis. All patients who died were aged ≥ 11 years, and 8 of those patients had metastases at diagnosis. In multivariate analysis, higher Breslow thickness predicted an increased risk of metastases, whereas age >10 years and the presence of metastases at diagnosis were associated with decreased survival. CONCLUSIONS: Similar to adults, the detection of metastases at diagnosis in children with melanoma was 1 of the main factors that influenced overall survival. Melanomas that were detected in children aged <11 years appeared to have a less aggressive behavior than those detected in adults.

Melanoma under 18 years and pregnancy: report of three cases.

Melanoma is an uncommon tumour in childhood. Only isolated cases in pregnant patients younger than 18 years old have been previously described, therefore the biological behaviour of cutaneous melanoma in this group of age remains largely unknown. We report a single-institution experience with three patients who developed cutaneous melanoma before the age of 18 years and became pregnant concomitantly or during the course of the disease. High tumour thickness was attributed to later diagnosis and could be responsible for the aggressive biological behaviour in these patients. This delay was in part due to patients considering changes in nevi to be normal during pregnancy. The effect of pregnancy on the prognosis of patients with melanoma, particularly the role of hormonal and immunological factors on clinical outcome, survival and risk of developing metastases, as well as the differences between adolescent and adult populations are still controversial. Since there are no specific treatment strategies for this group of patients, treatment recommendations should be established according to adult experience.

Nephrogenic systemic fibrosis: report of an additional case with granulomatous inflammation.

Nephrogenic systemic fibrosis (NSF) is a novel disease entity described over the past 10 years. NSF is a progressive systemic fibrosing disorder that occurs arguably exclusively in patients with impaired renal function who have been exposed to gadolinium-containing contrast agents. As no single clinical or histopathologic finding is diagnostic of NSF, a careful review of the cumulative characteristics of each case is essential in making a correct diagnosis. The spectrum of histologic variants of NSF continues to expand, including a report of NSF mimicking erythema nodosum and several case reports of NSF with giant cells and calcification. We report an additional case of NSF with the uncommon pathologic features of granulomatous and lymphocytic inflammation in the fibrous septae similar to erythema nodosum.

Spitzoid melanoma in children: clinicopathological study and application of immunohistochemistry as an adjunct diagnostic tool.

INTRODUCTION: The term spitzoid melanoma (SM) is reserved for a rare group of tumors with striking resemblance to Spitz nevus, often developing in children diagnosed in retrospect after the development of metastases. OBJECTIVES: To determine the biological significance of SM and to analyze the effectiveness of adjuvant diagnostic techniques. MATERIALS AND METHODS: A retrospective, observational study of 38 cases of SM in patients younger than 18 years. Histological type, Clark level and Breslow thickness, radial and vertical growth phase, mitotic count/mm(2), ulceration, regression, vascular and perineural invasion, satellitosis, cytology and associated nevi were reviewed. An immunohistochemical analysis with HMB45 and Ki67 was performed in 10 cases. These features were correlated to patient's stage and outcome. RESULTS: Analysis of histological and immunohistochemical features should allow accurate diagnosis in most cases. Given the low mortality rate, no conclusions about the prognostic significance of histological parameters of the primary tumor could be established. CONCLUSION: We report the largest series of SM from a unique center. Although these patients may have a better prognosis than adults, some patients with SM develop metastasis and die, particularly after age 11 years. Therefore, we recommend using the same treatments as in adults.

Necrotizing vasculitis with a polyarteritis nodosa-like pattern and selective immunoglobulin A deficiency: case report and review of the literature.

Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26-year-old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self-limited episodes of acute diarrhea, atopy, splenomegaly and a 4-year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life-threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium-sized arteries of the skin. Although IgAD results from a failure of B-cell differentiation, we propose that deregulated immune response with production of cross-reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.

Metastasizing adenocarcinoma and multiple neoplastic proliferations arising in a nevus sebaceus.

Nevus sebaceus of Jadassohn is a hamartoma of multiple skin structures. Many neoplasms have been reported to arise in association with nevus sebaceus, most commonly trichoblastoma/basal cell carcinoma and syringocystadenoma papilliferum. We report a case of a 66-year-old woman with an adenocarcinoma as well as multiple neoplastic proliferations arising in a long standing nevus sebaceus on the scalp, with subsequent occipital neck metastatic disease. On histologic evaluation, the epidermis showed changes reminiscent of tumor of the follicular infundibulum as well as basaloid proliferations resembling superficial trichoblastoma. A focus suggestive of syringofibroadenoma was also present. A small dermal collection of basaloid and more mature sebocytes was consistent with a sebaceoma/sebaceous epithelioma. Most of the lesion was composed of an adenocarcinoma with areas showing ductal differentiation with decapitation secretion, well-formed papillae and focal cribriform structures. Other portions demonstrated a high-grade neoplasm with prominent nuclear atypia and a solid pattern of growth resembling high-grade breast carcinoma. Anti-epithelial membrane antigen strongly labeled tumor cells and highlighted ductal structures. Less than 1% of cells expressed progesterone or estrogen receptors. Her2/neu reactivity was focally present, showing 1+ membranous reactivity in 10% of cells. Anti-p63 labeled basaloid cells surrounding the tumor lobules. A breast primary was ruled out by clinical and radiologic examination. This report illustrates an extraordinary case of adnexal neoplasia displaying various lines of differentiation arising in association with nevus sebaceus.

Expression of activated Akt in benign nevi, Spitz nevi and melanomas.

BACKGROUND: Activated Akt expression (p-Akt) is reportedly increased in many melanomas as compared with benign nevi. The purpose of this study was to evaluate and compare p-Akt immunohistological staining in benign nevi, Spitz nevi and primary melanomas. METHODS: Immunostaining for phosphorylated Akt was performed in 41 melanocytic lesions previously classified as benign intradermal nevus (14 lesions), Spitz nevus (9 lesions) or melanoma (18 lesions). Lesions were graded for intensity of p-Akt staining by two independent observers (0, no staining; 1, slightly positive; 2, moderately positive; 3, highly positive). Scores were averaged, and statistical analyses were performed. RESULTS: Benign nevi showed less staining (mean score 1.18) compared with Spitz nevi (mean score 2.11) and melanomas (mean score 2.19). This difference was statistically significant between benign nevi and melanomas (p = 0.0047) and benign nevi and Spitz nevi (p = 0.0271). No statistical difference was detected in staining between Spitz nevi and melanomas (p = 0.8309). CONCLUSIONS: Activated Akt expression is increased in Spitz nevi and melanomas as compared with benign intradermal nevi, but is unlikely to prove useful in differentiating between the former.

Primary cutaneous metaplastic carcinoma: report of a case involving angiosarcoma.

Primary cutaneous metaplastic carcinoma is an uncommon cutaneous malignancy comprising a mixture of epithelial and mesenchymal components. The former is most frequently a squamous cell or basal cell carcinoma, and the latter is most typically a pleomorphic sarcoma or atypical fibroxanthoma. We describe the first primary cutaneous metaplastic carcinoma with angiosarcoma as the mesenchymal component arising on the scalp of a 90-year-old woman.

Protothecosis.

Intravenous amphotericin B remains the most effective drug for eradicating Prototheca infections. It should be used as a first-line agent in cases of disseminated disease and in patients with severe underlying illness or with immunosuppression or immunocompromise. Azole antifungals and surgery should be reserved for patients with more localized disease. Itraconazole appears to be the most effective agent of this drug class, and it should be administered at 200 mg/day for 2 months. Surgical excision should be considered as a first-line therapy in patients who present with olecranon bursal infections.