Differential Clinicopathological Features in Spontaneous Regression of Melanomas and Melanocytic Naevi.

The aim of this study was to determine the clinical, histological and/or immunohistochemical features that enable differential diagnosis of regression of melanocytic naevi from regression of melanomas. All melanocytic neoplasms with histologically-confirmed regression diagnosed in our hospital between 2002 and 2009 were reviewed retrospectively. Lamellar and delicate fibrosis were associated with melanocytic naevi (p <0.0001 and p = 0.021, respectively). Compact fibrosis, high vessel density and higher number of granzyme B+ lymphocytes were associated with malignant melanoma (p = 0.011, p = 0.005 and p = 0.013, respectively). Density of inflammatory infiltrate (p = 0.016), vascular proliferation (p = 0.005), epidermal atrophy (p = 0.009), rate of apoptosis (p = 0.046) and granzyme B immunoreactivity (p = 0.013) was more common in severe-dysplastic naevi and melanomas than in the remaining melanocytic naevi. Logistic regression demonstrates that 5 variables (age, lamellar fibrosis, melanophages, vessel density, and granzyme B immunostaining) would serve to classify appropriately 87% of melanomas among melanocytic lesions with complete regression.

Histiocytosis with mixed cell populations.

Langerhans cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) are thought to originate from a common stem cell precursor, with divergent differentiation under different microenvironmental conditions. We describe an exceptional case of multiple cutaneous lesions in a 10-year-old boy, in which the coexistence of both LCH and JXG cell populations is found in every single lesion. The presence of Birbeck granules and CD207 (langerin) immunostaining in the LCH component would argue against the diagnosis of indeterminate cell histiocytosis (ICH). This unique case gives additional support to the hypothesis of a potentially common histogenesis for LCH and JXG.

Parvovirus B19-Associated Microvesicular Eruption.

We report on a 3-year-old girl with a microvesicular generalized rash in whom primary infection by parvovirus B19 was demonstrated by seroconversion. To our knowledge, this is the first instance of an eruption arising from parvovirus B19 with this peculiar clinical pattern.

Spontaneous regression of multiple melanocytic nevi after melanoma: report of 3 cases.

Complete spontaneous regression of multiple melanocytic nevi after melanoma is an extremely rare phenomenon. We report 3 cases of patients with a history of melanoma that showed regression of almost all melanocytic nevi over time. One of the patients had 2 simultaneous primary cutaneous melanomas without metastasis. In the other 2 patients, regression of the melanocytic nevi was seen after the development of metastasis in lymph nodes. These patients had spontaneously developed an efficient immune response against melanocytes, and they would represent paradigmatic examples of the spontaneous immune responses in melanoma patients. Better understanding of the mechanisms involved in the complete regression of melanocytic lesions would lead to a better selection of melanoma patients for immunotherapy.

Complete regression of a melanocytic nevus under intense pulsed light therapy for axillary hair removal in a cosmetic center.

Intense pulsed light (IPL) therapy using noncoherent broad-spectrum light has been reported to be effective for hair removal, and also for treating superficial pigmented lesions like ephelides and solar lentigines. We report complete regression of a pigmented melanocytic nevus, histologically confirmed, after hair removal treatment with IPL. The use of lasers and IPL is a common procedure used by dermatologists and even other professions for the treatment of cosmetically troubling skin conditions. The main advantage of such treatment is a reduction of surgical scars, thus producing a favorable cosmetic outcome, but a major limitation is that histopathologic diagnosis is not usually obtained prior to treatment. Such devices should be carefully used in patients with potentially dangerous melanocytic lesions. We also review the recent literature regarding inadequate treatment of melanocytic lesions with lasers.

Contribution of hyperammonemia and inflammatory factors to cognitive impairment in minimal hepatic encephalopathy.

To assess the contribution of hyperammonemia and inflammation to induction of mild cognitive impairment (or MHE). We analyzed the presence of mild cognitive impairment (CI) by using the PHES battery of psychometric tests and measured the levels of ammonia and of the inflammatory cytokines IL-6 and IL-18 in blood of patients with different types of liver or dermatological diseases resulting in different grades of hyperammonemia and/or inflammation. The study included patients with 1) liver cirrhosis, showing hyperammonemia and inflammation; 2) non-alcoholic fatty liver disease (NAFLD) showing inflammation but not hyperammonemia; 3) non-alcoholic steatohepatitis (NASH) showing inflammation and very mild hyperammonemia; 4) psoriasis, showing inflammation but not hyperammonemia; 5) keloids, showing both inflammation and hyperammonemia and 6) controls without inflammation or hyperammonemia. The data reported show that in patients with liver diseases, cognitive impairment may appear before progression to cirrhosis if hyperammonemia and inflammation are high enough. Five out of 11 patients with NASH, without liver cirrhosis, showed cognitive impairment associated with hyperammonemia and inflammation. Patients with keloids showed cognitive impairment associated with hyperammonemia and inflammation, in the absence of liver disease. Hyperammonemia or inflammation alone did not induce CI but the combination of certain levels of hyperammonemia and inflammation is enough to induce CI, even without liver disease.

Dermatofibrosarcoma protuberans: a clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor, usually low grade, except for the fibrosarcomatous variant (DFSP-FS). OBJECTIVES: We sought to compare the clinicopathological, immunohistochemical, genetic, and therapeutic features between DFSP and DFSP-FS. METHODS: The clinicopathological features were reviewed in 63 DFSP and 12 DFSP-FS. Immunohistochemistry and multiplex reverse transcriptase-polymerase chain reaction were carried out using formalin-fixed, paraffin-embedded tissue, using specific primers for collagen type I alpha 1 (COL1A1) and platelet-derived growth factor beta (PDGFB). RESULTS: DFSP-FS was associated with tumor history longer than 5 years (P = .009), tumor size greater than 4 cm (P = .001), more stages of modified Mohs micrographic surgery (P = .005), expansive subcutaneous infiltration (P = .005), muscular invasion (P = .0001), absence of CD34 staining (P = .018), p53 positivity (P = .006), and increased proliferative activity (P = .004) compared with DFSP. The COL1A1-PDGFB fusion transcript was found in 100% DFSP-FS and 72% DFSP. No association was found between the different COL1A1-PDGFB fusion transcripts and the different histologic subtypes. Wide local excision (2 cm) was performed in 47% of cases and modified Mohs micrographic surgery in 53%. After a mean follow-up of 73 months (range 21-235), 6 patients had local recurrence (5 DFSP, 1 DFSP-FS) and one died of disease (DFSP-FS). The only factor related to local recurrence was the type of surgery (17% wide local excision vs 0% modified Mohs micrographic surgery) (P = .006). LIMITATIONS: Our study is retrospective. Prospective studies are necessary to confirm our results. CONCLUSIONS: DFSP-FS reflects tumor progression in DFSP, with larger size, particular invasive patterns, p53 expression, and increased proliferative activity. However, as in low-grade DFSP, appropriate surgery permits a tumor-free excision. COL1A1-PDGFB is a useful tool for diagnosis of DFSP and particularly for DFSP-FS.

Lymphoepitelioma-like carcinoma of the skin: report of three cases.

Primary cutaneous lymphoepithelioma-like carcinoma (LELC) is an extremely rare cutaneous neoplasm with histopathological features similar to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. Microscopically, the tumor is well circumscribed and is composed of irregular nests of malignant epithelial cells in a background of reactive lymphoid cells including mature plasma cells. Its histogenesis remains unknown although an adnexal or epidermic origin has been proposed, and despite its poorly differentiated histology, the LELC prognosis is relatively good. We describe three new cases of this entity that support an epidermic origin.

Granular cell tumor on the sole of a child: a case report.

Granular cell tumor, also known as Abrikossoff tumor, is a rare infrequent neoplasm of unclear etiology which has been rarely described in children. Involvement of the feet is extremely rare. We report a 7-year-old boy presenting a granular cell tumor on the sole.

Poroid hidradenoma in a child: an unusual presentation.

Poroid neoplasm is a benign sweat gland neoplasm that accounts for 10% of sudoriferous tumors. Poroid hidradenoma is an uncommon variant that usually affects adults, with a peak of incidence in the seventh decade. It is rare in children. We report the first case of poroid hidradenoma presenting in a 13-year-old boy.

Multiple cutaneous angiomyxomas in a child.

Cutaneous myxomas are rare, benign, connective tissue tumors composed of stellate cells set in a loose mucoid stroma containing delicate reticulin fibers running in various directions. They are also called angiomyxomas to reinforce their vascular component. These lesions have been recognized as part of Carney complex. We report an 11-year-old boy affected by disseminated cutaneous angiomyxomas since the age of 2, without any other component of Carney complex.

Piebaldism in a 2-year-old girl.

Piebaldism is a rare autosomal dominant skin disorder characterized by a white forelock and depigmented patches of skin, generally located on the forehead, central chest and abdomen, upper arms, and lower extremities. We report a case of a 2-year-old girl with a typical presentation and review the literature concerning this condition.

Pseudoxantoma elasticum-like dermal elastolysis: a case report.

Elastic fibers are components of dermal connective tissue that can be affected in several acquired disorders. Recently, a new entity known as pseudoxanthoma-like papillary dermal elastolysis has been described. We present a case in a 61-year-old woman.

An atypical presentation of a blastic plasmacytoid dendritic cell tumors.

Blastic plasmacytoid dendritic cell tumor is a rare lymphoma, which has been included as an independent entity in the World Health Organization (WHO) 2008 classification for cutaneous lymphomas. This neoplasm usually affects middle-aged or elderly patients with predominant skin or soft tissues involvement. Although it usually has a good initial response to chemotherapy, relapses are the rule and they occur rapidly. We report a new case of an 81-year-old woman with a blastic plasmacytoid dendritic cell tumors with an unusual presentation.

Primary cutaneous mucoepidermoid carcinoma.

Mucoepidermoid carcinoma represents between 10-30% of primary carcinomas of the submandibulary, parotid and minor salivary glands. Cutaneous involvement is extremely rare and more, as a primary origin of the tumor. A few cases of primary mucoepidermoid carcinoma of the skin have been described. We report an 83-year-old man presenting a primary cutaneous mucoepidermoid carcinoma over his right cheek.

Eccrine angiomatous hamartoma in a child.

Eccrine angiomatous hamartoma (EAH) is a rare, benign malformation characterized by both eccrine and vascular components. It usually presents at birth or during early infancy and childhood as a nodule or a plaque, usually solitary, involving acral skin. Eccrine angiomatous hamartoma is usually asymptomatic, although focal hyperhidrosis, hypertrichosis, and pain can be observed. We report an additional case of this rare entity presenting in a 14-year-old boy.