Demographics and additional haematologic cancers of patients with histiocytic/dendritic cell neoplasms.

Aims: The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics and additional haematologic cancers of patients diagnosed with histiocytic disorders in The Netherlands.

Methods And Results: We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga).

Tumor thickness and adnexal extension of superficial basal cell carcinoma (sBCC) as determinants of treatment failure for methylaminolevulinate (MAL)-photodynamic therapy (PDT), imiquimod, and 5-fluorouracil (FU).

Background: Noninvasive treatments are frequently used in treatment of superficial basal cell carcinoma (sBCC) because of better cosmetic results, lower costs, and less burden on health care services when compared with surgical excision. However, probability of treatment failure is higher after noninvasive therapies and may depend on histologic tumor characteristics.

Objective: We sought to investigate whether tumor thickness and adnexal extension are determinants of treatment failure in sBCC treated with topical methylaminolevulinate-photodynamic therapy, imiquimod, or 5-fluorouracil.

BCR-ABL negative myeloproliferative neoplasia: a review of involved molecular mechanisms.

The clonal bone marrow stem cell disorders essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome negative myeloproliferative neoplasia (Ph- MPN). In 2005 the JAK2(V617F) mutation was discovered which has generated more insight in the pathogenetic mechanism of the MPNs. More mutations have been detected in MPN patients since.

Correlation between histological findings on punch biopsy specimens and subsequent excision specimens in cutaneous squamous cell carcinoma.

Diagnosis and subsequent treatment of cutaneous squamous cell carcinoma are frequently based on punch biopsies. Regarding the current TNM classification and stage grouping for cutaneous squamous cell carcinoma, it is important to identify the high-risk features (infiltration depth > 4 mm, perineural and/or lymphovascular invasion and poor differentiation). This study investigates the agreement of histological high-risk features and TNM grouping stage on 3 mm punch biopsies and subsequent surgical excision in 105 patients diagnosed with cutaneous squamous cell carcinoma.

Anti-apoptotic pathways in bone marrow and megakaryocytes in myeloproliferative neoplasia.

Objective: Proliferative activity contributes to bone marrow cellularity in myeloproliferative neoplasia (MPN). Megakaryocytes are the most important cells in MPN bone marrow pathology. JAK2(V617F) mutation constitutively activates JAK2, pErk (phosphorylating extracellular signal-regulated kinase) and PI3K (phosphatidylinositol 3-kinase)-Akt signaling.

Clinical and histological prognostic factors for local recurrence and metastasis of cutaneous squamous cell carcinoma: analysis of a defined population.

Cutaneous squamous cell carcinomas (cSCC) can recur locally and can metastasize. The objective of this study was to identify clinical and histopathological prognostic factors for local recurrence and metastasis in cSCCs at any body site. Clinical and histopathological data were collected from 224 patients with cSCC.

Reproducibility of histologic classification in nonfibrotic myeloproliferative neoplasia.

Early phases of polycythemia vera, essential thrombocythemia, and primary myelofibrosis (PMF) can be difficult to distinguish by morphologic studies alone because they share many morphologic characteristics. Histologic criteria according to the 2008 World Health Organization (WHO) classification are part of the myeloproliferative neoplasia (MPN) diagnosis. Our aim was to assess the reproducibility of morphologic characteristics and determine their relative importance for histologic diagnoses on selected trephine biopsy sections.

Differentiation between basal cell carcinoma and trichoepithelioma by immunohistochemical staining of the androgen receptor: an overview.

Clinical and histopathological differentiation between basal cell carcinoma (BCC) and trichoepithelioma (TE) is a frequent problem. Attempts have been made to identify immunohistochemical markers helpful in differentiating them. A correct diagnosis is important because the tumours are treated differently.

Correlation between histologic findings on punch biopsy specimens and subsequent excision specimens in recurrent basal cell carcinoma.

Background: The type of treatment for a basal cell carcinoma (BCC) depends on the histologic subtype. Histologic examination is usually performed on incisional biopsy specimens. In primary BCC, the histologic subtype is correctly identified with a punch biopsy in 80.

T-cell large granular lymphocytic leukaemia with an uncommon clinical and immunological phenotype.

A 39-year-old man presented with a rapidly growing unilateral painless nodule on the right cheek. Histopathological examination and peripheral blood analysis both showed a population of T-cell large granular lymphocytes, which were CD1+, CD2+, CD5+, CD7+ and CD16+, with expression of cutaneous lymphocyte-associated antigen. Further laboratory examination revealed severe neutropaenia, relative lymphocytosis and a clonally rearranged T-cell receptor.

Hyperkeratosis lenticularis perstans (Flegel's disease) - successful treatment with topical corticosteroids.

Flegel's disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small, red-brown, hyperkeratotic, papules that are usually located on the lower extremities. The diagnosis is based on the clinical appearance in association with the typical histologic features of orthohyperkeratosis and a subepidermal band-like infiltrate. Treatment is difficult and rarely fully effective.

Eruption of lymphocyte recovery or autologous graft-versus-host disease?

Maculopapular exanthemas have a particular high incidence among patients treated with autologous hematopoietic stem cell transplantation (HSCT). In most cases, a viral or drug induced origin is easily identified. However, the transplantation itself may also induce similar skin changes.

Coincidence of cutaneous follicle center lymphoma and diffuse large B-cell lymphoma.

Primary cutaneous follicle center lymphoma (PCFCL) is a neoplasm with differentiation of centrocytes and centroblasts presenting in the skin. At the time of initial manifestation, extracutaneous involvement is absent. PCFCL is considered as an indolent variant of primary cutaneous B-cell lymphomas since dissemination to extracutaneous sites is rare and the prognosis is favorable.

Absence of fumarate hydratase mutation in a family with cutaneous leiomyosarcoma and renal cancer.

A 41-year-old man was diagnosed with a cutaneous leiomyosarcoma on the left shoulder. Family history revealed that his brother had died of a metastatic kidney tumor at young age. Although apparently rare, the familial occurrence of cutaneous leiomyosarcoma with renal cancer has been described in the context of hereditary cutaneous leiomyomatosis and renal cell cancer (HLRCC).

Basal cell carcinoma and trichoepithelioma: a possible matter of confusion.

Difficulty in differentiation between a solitary basal cell carcinoma, which is known as a malign skin lesion and a benign trichoepithelioma, is a frequent problem in all day dermatologic practice. Clinically as well as histopathologically there are a lot of resemblances between these skin tumors. By means of two real life cases, we give here an overview of the possible problems and appliances in distinguishing these two entities; at the end we do some recommendation about the policy.

Penile pyoderma gangrenosum: successful treatment with colchicine.

Pyoderma gangrenosum is an ulcerative skin disease with variable clinical outcomes. The diagnosis is based on clinical features and exclusion of other ulcerative diseases. To date, a specific treatment is not known.

The absence of high-risk HPV combined with specific p53 and p16INK4a expression patterns points to the HPV-independent pathway as the causative agent for vulvar squamous cell carcinoma and its precursor simplex VIN in a young patient.

Simplex vulvar intraepithelial neoplasia (VIN) is an important precursor of vulvar invasive squamous cell carcinoma and characteristically occurs in postmenopausal women. In this report, the absence of high-risk human papillomavirus (HPV) combined with specific p53 and p16INK4a expression patterns points to the HPV-independent pathway as the causative agent for vulvar squamous cell carcinoma in a 28-year-old woman. Its precursor simplex VIN was initially interpreted as eczema.

Multicentric reticulohistiocytosis: case report.

A 64-year-old woman developed slowly expanding asymptomatic red bumps on the hands and in the face since 1 year. Histopathological examination showed mononucleated and multinucleated giant cells with a characteristic "ground glass" appearance. Based on the clinical and histopathological findings, we made the diagnosis of multicentric reticulohistiocytosis (MRH).

Cutaneous granulomas in rheumatoid arthritis.

A 57-year-old man with rheumatoid arthritis and myasthenia gravis developed an asymptomatic erythematous plaque on his nose and skin-colored nodules on the elbows. Histopathological examination was suggestive of palisaded neutrophilic granulomatous dermatitis (PNGD). PNGD is a rather uncommon cutaneous finding in different collagen vascular disorders that can manifest with a broad range of clinical symptoms and histological signs.

Lupus erythematosus associated with erythema multiforme: Rowell's syndrome.

About 45 years ago, the association of lupus erythematosus with erythema multiforme and specific immunological laboratory changes was described for the first time. Since then, several patients with similar constellations of clinical symptoms and laboratory parameters have been reported. Today, this disease is known as Rowell's syndrome.

Erythema nodosum as an early sign of Crohn's disease.

Erythema nodosum is the most common form of septal panniculitis and the most frequent skin manifestation associated with inflammatory bowel disease. Since the development of erythema nodosum is closely related with a variety of disorders and conditions, it can serve as an important early sign of systemic disease. We report on a 25-year-old woman with Crohn's disease following a long history of recurrent erythema nodosum.

Lichen planus with bullous manifestation on the lip.

A 75-year-old Caucasian female with a previous history of cutaneous lichen planus (LP) developed vesiculae and blisters on the lip. This special variant of oral LP is very rare and has to be distinguished from LP pemphigoides, bullous pemphigoid, pemphigus vulgaris, erythema multiforme, and herpes simplex virus infection. To date, there are only few reports on bullous oral LP on the lower lip.