Extranodal Follicular Dendritic Cell Sarcoma Presenting on the Skin.

ABSTRACT: Follicular dendritic cell sarcoma is a rare intermediate-grade malignancy characterized by a proliferation of ovoid to spindle-shaped cells with morphologic and immunophenotypic features similar to normal follicular dendritic cells. It may develop in lymph nodes or extranodal sites. Its presentation in extranodal tissues is a diagnostic challenge. It requires a high index of suspicion because follicular dendritic cell markers are not included in the routine immunohistochemical panels used for differential diagnosis. In an extensive review of the English literature, we found 3 cases of follicular dendritic cell sarcoma developing on the skin. We report a case of a primary cutaneous follicular dendritic cell sarcoma in a 28-year-old man, which presented as a 6-mm skin-colored nodule on the right forearm. We describe the morphologic and immunohistochemical features and a review of the literature.

Orbital vascular malformation: Successful outcome in two patients treated with rapamycin.

Combined vascular malformations are complex vascular anomalies that have high morbidity and therefore, therapeutic strategies are hard to establish. In this report, we aim to present two pediatric cases of ocular combined vascular malformations successfully treated with rapamycin.

Skin collision tumour and dermoscopic diagnosis of melanoma in situ and dilated pore of Winer.

Collision tumours are a random association of two or more neoplasms arising in the same anatomical site. Dilated pore of Winer (DPW) - an adnexal tumour of pilar origin - has been described in collisions with basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and trichoblastoma. We present a literature review of melanoma-associated skin lesions and describe the first collision of a DPW with a melanoma in situ (MIS) and the sequential dermoscopic examination for diagnosis.

Predictive Model for Response Rate to Narrowband Ultraviolet B Phototherapy in Vitiligo: A Retrospective Cohort Study of 579 Patients.

Vitiligo is an acquired depigmenting disorder. To date, there is no predictive model for its response rate to narrowband ultraviolet B (NBUVB) phototherapy. The aim of this study was to investigate the different types of response of patients with non-segmental vitiligo undergoing NBUVB 3 times a week. Many patients who were previously considered non-responders were given the opportunity to continue the treatment. Long-term maintenance of treatment and follow-up of a cohort of 579 patients enabled different subtypes of response (very rapid, rapid, average, slow and "non-responders") to be described for the first time, and a predictive model of response to be constructed based on repigmentation rate in the first 48 sessions of NBUVB. Among those patients who did not respond during the first 48 sessions, a new subgroup of patients was found, termed "very-slow" responders, who achieved a low, but significant, level of repigmentation after 96 sessions of NBUVB.

Persistent Nasal Infantile Hemangioma: A Surgical Treatment Algorithm.

Treatment of infantile hemangioma is usually medical. The nose is one of the most important aesthetic and functional units of the face; therefore, surgical management is preferred in persistent lesions (fibro-fatty tissue component) that do not respond to medical treatment. Herein, the authors analyze the results of surgical nasal hemangioma treatment in their center, a literature review and propose an algorithm for surgical management. A retrospective analysis of 23 persistent nasal hemangioma operated between 1996 and 2014 at our institution was made. The authors recollected the following demographic and clinical data: hemangioma subtype, phase of evolution, affected aesthetic nasal subunit, previous treatment, surgery type, complications and follow-up period. The Strasser scale was chosen for assessment of postoperative photographic results. Surgical treatment was performed. Age average was 6.8 years old (range 2-19). Mixed hemangioma was the most common subtype (83%). Surgery was mainly performed in the involution phase (87%). The most affected aesthetic subunit was nasal tip (44%). All lesions received previous medical treatment. The most common surgical technique was open rhinoplasty approach. Follow-up was an average of 26.6 months. Aesthetic results according to the Strasser method included: 3 excellent scores, 15 good, 5 regular, and none poor. Surgery was performed on patients in order to correct nasal persistent hemangiomas sequel. The nose has serious psychosocial impact and mixed infantile hemangiomas appear to have a higher rate of persistent hemangioma requiring surgery, but further studies are needed. Aesthetic outcome with surgical procedures chosen were acceptable and no complications were reported.

Bullous systemic lupus erythematosus: An uncommon manifestation in pediatric population. / Lupus eritematoso sistémico buloso: Una manifestación infrecuente en población pediátrica.

INTRODUCTION: Bullous systemic lupus erythematosus (BSLE) is an autoimmune subepidermal blistering disease se condary to the presence of autoantibodies against type VII collagen of the basement membrane zone. It is considered a variant of Systemic Lupus Erythematosus (SLE) and is uncommon in the pediatric population. OBJECTIVE: To describe the case of a pediatric patient with a bullous eruption compati ble with BSLE. CLINICAL CASE: A 16-year-old female patient of Mapuche descent with history of SLE diagnosed at age 10, undergoing treatment. She consulted due to a six-week history of a generalized bullous eruption with no systemic symptoms. Biopsy for histology and direct immunofluorescence (DIF) confirmed the diagnosis of BSLE. The patient responded favorably to dapsone 100 mg/day (associated with her baseline treatment), without new reactivations after 8 years of follow-up. Con clusion: BSLE is an infrequent manifestation of SLE. The clinical presentation is similar to other bullous dermatoses, but the histopathology and DIF in correlation with the presence of SLE confirm the diagnosis. Although indigenous ancestry is associated with SLE high-risk alleles, studies regarding the association of BSLE in this ethnic group are still lacking.

Unusual Clinical Presentations of Malignant Melanoma: A Review of Clinical and Histologic Features with Special Emphasis on Dermatoscopic Findings.

This review presents the main challenges encountered when diagnosing unusual variants of malignant melanoma with the aim of raising awareness to allow application of the most appropriate treatment strategies. Although these melanomas are often rare, their misdiagnosis potentially jeopardizes patients' health and survival, and has medicolegal implications. The clinical and histologic presentations of melanoma vary greatly, and assessment of uncommon melanomas can be difficult for practitioners because of their scarcity and resemblance to other dermatologic entities. The most problematic melanoma types are desmoplastic melanoma, polypoid melanoma, primary dermal melanoma, verrucous malignant melanoma, pigmented epithelioid melanocytoma, mucosal melanoma, follicular melanoma and melanoma with non-melanocytic differentiation. The two most difficult-to-diagnose subtypes of melanoma are the nevoid and the amelanotic melanomas. Some specific attributes of these variants can be more easily recognized with digital dermatoscopy, facilitating early detection and possibly avoiding invasive procedures. Key cases with the most notable clinical, dermatoscopic, and histopathologic features are presented, highlighting the practical issues of making an accurate diagnosis and choosing the best therapy.

Formas cutáneas y mucosas infrecuentes de melanoma: parte I / Skin and mucosal forms infrequent melanoma: part I

Se han descrito varios subtipos infrecuentes de melanoma maligno en la literatura médica. A pesar de su baja frecuencia ­ que no suele superar el dos por ciento en varias series de melanoma ­ es muy importante reconocerlos precozmente pues un diagnóstico tardío está asociado a un muy mal pronóstico. En esta primera parte incluiremos; melanoma animal, melanoma desmoplásico, melanoma dérmico primario, melanoma en mucosas y melanoma nevoide.

Several infrequent subtypes of malignant melanoma have been described in the medical literature. Although their low frequency ­ less than two percent in various series of melanoma ­ it is very important to early recognize them because late diagnosis is associated with a very poor prognosis. In this first manuscript we will include; animal melanoma, desmoplastic melanoma, dermal melanoma, mucous melanoma and nevoid melanoma.

Formas cutáneas y mucosas infrecuentes de melanoma: parte II / Skin and mucosal forms infrequent melanoma: part II

Se han descrito varios subtipos infrecuentes de melanoma maligno en la literatura médica. A pesar de su baja frecuencia ­ que no suele superar el dos por ciento en varias series de melanoma ­ es muy importante reconocerlos precozmente pues un diagnóstico tardío está asociado a un muy mal pronóstico. En esta segunda parte incluiremos: melanoma verrugoso, melanoma polipoideo, melanoma hipomelanótico y amelanótico, melanoma folicular, melanoma spitzoide, melanoma con diferenciación no melanocítica.

Several infrequent subtypes of malignant melanoma have been described in the medical literature. Although their low frequency ­ less than two percent in various series of melanoma ­ it is very important to early recognize them because late diagnosis is associated with a very poor prognosis. In this second part we will include: verrucous melanoma, polypoid melanoma, hypomelanotic and amelanotic melanoma, follicular melanoma, spitzoid melanoma and non-melanocytic differentiation melanoma.