Lentiginous melanoma (lentigo maligna and lentigo maligna melanoma) in Australia: clinicopathological characteristics, management and recurrence rates after 10-year follow-up at a tertiary centre.

BACKGROUND: Lentiginous melanoma or lentigo maligna is a slow-growing type of melanoma frequently arising in sun-damaged skin and often first diagnosed in the elderly. Few studies report long-term follow-up. OBJECTIVES: To define characteristics of lentiginous melanoma in situ (LM) and invasive lentiginous melanoma (LMM) in Australian patients managed at a tertiary centre and describe local recurrence or treatment failure rates after long-term follow-up. METHODS: Retrospective single-centre study of LM/LMM patients evaluated between January 2005 and March 2007. Medical and photographic records were reviewed. RESULTS: One hundred two patients were included, with a total of 117 lesions (70 LM and 47 LMM). Seventy-nine were new primary LM/LMM, and 38 were recurrences. Primary cases were mostly pigmented (71%), while 77% of recurrent cases were partially pigmented/light brown or amelanotic. The margins were clinically ill-defined in the majority of cases (64% of primary cases and 94% of recurrent cases). Dermoscopy of the primary LM/LMM showed either classic 'common' melanoma features (33%) or classic LM/LMM features (41%), while 95% of recurrent cases had no features for melanoma or LM/LMM. Primary cases that were initially excised (113, 97%) had mean histopathological clear margins of 4.9 mm (range 0.1-22 mm). The median follow-up time was 7.5 years (95% CI 5.2-10.0) with more than 10-year follow-up in 32% and 5-10 years in 24% of patients. There were 44 (38%) recurrences over the entire follow-up period. Half of the patients who recurred did so within the first 3.8 years after the first treatment. CONCLUSION: LM/LMM often recur late and are clinically subtle; therefore, careful monitoring and long-term follow-up are required.

Cutaneous toxicities of new treatments for melanoma

New drugs against advanced melanoma have emerged during last decade. Target therapy and immunotherapy have changed the management of patients with metastatic disease. Along with its generalized use, drug toxicities have appeared and the skin is the target organ of a significant part of them. This revision summarizes the most common side effects and consensus management to improve the compliance of therapies and patients’ quality of life. Among the BRAF inhibitors, main cutaneous side effects are photosensitivity, plantar hyperkeratosis, and the appearance of verrucal keratosis or squamous cell carcinoma. Special attention must be paid to the development of new primary melanomas or changes on nevi during BRAF inhibitor therapy. The most common cutaneous side effects of immunotherapy are rash, pruritus, and vitiligo. It remains controversial the possible role of these toxicities as markers of response to therapy

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Cutaneous toxicities of new treatments for melanoma.

New drugs against advanced melanoma have emerged during last decade. Target therapy and immunotherapy have changed the management of patients with metastatic disease. Along with its generalized use, drug toxicities have appeared and the skin is the target organ of a significant part of them. This revision summarizes the most common side effects and consensus management to improve the compliance of therapies and patients' quality of life. Among the BRAF inhibitors, main cutaneous side effects are photosensitivity, plantar hyperkeratosis, and the appearance of verrucal keratosis or squamous cell carcinoma. Special attention must be paid to the development of new primary melanomas or changes on nevi during BRAF inhibitor therapy. The most common cutaneous side effects of immunotherapy are rash, pruritus, and vitiligo. It remains controversial the possible role of these toxicities as markers of response to therapy.

In vivo confocal microscopy for the oral cavity: Current state of the field and future potential.

Confocal microscopy (CM) has been shown to correlate with oral mucosal histopathology in vivo. The purposes of this review are to summarize what we know so far about in vivo CM applications for oral mucosal pathologies, to highlight some current developments with CM devices relevant for oral applications, and to formulate where in vivo CM could hold further application for oral mucosal diagnosis and management. Ovid Medline® and/or Google® searches were performed using the terms 'microscopy, confocal', 'mouth neoplasms', 'mouth mucosa', 'leukoplakia, oral', 'oral lichen planus', 'gingiva', 'cheilitis', 'taste', 'inflammatory oral confocal', 'mucosal confocal' and 'confocal squamous cell oral'. In summary, inclusion criteria were in vivo use of any type of CM for the human oral mucosa and studies on normal or pathological oral mucosa. Experimental studies attempting to identify proteins of interest and microorganisms were excluded. In total 25 relevant articles were found, covering 8 main topics, including normal oral mucosal features (n=15), oral dysplasia or neoplasia (n=7), inflamed oral mucosa (n=3), taste impairment (n=3), oral autoimmune conditions (n=2), pigmented oral pathology/melanoma (n=1), delayed type hypersensitivity (n=1), and cheilitis glandularis (n=1). The evidence for using in vivo CM in these conditions is poor, as it is limited to mainly small descriptive studies. Current device developments for oral CM include improved probe design. The authors propose that future applications for in vivo oral CM may include burning mouth syndrome, intra-operative mapping for cancer surgery, and monitoring and targeted biopsies within field cancerization.

Agminated cellular blue naevi of the penis: dermoscopic, confocal and histopathological correlation of two cases.

Blue naevi may present rarely as multiple lesions grouped in a circumscribed area, described as agminated blue naevi. This clinical presentation may mimic metastatic malignant melanoma. We present two cases of agminated cellular blue naevi of the penis, with dermoscopy, reflectance confocal microscopy and histopathological correlation. Dermoscopy of the area showed multiple grouped lesions of homogeneous dark-brown to blue colour. Using reflectance confocal microscopy, focusing on the bluish areas, predominantly bright dendritic cells were visible at the dermoepidermal junction and papillary dermis, while in the brownish areas the presence of dendritic and bright cells predominated in the basal layer. Our patients are of special interest as they are the first cases, to our knowledge, reported of agminated blue naevi on the penis, studied by both dermoscopy and confocal microscopy, confirming the diagnosis with histopathological correlation. Moreover, one case represented a divided or 'kissing' blue naevus of the penis.

Leiomiomatosis cutánea y uterina múltiple o síndrome de Reed: estudio retrospectivo de 13 casos / Multiple Cutaneous and Uterine Leiomyomatosis or Reed Syndrome: A Retrospective Study of 13 Cases

Introducción: La leiomiomatosis cutánea y uterina múltiple (MCUL) o síndrome de Reed se caracteriza por la presencia de leiomiomas cutáneos de origen pilar, leiomiomas uterinos en las mujeres y, en algunos casos, asociación con carcinoma renal. Este síndrome, de herencia autosómica dominante, se produce por una mutación heterocigótica en la línea germinal del gen de la fumarato hidratasa, una enzima del ciclo de Krebs que actúa como supresor tumoral. Objetivo: Revisar los casos de MCUL diagnosticados en 2 hospitales universitarios durante un periodo de 5 años (2008-2013). Material y métodos: Estudio retrospectivo de 13 casos de MCUL, en el que se recogieron características demográficas, clínicas e histológicas, así como posibles asociaciones con otras enfermedades y tratamientos recibidos. Resultados: Trece pacientes fueron diagnosticados de MCUL (10 mujeres y 3 hombres, con una edad media al diagnóstico de 53 años). El 100% de los casos presentaba leiomiomas cutáneos múltiples, distribuidos de forma difusa (69%), agrupada (92%) y/o lineal (7,7%). El 90% de las mujeres presentaba además miomas uterinos y todas ellas habían precisado histerectomía porese motivo. No encontramos ningún caso de carcinoma renal en los pacientes explorados (9/13), pero sí lesiones renales benignas (4/9). Conclusión: Describimos 13 casos de MCUL, que presentan características clínicas e histológicas similares a las descritas en la literatura, siendo la manifestación cutánea más frecuente la segmentaria tipo 2. Es importante que el dermatólogo identifique los casos de leiomiomas cutáneos y conozca su posible relación con MCUL (AU)

Introduction: Multiple cutaneous and uterine leiomyomatosis (MCUL), or Reed syndrome, is characterized by the presence of cutaneous leiomyomas arising from the arrector pili muscles and, in women, by uterine leiomyomas. In some cases, MCUL is associated with renal cell carcinoma. This syndrome is an autosomal dominant disorder caused by a heterozygous germline mutation of the gene that encodes fumarate hydratase, a Krebs cycle enzyme that acts as a tumor suppressor. Objective: To review the cases of MCUL diagnosed at 2 university hospitals over a 5-year period (2008-2013). Material and methods: This was a retrospective study of 13 cases of MCUL that investigated demographic, clinical, and histologic characteristics, as well as possible associations with other diseases and treatments received. Results: We identified 13 patients (10 women and 3 men) who had been diagnosed with MCUL. The mean age at diagnosis was 53 years. All the patients had multiple cutaneous leiomyomas; in 12 (92%) the distribution was clustered and 9 (69%) also had disseminated solitary lesions. In 1 patient (7.7%), the pattern of distribution was linear. Uterine fibroids requiring hysterectomy were present in 90% of the women. Nine patients were screened for renal lesions; no cases of renal cell carcinoma were detected but benign renal lesions were found in 4 patients. Conclusion: The clinical and histologic characteristics of the 13 cases of MCUL reviewed were similar to those reported in the literature. The most common cutaneous manifestation was a type 2 segmental pattern. It is important for dermatologists to identify cutaneous leiomyomas and be aware of the possible association with MCUL (AU)

FR-Lentigo maligno, actualización en técnicas diagnósticas y terapéuticas / Diagnostic and Therapeutic Techniques in Lentigo Maligna: An Update

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Multiple cutaneous and uterine leiomyomatosis or reed syndrome: a retrospective study of 13 cases.

INTRODUCTION: Multiple cutaneous and uterine leiomyomatosis (MCUL), or Reed syndrome, is characterized by the presence of cutaneous leiomyomas arising from the arrector pili muscles and, in women, by uterine leiomyomas. In some cases, MCUL is associated with renal cell carcinoma. This syndrome is an autosomal dominant disorder caused by a heterozygous germline mutation of the gene that encodes fumarate hydratase, a Krebs cycle enzyme that acts as a tumor suppressor. OBJECTIVE: To review the cases of MCUL diagnosed at 2 university hospitals over a 5-year period (2008-2013). MATERIAL AND METHODS: This was a retrospective study of 13 cases of MCUL that investigated demographic, clinical, and histologic characteristics, as well as possible associations with other diseases and treatments received. RESULTS: We identified 13 patients (10 women and 3 men) who had been diagnosed with MCUL. The mean age at diagnosis was 53 years. All the patients had multiple cutaneous leiomyomas; in 12 (92%) the distribution was clustered and 9 (69%) also had disseminated solitary lesions. In 1 patient (7.7%), the pattern of distribution was linear. Uterine fibroids requiring hysterectomy were present in 90% of the women. Nine patients were screened for renal lesions; no cases of renal cell carcinoma were detected but benign renal lesions were found in 4 patients. CONCLUSION: The clinical and histologic characteristics of the 13 cases of MCUL reviewed were similar to those reported in the literature. The most common cutaneous manifestation was a type 2 segmental pattern. It is important for dermatologists to identify cutaneous leiomyomas and be aware of the possible association with MCUL.

Use of biological treatments in patients with hidradenitis suppurativa.

INTRODUCTION: Hidradenitis suppurativa (HS) is a chronic skin disease which causes a great impact in the quality of life. Multiple therapeutic options have been proposed, and recently the potential use of biological drugs in severe cases has been postulated. MATERIAL AND METHODS: A retrospective study from seven tertiary Spanish centers reviewing the charts of patients with HS treated with biological drugs was performed. Retrieved information included epidemiological data, clinical features, pain intensity, Hurley stage, laboratory data and therapeutic outcomes. RESULTS: Nineteen patients were included in the study; 10 men (52.6%) and 9 women. Eight patients (42%) showed a Hurley severity stage II and 11 a stage III (57.8%). Adalimumab was prescribed as the first biological treatment in nine out of 19 cases (47.3%), whereas infliximab was prescribed in seven cases (36.8%), ustekinumab in two cases (10.5%) and etanercept in one (5.2%). A complete response was observed in three patients (two cases with infliximab and one case with ustekinumab), a partial improvement in 10 patients and in six patients no clinical improvement was noted. One patient referred worsening of the skin symptoms. In 6 cases, a second biological treatment was prescribed. In three of such cases, a partial improvement was noted, whereas in three cases no clinical improvement was observed. In two cases a switch to a third biological drug was indicated, with a partial improvement in one case. DISCUSSION AND CONCLUSIONS: Biological drugs could be a potential and effective therapeutic option for patients with severe HS. Complete and persistent clinical responses are rarely obtained (15%) and partial responses are achieved in approximately 50% of patients. No specific markers for a therapeutic response have been identified. No definitive conclusions regarding the most effective biological drug for HS could be drawn. Higher dosage schedules seem to be associated with higher response rates. The lack of response of one particular drug does not preclude a potential efficacy to another biological treatment.

Dermanyssus gallinae (chicken mite): an underdiagnosed environmental infestation.

Dermanyssus gallinae is a mite that normally parasitizes small birds but may occasionally bite humans. We report an unusual case of an 82-year-old woman who presented with pruritus and bite-like lesions over her trunk. Other members of the household were also affected. On physical examination, mites < 1 mm in size were found on the patient's body. The family were residing in the city centre and had no pets, but there were pigeon nests in close proximity to the house. Most dermatologists have difficulties identifying ectoparasitosis. In the case of D. gallinae, the small size of the mites and the fact that they leave the host after feeding means that they may not be seen at presentation, thus such infestations are likely to be underdiagnosed. Physicians should be aware that infection with this mite is possible even in patients from urban areas, and it should be included in the differential diagnosis of conditions causing recurrent pruritus unresponsive to standard treatments.

Psoriasis ungueal: tratamiento con ungüento hidrófilo de tazaroteno al 0.1 por ciento / Nail Psoriasis: Treatment With Tazarotene 0.1 percent Hydrophilic Ointment

Introducción: La afectación ungueal de la psoriasis es una presentación frecuente que interfiere de manera significativa en la calidad de vida de los pacientes. Su presentación clínica va a depender del área ungueal afecta: lecho o matriz. Un 50% de los pacientes refiere dolor asociado. En este estudio evaluamos la eficacia y seguridad del tazaroteno 0,1% en ungüento hidrófilo. Material y métodos: Estudio abierto y observacional de 6 pacientes diagnosticados de psoriasis ungueal. Se aplicó ungüento de tazaroteno 0,1% (fórmula magistral) en oclusión nocturna, en su domicilio durante 6 meses, sin otro tratamiento tópico o sistémico. Se determinó el Nail Psoriasis Severity Index (NAPSI) y se evaluaron la hiperqueratosis subungueal, onicólisis, hemorragias en astilla, manchas de aceite y piquiteado ungueal, en la visita basal, a los 3 y 6 meses. Resultados: Se observó una mejoría estadísticamente significativa en todos los pacientes: NAPSI basal, media ± DE 14,3± 6,3; IC 95% 11,74-16,92; mediana 15; NAPSI a los 6 meses: media ± DE 2,3 ±1,21; IC 95% 1,84-2,83; mediana 2,5; p=0,007. El porcentaje de mejoría fue del 87,9% al final del tratamiento. No se registraron efectos adversos. Conclusión: Nuestro estudio muestra un potencial terapéutico del ungüento de tazaroteno en la psoriasis ungueal (AU)

Introduction: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. Material and methods: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. Results: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = 0.007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. Conclusion: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis (AU)

Nail psoriasis: treatment with tazarotene 0.1% hydrophilic ointment.

INTRODUCTION: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. MATERIAL AND METHODS: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. RESULTS: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = .007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. CONCLUSION: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis.