Specific Cutaneous Lesions in Patients With Neurosarcoidosis. / Lesiones cutáneas específicas en pacientes con neurosarcoidosis.

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis.

Changes in the Location of Cutaneous Melanoma Over the Past 30 Years. A Retrospective Observational Study. / Cambios en la localización del melanoma cutáneo en los últimos 30 años. Estudio observacional retrospectivo.

BACKGROUND: The location of cutaneous melanoma is associated with photoexposure. OBJECTIVES: To retrospectively analyze changes in the location of cutaneous melanoma over the past 30 years. PATIENTS AND METHODS: All patients treated at our hospital for cutaneous melanoma from 1988 through 2017 were prospectively collected. Data obtained in cases diagnosed from 1988 through June 2006 were compared to those diagnosed from July 2006 through 2017. RESULTS: A total of 1,937 patients (876 men and 1061 women; median age, 57 years; interquartile range 27) were diagnosed with primary cutaneous melanoma. The location of melanoma was head and neck (470 cases), trunk (745 cases), upper limbs (239 cases), and lower limbs (483 cases). From July 2006 through 2017 we detected an increase in the incidence of head and neck melanomas (19.9% vs 28.6%, p <0.001). A drop in the incidence of melanomas located in the lower extremities was also seen in women (39.8% vs 30.4%, p <0.001), and in the trunk men (57.5% vs 47.3%, p=0.003). In the multivariate analyses, only the decrease in melanomas located in lower extremities in women remained significant. CONCLUSION: The increased incidence of head and neck melanomas in both sexes and the decrease in trunk melanomas in men can be attributed to the aging of our population. The reduction in the incidence of melanomas in the lower extremities in women could be associated with changes in photoexposure patterns. Analyzing the factors possibly associated with these changes would contribute to better understanding the pathogenesis of cutaneous melanoma for prevention purposes.

Clear Cell Acanthoma: A Series of 70 Cases. / Acantoma de células claras. Estudio clínico de 70 pacientes.

BACKGROUND AND OBJECTIVE: Clear cell acanthoma (CCA) is an uncommon lesion histologically characterized by the presence of epidermal acanthosis with keratinocytes containing clear cytoplasm. Although many single cases of CCA have been described, few case series have been published. The aim of this study was to describe the clinical characteristics of CCA in our practice setting. MATERIAL AND METHODS: Retrospective study of patients diagnosed with CCA at Hospital Universitario de Bellvitge in Barcelona, Spain, between 1995 and 2021. We conducted a chart review to record age, sex, number and location of lesions, diameter, time since onset, clinical characteristics, suspected clinical diagnosis, and treatment. RESULTS: Seventy patients (30 women and 40 men) with a mean (SD) age of 62 (13) years were diagnosed with CCA during the study period. Median (interquartile range) time since onset was 2 (4) years and median lesion diameter was 6 (5) mm. One woman had multiple lesions. Lesions were located on the lower extremities in 57 patients (81%), the posterior aspect of the trunk in 8 (11%), the anterior aspect of the trunk in 4 (5%), and the upper extremities in 1 (1%). CCA was clinically suspected in 40% of patients seen by dermatologists. CONCLUSIONS: CCA presents as an erythematous, dome-shaped lesion with pinpoint vessels and an epidermal collarette. The accuracy of clinical diagnosis has improved relative to earlier series, possibly due to a better clinical understanding of this lesion and a greater use of dermoscopy.

Dactylitis Due to Subcutaneous Sarcoidosis as a Manifestation of Sarcoidosis. / Dactilitis producida por sarcoidosis subcutánea como presentación de sarcoidosis.

Dactylitis, or sausage fingers or toes, refers to the swelling of 1 or more digits. Sarcoid dactylitis is an uncommon form that tends to be caused by granulomatous bone involvement in the underlying phalanges. Sarcoid dactylitis can, however, clinically resemble subcutaneous sarcoidosis with finger or toe involvement. We describe 5 patients with dactylitis due to subcutaneous sarcoidosis of the fingers. In all cases, the proximal phalanges were involved; sarcoid dactylitis, by contrast, tends to affect the medial and distal phalanges. Differential diagnosis is important, as dactylitis due to bone involvement tends to be much more persistent and is generally seen in chronic forms of sarcoidosis.

Poroma: A Retrospective Series of 80 Patients at a Tertiary Care Hospital. / Poroma. Estudio retrospectivo de 80 pacientes en un hospital terciario.

BACKGROUND: Poroma is a benign, exclusively cutaneous, adnexal tumor with a predilection for palmoplantar skin. OBJECTIVE: To analyze the clinical characteristics of poroma in our population. MATERIAL AND METHODS: Retrospective study of patients diagnosed with poroma between 2002 and 2021. We conducted a chart review to record age; sex; number, location, and diameter of lesions; time since onset; clinical characteristics; suspected clinical diagnosis; resection margin status; recurrences; and follow-up duration. Categorical variables were compared using the Fisher exact test. Continuous variables were compared using the t test or the Mann-Whitney U test depending on whether they were normally or nonnormally distributed. RESULTS: We studied 80 patients (31 women and 49 men) with a median (interquartile range [IQR]) age of 65.5 (29) years. Median time since onset of poroma was 12 (21) months. Median lesion diameter was 8(7)mm, and none of the patients had multiple lesions. The lesions were located on the head and neck in 13 cases, the trunk in 13, the upper extremities in 11, and the lower extremities in 43. Twenty-three lesions (28.8%) were located at acral sites (5 on the palms and 18 on the soles). Women were more likely to have scalp lesions (P=.041). Acral lesions were more likely to be erythematous (P=.014). Five patients experienced local recurrence. CONCLUSIONS: Although poromas are particularly common in acral locations (especially the feet), most of the lesions in our series (71.3%) were located elsewhere. Acral lesions were more likely to show the classic clinical features of erythema and exophytic growth.

Clinicopathological features, MCPyV status and outcomes of Merkel cell carcinoma in solid-organ transplant recipients: a retrospective, multicentre cohort study.

BACKGROUND: The proportion of Merkel cell carcinomas (MCCs) in solid-organ transplant recipients (SOTR) harbouring Merkel cell polyomavirus (MCPyV) is unknown, as are factors affecting their outcomes. OBJECTIVE: To describe clinicopathological features of MCC in SOTR, investigate the tumoral MCPyV-status and identify factors associated with tumour outcomes. METHODS: Retrospective, international, cohort-study. MCPyV-status was investigated by immunohistochemistry and polymerase chain reaction. RESULTS: A total of 30 SOTR and 44 consecutive immunocompetent patients with MCC were enrolled. SOTR were younger at diagnosis (69 vs. 78 years, P < 0.001). Thirty-three percent of SOTR MCCs were MCPyV-positive vs. 91% of immunocompetent MCCs (P = 0.001). Solid-organ transplantation was associated with an increased cumulative incidence of progression (SHR: 3.35 [1.57-7.14], P = 0.002), MCC-specific mortality (SHR: 2.55 [1.07-6.06], P = 0.034) and overall mortality (HR: 3.26 [1.54-6.9], P = 0.002). MCPyV-positivity and switching to an mTOR inhibitor (mTORi) after MCC diagnosis were associated with an increased incidence of progression (SHR: 4.3 [1.5-13], P = 0.008 and SHR: 3.6 [1.1-12], P = 0.032 respectively) in SOTR. LIMITATIONS: Retrospective design and heterogeneity of SOTR cohort. CONCLUSIONS: MCPyV appears to play a less prominent role in the aetiopathogenesis of MCC in SOTR. SOTR have a worse prognosis than their immunocompetent counterparts and switching to an mTORi after the diagnosis of MCC does not improve progression.

Evolution of Kaposi sarcoma in the past 30 years in a tertiary hospital of the European Mediterranean basin.

BACKGROUND: The incidence of Kaposi sarcoma (KS) has reduced as a result of the introduction of antiretroviral therapy. It is currently considered a rare disease in developed countries, and there has been a paucity of clinical papers on the subject in recent years in Europe. AIM: To analyse the clinical features and evolution of the different clinical forms of KS in the past 30 years. METHODS: Patients with cutaneous lesions of KS diagnosed during the period 1987-2016 at Bellvitge Hospital (an 800-bed university referral centre in Barcelona, Spain) were enrolled. Data recorded included age, sex, ethnicity, involved site, number of lesions, extracutaneous involvement, leg oedema, treatment, blood haemoglobin level, and blood cell (leucocyte, lymphocyte and CD4) counts. RESULTS: Cutaneous lesions of KS were diagnosed in 191 patients (167 men, 24 women, mean ± SD age 51.95 ± 20.16 years). Clinical forms identified were classic KS (n = 53), acquired immunodeficiency syndrome (AIDS)-associated KS (n = 118), immunosuppression-associated KS (n = 18), and African endemic KS (n = 2). The number of patients diagnosed annually reached a maximum in the 1990s because of the AIDS epidemic, and has decreased since 2000. However, both classic KS and immunosuppression-associated KS doubled from the first to the second half of the analysed period. Cutaneous lesions involved the legs in 137 cases, and extracutaneous lesions were detected in 32 patients. In 46 of 118 patients with AIDS, the diagnosis of KS was simultaneous to the detection of human immunodeficiency virus infection. CONCLUSION: After a decrease in incidence since the middle of the 1990s, AIDS-associated KS continues to occur in Europe, and the number of annual cases of classic KS and immunosuppression-associated KS is increasing.

Cutaneous sebaceous tumours and Lynch syndrome: long-term follow-up of 60 patients.

BACKGROUND: Sebaceous neoplasms (SN) may appear sporadically in the general population but may also be part of the Muir-Torre variant of Lynch syndrome (MT-LS). There are few studies in southern Europe on the incidence of MT-LS in the population of patients with SN. AIM: To retrospectively review patients with SN and to analyse their clinical features and the incidence of MT-LS. METHODS: Patients with SN diagnosed between 1995 and 2015 were enrolled in the study. The diagnosis of MT-LS was made according to established clinical criteria and, whenever possible, was confirmed by germline mutation analysis. RESULTS: In 60 patients (32 men, 28 women, mean age 69.22 years), 96 SN were diagnosed: 65 adenomas (67.7%), 16 sebaceomas (16.7%) and 15 carcinomas (15.6%). Of the 60 patients, 50 (83.3%) had a single SN and 10 (16.7%) had multiple lesions. Patients diagnosed with MT-LS (12 patients, 20%) were younger (63.25 years vs. 70.71 years), and had a higher incidence of extrafacial SN (4/12 patients, 33.3%), and were significantly (P < 0.001) more likely to have multiple SNs (8/12, 75%) and keratoacanthomas (KAs) (6/12, 50%). CONCLUSION: Our study confirms that all patients with SN should be investigated, as 20% of our patients were diagnosed with MT-LS. The most specific features of SN associated with MT-LS in our study were the presence of multiple lesions and association with KAs.

Cutaneous leishmaniasis associated with anti-tumour necrosis factor-α drugs: an emerging disease.

Leishmaniasis is endemic in several geographic areas of the world. In each of these areas, particular species of Leishmania with differing aggressiveness to humans predominate. In the European Mediterranean basin, cutaneous leishmaniasis usually presents with discrete, self-healing skin lesions. Although it is known that tumour necrosis factor (TNF) inhibitors may increase the risk of developing infections such as tuberculosis, there is scarce literature on Leishmania infections in patients treated with these drugs. In recent months, we have observed three patients resident in the Catalan coast of Spain who were treated with TNF inhibitors for Crohn disease, and who developed unusually large and persistent cutaneous lesions of leishmaniasis. These lesions responded only to treatment with intravenous liposomal amphotericin B. In countries with a high incidence of infection by aggressive species of Leishmania, serological screening may be indicated to detect a possible latent leishmanial infection before prescription of TNF inhibitors.

Sweet syndrome: long-term follow-up of 138 patients.

BACKGROUND: Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy-associated SS have been published in recent years. AIM: To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long-term follow-up of patients with SS. METHODS: Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. RESULTS: In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug-induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P < 0.01) and absence of arthralgia (OR = 11.13, P < 0.01). CONCLUSIONS: Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy-associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.

Necrobiosis lipoidica: a descriptive study of 35 cases.

INTRODUCTION AND OBJECTIVES: Necrobiosis lipoidica (NL) is a chronic idiopathic granulomatous disease considered to occur in association with diabetes mellitus. Data on the frequency of this association, however, are inconsistent. Our aim was to retrospectively analyze the clinical characteristics of patients diagnosed with NL at our hospital and to investigate the association with diabetes mellitus and other diseases. MATERIAL AND METHODS: We performed a chart review of all patients with a clinical and histologic diagnosis of NL treated and followed in the dermatology department of Hospital de Bellvitge in Barcelona, Spain between 1987 and 2013. RESULTS: Thirty-five patients (6 men and 29 women with a mean age of 47.20 years) were diagnosed with NL in the study period. At the time of diagnosis, 31 patients had pretibial lesions. Thirteen patients (37%) had a single lesion at diagnosis, and the mean number of lesions was 3.37. Twenty-three patients (65.71%) had diabetes mellitus (type 1 in 10 cases and type 2 in 13). In 20 patients, onset of diabetes preceded that of NL by a mean of 135.70 months. The 2 conditions were diagnosed simultaneously in 3 patients. None of the 35 patients developed diabetes mellitus during follow-up. Six patients had hypothyroidism, and 4 of these also had type 1 diabetes. CONCLUSIONS: NL is frequently associated with type 1 and 2 diabetes. Although diabetes tends to develop before NL, it can occur simultaneously.

Evolution of cutaneous tuberculosis over the past 30 years in a tertiary hospital on the European Mediterranean coast.

BACKGROUND: There have been few studies on cutaneous tuberculosis (TB) in Europe in recent years. Objective. To retrospectively analyse the evolution of the various types of cutaneous TB over the past 30 years in an adult population in Spain. METHODS: Patients with cutaneous TB diagnosed between 1981 and 2011 at Bellvitge Hospital, Barcelona, Spain, were included in the study. Chest radiography was performed for all patients, and the presence of TB elsewhere in the body was excluded when clinically suspected. RESULTS: In total, 36 patients (15 male, 21 female, mean age 53.72 years) were diagnosed with cutaneous TB. There were 22 patients with lupus vulgaris (LV), 4 with scrofuloderma, 4 with miliary TB, 3 with tuberculous abscess/ulcer, and 1 each with orificial TB, warty TB, and an iatrogenic inoculation from underlying visceral focus. Of the 36 patients, 16 (38.88%) had TB presenting simultaneously in other organs. Mycobacterial culture from skin biopsies was positive for Mycobacterium tuberculosis complex in 17 of the 32 cases tested (53.12%), whereas stains for acid-fast bacilli in skin samples were positive in only 3 of 36 patients (8.33%). CONCLUSIONS: Although the number of cases of cutaneous TB diagnosed yearly in our population has declined over the past 30 years, cutaneous TB still exists in Europe, and its incidence is expected to increase, owing to the increased immigration into the continent in recent years. The most common type of cutaneous TB in our adult population was LV. It should be noted that despite being considered a benign form of TB, cutaneous TB can be accompanied by TB in internal organs, and severe complications can occur, such as the development of squamous cell carcinoma in long-lasting lesions.

Patterns of visceral metastasis in cutaneous melanoma: a descriptive study.

BACKGROUND AND OBJECTIVES: Some types of cancer tend to spread to certain organs. In the case of melanoma, uveal melanoma spreads almost exclusively to the liver, while cutaneous melanoma spreads to the liver and other organs. Although important advances have been made in our understanding of the molecular mechanisms underlying melanoma, few recent studies have focused on the patterns of visceral metastasis in cutaneous melanoma. The aim of this study was to retrospectively investigate whether clinicopathologic variants of cutaneous melanoma and primary tumor site might be associated with pattern and time of onset of metastasis to visceral sites, including the central nervous system (CNS). MATERIALS AND METHODS: We included patients diagnosed with cutaneous melanoma between 1988 and 2009 with at least 2 years' follow-up. RESULTS: Of the 1083 patients studied, 92 developed visceral metastasis. The CNS was affected in 21 cases, the lungs in 24, the liver in 17, the digestive tract in 7, and multiple organs simultaneously in 23. Metastasis to the lungs, the liver, and the digestive tract occurred within 5 years in most cases, while metastasis to the CNS and multiple organs occurred later (>5 years in 38% and 43% of cases, respectively). CONCLUSIONS: Unlike uveal melanoma, cutaneous melanoma spreads to different organs without any particular predilection. We observed no significant associations between the site of visceral metastasis and either clinicopathologic variant or location of the primary tumor. Metastasis occurred within 5 years of diagnosis in most cases, but it can occur after 10 years.

Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease.

BACKGROUND: Specific (granulomatous) cutaneous lesions are seen in 9-37% of cases of systemic sarcoidosis, and are usually classified into maculopapules, plaques, lupus pernio (LP), scar sarcoidosis, and subcutaneous sarcoidosis. Their prognostic significance has not been fully established. AIM: To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis. METHODS: The clinical charts of 86 patients (19 men, 67 women, mean age 46.82 years) with systemic sarcoidosis and granulomatous cutaneous involvement followed up for > 2 years at Bellvitge University Hospital were reviewed. RESULTS: Cutaneous lesions developed before or at the time of diagnosis of systemic sarcoidosis in 80.23% of patients. The main cutaneous lesions were classified as maculopapules (28 patients), plaques (31), LP (6), scar sarcoidosis (7) and subcutaneous sarcoidosis (14). Erythema nodosum (EN) was seen in 30 patients. Radiological stage was 0 for 8 patients, I for 48, II for 24, III for 5 and IV for 1. Systemic sarcoidosis activity persisted for > 2 years in 47 patients, and 42 received systemic corticosteroid treatment for their disease. Maculopapular and subcutaneous sarcoidosis were mainly seen in patients with EN and radiological stage I. Plaques and LP were associated with chronic disease and requirement for systemic corticosteroids. CONCLUSIONS: Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.

[Descriptive analysis of cutaneous recurrence patterns in patients with melanoma]. / Análisis descriptivo de los patrones de recidiva cutánea en los pacientes con melanoma.

BACKGROUND AND OBJECTIVES: Few studies have addressed cutaneous recurrence of melanoma. The aim of this retrospective study was to analyze the characteristics and prognostic significance of the different patterns of cutaneous recurrence. MATERIAL AND METHODS: Patients diagnosed with melanoma between 1988 and 2008 at Hospital de Bellvitge, Barcelona, Spain and for whom data were available for at least 2 years of follow-up were included in the study. Local recurrence was defined as melanoma invasion of the skin adjacent to the scar left by excision of the primary tumor, regional metastasis or recurrence as metastasis restricted to the area drained by a regional lymph node station, and distant cutaneous metastasis as metastasis occurring outside this area. The relationship between cutaneous recurrence pattern and age, sex, primary tumor site, tumor subtype, Breslow depth, and ulceration was assessed. RESULTS: Eighty-five out of 1,080 patients (7.87%) had cutaneous recurrence. In 71 of those patients (83.53%; 27 men and 44 women; mean age, 60.68 years), this was the first indication of melanoma recurrence. Thirty-two patients had local recurrence, 32 regional metastasis, and 7 distant metastasis. Significant differences were observed in survival time from diagnosis of the primary tumor (P=.044) and from diagnosis of cutaneous recurrence (P<.001) according to the type of recurrence. CONCLUSIONS: Our results suggest that the pattern of cutaneous recurrence is prognostically significant and related to the site of the primary tumor given that the majority of local and regional recurrences occurred in primary tumors located on the lower limbs and head.

Granular Cell Tumor: A Clinical Study of 81 Patients. / Tumor de células granulares. Estudio clínico de 81 pacientes.

INTRODUCTION: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. OBJECTIVES: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. MATERIAL AND METHODS: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients' medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. RESULTS: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. CONCLUSIONS: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.

[Lupus pernio. A report of a series of 8 patients]. / Lupus pernio. Presentación de una serie de 8 pacientes.

OBJECTIVES: Although lupus pernio (LP) is the most characteristic cutaneous lesion of chronic sarcoidosis, only a few cases have been reported in our country. The aim of this study was to review the frequency and clinical characteristics of patients with LP in a large series of patients with sarcoidosis. METHODS: A retrospective review of the frequency and characteristics of patients diagnosed as having LP from the series of sarcoidosis of our institution for a period of 35 years was performed. RESULTS: Eight (1.6%) out of 507 patients with sarcoidosis were diagnosed of LP. Mean age was 42 years. In 6 patients, LP was the presentation form of sarcoidosis. Five patients had involvement of the nasal skin and one patient severe involvement of the nasal mucosa. All the patients were treated with antimalarial drugs, 4 with oral corticosteroids, 2 with laser therapy, or with combinations with other drugs. None of the patient having nasal skin involvement showed remission of LP. CONCLUSIONS: LP is a rare clinical form of sarcoidosis in our country. No treatment is effective for nasal skin involvement. The recent introduction of infliximab may represent an advance in the treatment of LP.