Xeroderma pigmentosum skin: an immune privilege site for tumor development

A unique feature of the skin immune system is its proximity to cells continuously exposed to sun rays, as it is located in the interface between the body and the environment. In this study, we aimed to determine the impact of DNA damaged keratinocytes on the expression of apoptotic‐related molecules, in T‐cells of the inflammatory component of the tumor environment. Immunohistochemistry was performed on tissue sections derived from skin biopsies of basal cell carcinomas (BCCs) of xeroderma pigmentosum (XP) patients, non‐XP patients and nevoid basal cell carcinoma syndrome (NBCCS) patients, using antibodies against B‐cell lymphoma/leukemia‐2 (Bcl‐2), Bcl‐2 associated X protein (Bax), CD95, CD3, CD8 and CD56. Our results showed significantly lower levels of expression of the antiapoptotic Bcl‐2 molecule, in XP, in comparison with non‐XP and NBCCS T‐lymphocytes, leading to the highest Bax/Bcl‐2 ratio for XP T‐cells. For the CD95 receptor expression levels, there were significant differences among T‐cells of the three patient subgroups as well. The higher propensity of XP T‐cells to undergo apoptosis may have evolved in individual XP patients, apparently during the course of their disease, to maintain a special skin as an immune privilege site for tumors' development. Abid K, El Mezni F, Kamoun MR, Fazaa B, Zermani R, Hadouchi C, Hamzaoui K. Xeroderma pigmentosum skin: an immune privilege site for tumor development.     

Dermatoscopy: an auxiliary resource for the diagnosis of poroid neoplasms

Poroid neoplasms are benign epithelial proliferations with eccrine sweat gland differentiation. They are a challenging diagnosis because of the clinical heterogeneity, being able to mimic several malignant neoplasms. They are classified into classic poroma, hidroacanthoma simplex, dermal duct tumor and poroid hidradenoma. Association of histological subtypes occurs in more than 25% of cases. We report a case of a combined poroid neoplasia of classical poroma and poroid hidradenoma, reviewing its dermatoscopic features.     

Early development of multiple epithelial neoplasms in Netherton syndrome

We report a case of Netherton syndrome manifested as congenital ichthyosiform erythroderma, trichorrhexis invaginata and atopy, who in early adulthood developed multiple, aggressive epithelial neoplasms in sun-exposed areas of the skin, in areas with papillomatous skin hyperplasia and at the left parotid region. The occurrence of cutaneous neoplasia has been reported in syndromes with congenital ichthyosis and suggests that the underlying genetic defects may cause the development of cancer in prone patients.     

Lymphedema after sentinel lymph node biopsy for cutaneous melanoma: a report of 5 cases

BACKGROUND: Sentinel lymph node (SLN) biopsy has rapidly become the procedure of choice for assessing the lymph node status of patients with 1992 American Joint Committee on Cancer stages I and II melanoma. The procedure was designed to be less invasive and, therefore, less likely to cause complications than a complete lymph node dissection. To our knowledge, this is the first report in the literature documenting extremity lymphedema following SLN biopsy. OBSERVATION: We report 5 cases of lymphedema after SLN biopsy in patients being routinely followed up after melanoma surgery at the Massachusetts General Hospital Melanoma Center, Boston. Three cases were mild, and 2 were moderate. Potential contributing causes of lymphedema were present in 4 patients and included the transient formation of hematomas and seromas, obesity, the possibility of occult metastatic melanoma, and the proximal extremity location of the primary melanoma excision. Four of the patients underwent an SLN biopsy at our institution. We used the total number of SLN procedures (N = 235) that we have performed to calculate a 1.7% baseline incidence of lymphedema after SLN biopsy. CONCLUSIONS: Sentinel lymph node biopsy can be complicated by mild and moderate degrees of lymphedema, with an incidence of at least 1.7%. Some patients may have contributing causes for lymphedema other than the SLN biopsy, but many of these causes are difficult to modify or avoid.     

Lymphedema of the limb: predictors of efficacy of combined physical therapy

BACKGROUND: Limb lymphedema results from incompetence of the lymphatic system, and treatment of both primary and secondary forms involves manual lymphatic drainage and support. The effectiveness of treatment varies from patient to patient and can be unpredictable.OBJECTIVE: To investigate clinical and paraclinical criteria able to predict responses to combined physical treatment (lymphatic drainage and multilayered support) during hospitalization.PATIENTS AND METHODS: A retrospective study was performed in 45 patients admitted for one week's intensive treatment of limb lymphedema in the Lymphology Unit of the Department of Dermatology of the University Hospital of Tours. Lymphoscintigraphy was performed for all patients on admission. Loss of volume in affected limbs was studied to evaluate the effectiveness of treatment.RESULTS: Two criteria were predictive of resistance to combined treatment, i.e. venous insufficiency and continuing lymph node evidence of scintigraphic activity four hours after lymphoscintigraphy. The two factors were significantly related.CONCLUSION: Failure of combined physical treatment for lymphedema appears to be related to venous insufficiency. It is therefore essential to investigate and treat venous insufficiency, particularly in patients with continuing evidence of scintigraphic activity in lymph nodes.     

Condylomata lata of the palms: an unusual site.

In an unusual case of condylomata lata of the palms the development of moist hypertrophic papular eruptions is probably explained by the fact that the patient was a car-driver and had hyperhidrosis of the palms.     

Lymphedema of male external genitalia: a retrospective study of 33 cases

INTRODUCTION: The aim of this retrospective study was to describe the main characteristics and treatment of male external genitalia lymphedema. PATIENTS AND METHODS: From 1987 to 2003, all patients seen in a single hospital for lymphedema of male external genitalia were included. For each patient, the following characteristics were recorded: primary or secondary lymphedema, cause of secondary form, date of onset of lymphedema, associated lower limb lymphedema, clinical signs, and complications. In the primary forms, lower limb lymphoscintigraphy was performed. Specific surgery was proposed in all cases of symptomatic lymphedema (circumcision, scrotum and/or penile cutaneous excision). RESULTS: Thirty-three patients with lymphedema of external genitalia (17 primary, 16 secondary) were recruited. Two primary lymphedema were congenital, one isolated. Mean age +/- SD of the onset of the 15 other primary genital lymphedema was 23.4 +/- 17.5 years, always after the appearance of lower limb lymphedema. Sixteen men had secondary lymphedema (bladder, prostate, or rectum cancer, Hodgkin or non-Hodgkin lymphoma, aorto-bifemoral bypass grafting, biopsy or curretage of inguinal nodes). Secondary genitalia lymphedema was not associated with lower limb lymphedema in two cases and, in the others it occurred 66 +/- 122 months after (n=11), at the same time (n=2) or before lower limb lymphedema (n=1). Clinically, we noted genitalia heaviness (n=31), lower limb lymphedema (n=30), vaginal hydrocele (n=13), impaired miction due to prepucial swelling (n=10), leakage of lymphatic fluid (n=10). Lower limb lymphedema was complicated by at least one erysipelas (n=20), spreading to the external genitalia (n=4). In primary forms, lymphoscintigraphy showed ipsilateral hypoplasia of inguinal nodes in lower limb lymphedema (n=14) and/or external genitalia backflow (n=7). Surgical treatment was performed in 17 cases (11 primary, 6 secondary) with good results after 21 months' median follow up (1 month-10 years). Two patients died of cancer. One secondary lymphedema improved spontaneously and one disappeared after withdrawal of lower limb pneumatic compression. DISCUSSION: Lymphedema of external genitalia is responsible for discomfort and local complications. Surgical treatment is the main procedure of this disorder.     

Palmar erythema: cutaneous marker of neoplasms

BACKGROUND: Acral erythema on the palms is observed in several conditions. However, the relationship with malignant tumors has only been reported exceptionally. It should be noted that tumors produce angiogenic mediators. OBJECTIVE: These mediators might promote palmar erythema (PE), and the aim of the present study was to investigate the vasodilation of palmar skin capillaries and angiogenesis, mainly with tumors of the central nervous system. METHODS: In a prospective study of 107 patients affected by brain tumors, we assessed PE clinically and the rate of dilated vessels histologically. We also evaluated the mean surface of the lumen of capillaries on skin biopsies and brain tumors. RESULTS: 6.5% of the patients had an important erythema and 18.5% had slight and/or localized PE. In the skin biopsies, the rate of dilated vessels and the mean surface of the lumen of capillaries were higher than in normal skin. Moreover, the intensity of palmar redness was related to the increase in these vascular changes in the histopathological slices of brain tumors. The intensity also depended on the type of tumor and on its growth. CONCLUSION: The results of the present study strongly suggest that acral erythema is associated with malignant tumors and that the intensity of erythema and the vascular changes of brain tumors are related, probably due to angiogenic factors.