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Background The incidence of xeroderma pigmentosum (XP) in Yemen seems to be quite high but there are no previous reports. Objective To study the clinicoepidemiologic aspect of XP in Yemen. Methods All 40 patients (24 male and 16 female patients from 32 families) treated and followed between 1997 and 2014 were subjected to detailed analysis with the help of a standardized protocol. The diagnosis was based on clinical features and histopathologic data, when needed. The diagnosis of tumors was confirmed by histopathologic examination in all cases. Results The median age of onset of initial manifestations was 9.5 months, and that of malignant lesions was 7 years. Parents of the patients were not affected, but history of consanguinity was recorded in 38. Initial lesions, such as dryness of the skin and freckles on the face, were noticed in all patients. In addition, erythema of the face with photosensitivity was observed in 21 patients. Premalignant and malignant skin lesions observed later were actinic keratosis in 15 patients, lentigo maligna in one, squamous cell carcinoma (SCC) in 10, and basosquamous carcinoma in one. Eyes were affected with SCC in seven and malignant melanoma (MM) in two patients. SCC of the lip developed in two patients and that of the tongue in one patient. Judicious use of acitretin in 12 patients showed good result. Conclusion XP in Yemen is characterized by a relatively high incidence, high percentage of consanguinity in parents of the patients, early onset of initial manifestations, malignant tumors, and severe ocular and oral lesions. Acitretin showed good result.

Background Recent epidemiological studies suggest that past data where superficial spreading melanoma was by far the most common subtype of melanoma may not reflect current patterns of sun exposure or other risk factors more involved in other subtypes of melanoma as lentigo maligna (LM) or lentigo maligna melanoma (LMM). Methods In order to measure the current situation in our country, all cases of LM and LMM diagnosed in 23 hospitals in Catalonia, from 2000 to 2007, were recorded. Results Although for the global period LM/LMM represented only 8.4% of cases, an increasing trend in this percentage was observed throughout the study period (from 6.9% [27 cases] in 2000 to 13.1% [94 cases] in 2007). Also, an increasing incidence of LM/LMM was observed, especially in chronically sun-exposed areas (85.5% involving the head and neck region). During the 8 years of the registry, the mean Breslow thickness of LMM remained stable. However, the increase in the number of LM (in situ) cases was significantly higher than the increase of the invasive ones. Conclusions An important observation from this data is that aging of population and current sun exposure patterns could keep increasing the incidence of LM/LMM, which may become an important public healthcare problem, over the other histological subtypes. In order to establish primary or secondary preventive measures to the LM/LMM risk-population, it is imperative to highlight the importance of chronic sun damage as a melanoma risk factor, and not only sunburn, most commonly addressed in melanoma prevention campaigns.

Background Given the critical position of central glabella among the frontal, nasal, and supraorbital aesthetic subunits of the face, the reconstruction of large defects in this area represents a surgical challenge. Methods We describe a surgical technique based on a modified, curved, A-T flap to repair a large glabellar defect. Results Our modification is useful for large glabellar defects because it enables the distribution of the tension all over the reconstruction sides, avoiding a stressed central area and the subsequent risk of necrosis; functionally, it respects the eyebrows position and since the advancement is parallel to their major axes, it avoids the reduction of the distance between them. Conclusions The “Batman flap” enables reconstructing a glabellar defect, with a good aesthetical result and the respect of the relevant aesthetical subunits.

Background Melanoma is responsible for 75% of all skin cancer-related deaths. Lentigo maligna (LM), the most prevalent melanoma in situ (MIS), accounts for 79–83% of all MIS. Its location in the head and neck area requires that tissue be spared in the course of clearing the tumor. Methods We conducted a therapeutic study based on our practice experience with the so-called “collarette” skin biopsy technique used in the management of LM. Results Thirty-one patients were diagnosed with LM in the head and neck area. These included 16 women and 15 men with a mean ± standard deviation (SD) age of 64.4 ± 13.6 years (range: 33–88 years). The majority (36%) of lesions were located on the cheek. To clear the tumor, surgery was performed using margins of >10 mm in 71% of cases. The average surface area of the surgical defect was 5.8 cm2 (range: 1.1–15.5 cm2). Reconstructive skin grafts were performed in the majority (68%) of cases. After the removal of the central tumor, 16 cases of MIS and 15 cases of invasive melanoma were identified. Only one patient experienced recurrence over a mean ± SD follow-up of 31 ± 16 months. Conclusions The so-called “collarette” or “spaghetti” skin excision and biopsy technique is an advantageous and efficient way to achieve tumor clearance and represents an equally effective alternative to Mohs micrographic surgery in the treatment of LM in the head and neck area.

The treatment of melanoma in situ (MIS), particularly the lentigo maligna (LM) subtype, has been a controversial subject in the literature for over a decade. Surgical excision with 5 mm margins is the standard of care in the USA, while several variations of Mohs surgery are frequently used to treat clinically ill-defined lesions. Radiation is much less frequently used. Topical imiquimod has also been more recently proposed, in small case reports in the literature, as a therapy for MIS. However, controversies exist with all approaches.To review the current literature regarding topical imiquimod, radiation therapy, surgical excision, and the various forms of Mohs surgery for MIS, focusing on the LM subtype.A literature search was performed in the PubMed database using the following terms: "melanoma in situ,""lentigo maligna,""excisional surgery,""Mohs micrographic surgery,""radiation therapy," and "imiquimod." Articles relevant to the treatment of MIS were reviewed and reported herein.Studies of imiquimod therapy for MIS are hampered by small study numbers and short follow-up periods. The few, better-designed studies reveal relatively low cure rates. In addition, literature review reveals that a significant percentage of cases of MIS that are initially diagnosed as in situ disease by biopsy subsequently prove to have an invasive component upon complete excision. This finding suggests that topical therapy of any kind may be problematic. Studies of radiation therapy for MIS have relatively small numbers of patients and short follow-up. Multiple studies of excisional surgery have shown that 5 mm margins are often insufficient to clear the LM subtype of MIS due to unseen subclinical extension, accounting for this treatment's reported 8-20% recurrence rate. Finally, a number of variants of traditional frozen section Mohs surgery have been utilized to try and achieve complete peripheral margin assessment of clinically ill-defined LM lesions. All studies are retrospective, and most are single-institution and frequently single-operator, limiting the meaningfulness of their results. Nevertheless, they involve moderate numbers of patients, and many have at least a five year follow-up. Collectively, they suggest recurrence rates in the range of 0.5-3.0%.Topical imiquimod therapy appears to provide relatively low cure rates for MIS, and because some of these lesions contain an unrecognized invasive component, should be used with extreme caution to treat this disease. Radiation therapy may be a useful second-line therapy if surgery is contraindicated. Excisional surgery is an appropriate therapy for clinically well-defined MIS; however, margins larger than 5 mm may be required when treating larger or indistinct lesions. Finally, for clinically ill-defined LM arising on sun-damaged skin, especially in regions of aesthetic concern, some form of complete peripheral margin assessment - one of the various forms of Mohs surgery - may provide the highest cure rate and create the smallest surgical defect.

Background. Cutaneous malignant melanoma (CMM) is a recognized feature of the Lynch type II cancer-family syndrome and the Li-Fraumeni's syndrome. A significant contribution of these syndromes to the total burden of CMM would be reflected in an increased risk of nonmelanoma cancers in first degree relatives. Methods. Pedigrees were taken from 85 patients with CMM using a family history questionnaire. The relative risk of death from all cancers and individual cancers in first degree relatives was calculated. Results. Of the 85 questionnaires, those of 79 patients were completed and of adequate quality for analysis. The first degree relatives of CMM patients showed no increased risk of cancer death, the relative risk of cancer death being 1.0. Six patients (7.6%) had first degree relatives with CMM. One patient had a family history compatible with the dominant transmission of a predisposition to cancer. Conclusions. It is important to establish whether an increased cancer risk is present in relatives of patients with malignancies so that screening programs may be offered. This study provides little evidence to support seeing relatives for noncutaneous malignancies in the absence of a dominant family history of predisposition to cancers. The increased frequency of CMM in relatives suggests that relatives of CMM patients should be counseled on protection from the sun and examination of the skin for melanoma.