E. Cinotti

Scleromyxedema: A multicenter study of characteristics, comorbidities, course, and therapy in 30 patients

BACKGROUND Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series. OBJECTIVE We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course. METHODS We conducted a retrospective and prospective multicenter study. RESULTS We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency. LIMITATIONS This is mainly a retrospective study. CONCLUSIONS Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required.

Reflectance confocal microscopy of mucosal pigmented macules: a review of 56 cases including 10 macular melanomas

Although most mucosal pigmented macules are benign, it can be clinically challenging to rule out an early melanoma. Reflectance confocal microscopy (RCM) is a noninvasive imaging technique useful in discriminating between benign and malignant skin lesions.

Reflectance Confocal Microscopy for the Diagnosis of Vulvar Melanoma and Melanosis: Preliminary Results

BACKGROUND In the early stages, vulvar melanoma can mimic vulvar melanosis and therefore the diagnosis is often late and carries a poor prognosis. In vivo reflectance‐mode confocal microscopy (RCM) is an emerging technique that allows noninvasive high‐resolution imaging of the skin and mucosa, but it has not been employed in the study of genital pigmentation. OBJECTIVE To analyze the characteristics of vulvar melanosis and vulvar melanoma using RCM to define the confocal aspects that allow a correct differential diagnosis. METHODS AND MATERIALS Features of eight melanoses and two melanomas of the vulva were analyzed using RCM. RCM diagnosis was then compared with clinical and histologic diagnosis. RESULTS Two major characteristics are associated with vulvar melanosis: papillae rimmed by bright monomorphous cells and possible presence of a few dendritic bright cells in the basal layer of the epithelium. Two major features of vulvar melanoma have been identified: atypical cells in the epithelium and loss of normal architecture of chorion papillae. CONCLUSIONS Reflectance Confocal Microscopy can play a role in noninvasive differentiation between vulvar melanoma and vulvar melanosis, but further broader studies are needed to validate our observations.

In Vivo Reflectance Confocal Microscopy to Optimize the Spaghetti Technique for Defining Surgical Margins of Lentigo Maligna

BACKGROUND Lentigo maligna (LM) is a therapeutic challenge for surgeons because of its location in aesthetic areas and the difficultly in determining margins. OBJECTIVE To investigate a new procedure combining the “spaghetti” technique described by Gaudy‐Marqueste and colleagues in 2011 with in vivo reflectance confocal microscopy (RCM) to define the margins of LM more accurately and allow strict histologic control. METHODS and MATERIALS Thirty‐three consecutive patients with LM of the head underwent a RCM‐guided delineation of the margins followed by the “spaghetti” technique. RESULTS The excision of the first “spaghetti” in a tumor‐free area was obtained in 28 of 33 patients. In the other five cases, persistence of LM foci was found in <5% of the length of spaghetti. The average number of pieces of “spaghetti” was 1.2 (range 1–3). Definitive histologic examination of the lesion showed a minimum average margin of 2.7 mm. Follow‐up in 27 patients after an average of 10 months (range 4–25 months) did not show any recurrence. CONCLUSION This procedure allows accurate definition of the surgical margins of LM, with a low rate of multiple excisions, sparing tissue in functional and aesthetic areas. These results should be confirmed on the basis of a larger series with longer follow‐up.

Handheld Reflectance Confocal Microscopy for the Diagnosis of Conjunctival Tumors

PURPOSE To evaluate whether the handheld in vivo reflectance confocal microscopy that has been recently developed for the study of skin tumors is suitable for the diagnosis of conjunctival tumors. DESIGN Prospective study, observational case series. METHODS We prospectively evaluated the reflectance confocal microscopy features of 53 conjunctival lesions clinically suspicious for tumors of 46 patients referred to the University Hospital of Saint-Etienne (France) by using the handheld device. Twenty-three lesions were excised (3 nevi, 10 melanomas, 5 squamous cell carcinoma, 2 lymphomas, and 3 pinguecula/pterygium) while the other 30, presenting no reflectance confocal microscopy malignant features, were under follow-up for at least 1 year. Clinical reflectance confocal microscopy and histologic diagnosis were compared. RESULTS In vivo reflectance confocal microscopy diagnosis was in agreement with the histologic diagnosis in all cases and none of the lesions that were not excised show any clinical progression under follow-up. CONCLUSION In vivo reflectance confocal microscopy with a handheld dermatology-dedicated microscope can play a role in the noninvasive diagnosis of conjunctival lesions. Further studies should be performed to better define the diagnostic ability of this technique.

Laser photodynamic treatment for in situ squamous cell carcinoma of the glans monitored by reflectance confocal microscopy

While mutilating surgery can be avoided with non‐surgical treatment of in situ squamous cell carcinoma (SCC) of the penis, such as photodynamic therapy (PDT), this procedure is not followed by histological evaluation to verify the total removal of the lesion, leading to possible recurrence. We present the first case of in situ penile SCC treated with laser PDT, where the efficacy of the treatment was monitored by reflectance confocal microscopy (RCM) using a handheld camera. In the future RCM may be regarded as a complementary technique to assess the efficacy of non‐surgical treatment of mucous membrane cancers.

Reflectance confocal microscopy for quantification of Sarcoptes scabiei in Norwegian scabies

Background and Objectives  In vivo reflectance‐mode confocal microscopy (RCM) can be used for the diagnosis of scabies. This study quantifies S. scabiei and its eggs and droppings in a patient affected by Norwegian Scabies (NS), and describes their distribution within the epidermis and in different body areas.

Reflectance confocal microscopy for cutaneous infections and infestations

Reflectance confocal microscopy (RCM) is a high‐resolution emerging imaging technique that allows non‐invasive diagnosis of several cutaneous disorders. A systematic review of the literature on the use of RCM for the study of infections and infestations has been performed to evaluate the current use of this technique and its possible future applications in this field. RCM is particularly suitable for the identification of Sarcoptes scabies, Demodex folliculorum, Ixodes, Dermatophytes and Candida species in the clinical practice and for the follow‐up after treatment. The cytopathic effect of herpes simplex virus, varicella zoster virus and molluscipoxvirus is also detectable by this imaging technique even in a pre‐vesicular stage. In addition, thanks to its non‐invasiveness, RCM allows pathophysiological studies.

Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients.

The prognostic and therapeutic features of scleredema are poorly documented.

In vivo confocal microscopic substrate of grey colour in melanosis.

Melanosis is the most common cause of mucosal pigmentation and can be clinically difficult to differentiate from early melanoma (MM). Dermoscopy can help in the distinction between melanosis and MM, but in some instances, melanoses may exhibit overlapping features with MM such as the presence of grey colour.