Understanding the anatomy of dermoscopy of melanocytic skin tumours: Correlation in vivo with line-field optical coherence tomography.

BACKGROUND: Early melanoma detection is the main factor affecting prognosis and survival. For that reason, non-invasive technologies have been developed to provide a more accurate diagnosis. Recently, line-field confocal optical coherence tomography (LC-OCT) was developed to provide an in vivo, imaging device, with deep penetration and cellular resolution in three dimensions. Combining the advantages of conventional OCT and reflectance confocal microscopy, this tool seems to be particularly suitable for melanocytic lesions. OBJECTIVES: The objective of this study was to identify and describe the correlation between specific dermoscopic criteria and LC-OCT features in three dimensions associated with melanocytic lesions. METHODS: Dermoscopic and LC-OCT images of 126 melanocytic lesions were acquired in three different centres. The following dermoscopic criteria have been considered: reticular pattern, dots and globules, structureless areas, blue-whitish veil, regression structures, negative network, homogeneous pattern, streaks and blotches. RESULTS: 69 (55%) benign and 57 (45%) malignant lesions were analysed. A regular reticular pattern was found associated in the 75% of the cases with the presence of elongated rete ridges with pigmented cells along the basal layer, while atypical reticular pattern showed an irregular organization of rete ridges with melanocytic hyperplasia, broadened and fused ridges and elongated nests. Both typical and atypical dots and globules were found associated with melanocytic nests in the dermis or at the dermoepidermal junction (DEJ), as well as with keratin cysts/pseudocysts. Grey globules corresponded to the presence of melanin-containing dermal inflammatory cells (melanophages) within the papillae. Structureless brown/black areas correlated with alterations of the DEJ. We observed the same DEJ alterations, but with the presence of dermal melanophages, in 36% of the cases of blue/white/grey structureless areas. A description of each LC-OCT/dermoscopy correlation was made. CONCLUSIONS: LC-OCT permitted for the first time to perform an in vivo, 3D correlation between dermoscopic criteria and pathological-like features of melanocytic lesions.

Lymphomatoid papulosis types D and E: a multicentre series of the French Cutaneous Lymphomas Study Group.

BACKGROUND: Lymphomatoid papulosis (LyP) type D (LyP D) and type E (LyP E) have recently been described in small series of cases or isolated case reports. AIM: To further describe the clinical and histological features of LyP D and E based on a retrospective multicentre study. METHODS: The clinical and histopathological features of 29 patients with an initial diagnosis of LyP D or LyP E were retrospectively assessed using standardized forms. RESULTS: After exclusion of 5 cases, 24 patients (14 LyP D, 10 LyP E) were enrolled in the study. The median follow-up was 2.5 years (range 1 month to 13 years). LyP D was characterized by multiple recurrent self-regressing small papules that developed central erosion or necrosis, whereas LyP E presented as papulonodular lesions that rapidly evolved into necrotic eschar-like lesions > 10 mm in size. Epidermal changes were more frequent in LyP D, whereas dermal infiltrates were deeper in LyP E. Anaplastic cytology was rare and the DUSP22 rearrangement was never observed. Two patients (8%) had an associated cutaneous lymphoma. CONCLUSION: LyP D and E have distinct clinical findings and may be associated with other cutaneous lymphomas.

[Zosteriform cutaneous metastasis in melanoma]. / Mélanome avec métastases cutanées zostériformes.

INTRODUCTION: Cutaneous metastases are common in patients with malignant melanoma. In rare cases, they are distributed on a dermatome, in which case they are known as zosteriform metastases. OBSERVATION: We report the case of a patient with zosteriform metastasis of a malignant melanoma, progressing unfavourably despite surgical excision and immunotherapy. DISCUSSION: The physiopathology of this condition continues to be poorly understood.

Role of obesity on the thickness of primary cutaneous melanoma.

BACKGROUND: Thick primary cutaneous melanoma (PCM) is associated with older age, male sex, being single, a low educational level, self-detection and general practice detection, nodular melanoma (NM) and acral lentiginous melanoma (ALM) types; and are found in the head-neck and lower limb locations. Obesity plays a direct role on melanoma tumour growth, as it has been shown in animal models, but its role in the thickness of PCM remains unknown. OBJECTIVES: We investigated the impact of obesity on the thickness of invasive PCM. METHODS: A cross-sectional study was performed in a prospective cohort for which we collected several clinical and histological data already known to be associated with thick PCM and the Body Mass Index from new cases of invasive PCM which were referred to the dermatology department in Valence. RESULTS: Four hundred and twenty-seven patients were studied. In an univariate analysis, thick PCM was associated with low educational level, obesity, identification by the patient or the general practitioner (GP), location on the cephalic extremity, in a non-visible area of the body, the NM and ALM type, and an ulceration. In a multivariate analysis, NM, ulceration, topography of the melanoma and identification of the melanoma by the patient or GP were significantly associated with thick melanoma. When including only clinical features in the model, low educational level, mode of melanoma identification and obesity were significantly associated with a risk of thick melanoma. CONCLUSIONS: Obesity is a clinical independent risk factor of thick PCM. For health policies, governments should pay greater attention to detect melanoma in obese patients. Our results encourage the basic research on tumoural growth mechanisms due to obesity in melanoma.

[Primary eccrine porocarcinoma: a clinicopathological study of 50 cases]. / Étude clinique et histopronostique de 50 cas de porocarcinome eccrine.

BACKGROUND: Eccrine porocarcinoma is a rare cutaneous tumor arising from the intra-epidermal portion of eccrine sweat glands, the acrosyringium. Histoprognostic studies in large series are rare. Herein, we report a retrospective study of 50 cases. PATIENTS AND METHODS: Fifty cases of porocarcinoma were retrieved from a histopathological register. Each histopathological sample was evaluated for the following criteria: presence or absence of dermal invasion, pattern of the infiltrative component (pushing or infiltrative), tumor thickness, lymphovascular emboli, perineural invasion and mitotic index. Clinical data and outcome were also retrieved for each patient. RESULTS: Mean patient age was 77 years (range: 43-99 years). The mean duration of progression prior to diagnosis was 4 years and 5 months. The 2 most common skin locations were the head (38%) and lower limbs (20%). The lesions showed no specific distinctive clinical features. Six cases were in situ, and 44 were invasive (23 with limited infiltration and 20 with scattered infiltration). Mean tumor thickness was 4.37 mm (range: 0.5 to 20 mm). Neighboring or remote epidermal involvement was noted in 7 cases. Lymphovascular emboli were observed in 3 cases. No cases of neurotropism were observed. The average mitotic index was 6.5 mitoses/high power (×400) field. A mean follow-up of 24.3 months was available for 48 patients. Local recurrence was noted in 5 patients, 4 of whom died from visceral metastases. These 5 cases showed no distinctive clinical features, a scattered pattern of the invasive component, cuticular cells, significant tumor thickness (mean 12.8 mm, range 9-20 mm), and an elevated mitotic index. Two histopathological criteria were significantly associated with a metastatic outcome: scattered pattern of the dermal invasive component (P=0.04) and significant tumor thickness, above 10mm (P<0.01). CONCLUSION: Porocarcinoma is a tumor without any particular clinical criteria to distinguish it from squamous cell carcinoma. The architecture of the invasive component and tumor thickness constitute 2 important histoprognostic criteria.

Prognostic factors associated with healing of venous leg ulcers: a multicentre, prospective, cohort study.

BACKGROUND: Some prognostic markers of venous leg ulcer (VLU) healing have been evaluated, mostly in retrospective studies. OBJECTIVES: To identify which clinical characteristics, among those known as possible prognostic factors of VLU healing, and which VLU-associated sociodemographic and psychological factors, are associated with complete healing at week 24 (W24). METHODS: A prospective, multicentre, cohort study was conducted in 22 French dermatology departments between September 2003 and December 2007. The end point was comparison between healed and nonhealed VLUs at W24, for patient clinical and biological characteristics; psychological, cognitive and social assessments; affected leg inclusion characteristics; venous insufficiency treatment and percentage of initial wound area reduction during follow-up. RESULTS: In total, 104 VLUs in 104 patients were included; 94 were analysed. The mean VLU area and duration were 36.8 ± 55.5 cm2 and 24.8 ± 45.7 months, respectively. At W24, 41/94 VLUs were healed. Univariate analysis significantly associated complete healing with superficial venous surgery (P = 0.001), adherence to compression therapy at W4 (P = 0.03) and W24 (P = 0.01), ankle-joint ankylosis (P = 0.01) and mean percentage of VLU area reduction at W4 (P = 0.04). Multivariate analysis retained superficial venous surgery during follow-up [odds ratio (OR) 8.4, 95% confidence interval (CI) 1.9-48.2] and percentage reduction of the VLU area at W4 (OR 1.6, 95% CI 1.0-2.14) as being independently associated with healing. CONCLUSIONS: These results indicate that complete healing of long-standing, large VLUs is independently associated with ablation of the incompetent superficial vein and percentage of wound area reduction after the first 4 weeks of treatment.

Atypical granular cell tumor occurring in an individual with Noonan syndrome treated with growth hormone.

We report a large infiltrating atypical granular cell tumor in a child with Noonan syndrome. Even though granular cell tumors are rare in childhood, five cases have been reported in children with Noonan syndrome. This study compares these different cases and explores the possibility of activation of the granular cell by the Ras pathway.

[Topical imiquimod for the treatment of anogenital warts in an infant]. / Condylomes acuminés du nourrisson traités par imiquimod local.

BACKGROUND: Treatment of anogenital warts (AW) in children and infants is painful because of the destructive techniques involved, as a result of which general anaesthesia may be required. For adults, topical imiquimod is an efficient and well-tolerated product used for these lesions. Here, we report a case demonstrating the benefits of topical imiquimod in the treatment of AW in infant. PATIENTS AND METHODS: A ten-month-old infant was presenting large AW in the inguinal and perianal folds for a period of two months, which persisted despite one month of treatment with podophyllotoxin. After a month of daily application of imiquimod, the AW was completely cured, with no recurrence at six months. DISCUSSION: In the literature, we found four similar case reports concerning the efficacy and safety of topical imiquimod for AW in infants. Although topical imiquimod is not licensed for paediatric use, these case reports highlight the benefits of this approach in infants.

Morphoea-like plaques revealing an eosinophilic (Shulman) fasciitis.

Eosinophilic fasciitis (EF) is a connective-tissue disease characterized by thickened fascia. Involvement of the dermis may occur during the progression of the disease, characterized clinically by morphoea-like plaques (MLPs). This more superficial feature of EF carries a poor prognosis, suggestive of refractory EF that requires intensive therapy. We report a case in which morphoea-like plaques occurred before the EF. This case and previous cases of MLPs show some differences between MLPs and classic morphoea. Clinically, MLPs present with ill-defined brown plaques, with no lilac ring or ivory colour. Histologically, there is a more marked inflammatory infiltrate and an increase in the number of eosinophils. The discovery of MLPs at an early stage should prompt examination of the fascia with magnetic resonance imaging or surgical biopsy, and aggressive treatment.

Large plaque-type blue naevus with subcutaneous cellular nodules.

We report a new case of 'large plaque-type blue naevus (PTBN) with subcutaneous cellular nodules' involving the breast. A 25-year-old-woman presented with a large plaque, 140 x 100 mm in size, on the right chest wall involving the right breast, associated with recent subcutaneous nodules. Histopathological examination revealed features of cellular and common blue naevus. Dermatoscopy was performed, and the diagnosis of PTBN was made. PTBN is a rare condition with an imprecise prognosis. In cases of PBTN lesions on the head, clinicians must keep in mind the risk of malignant transformation of PTBN or an association with cutaneous neurocristic hamartoma. For this new variant of PTBN appearing on the trunk, we propose close monitoring of the patient if surgical excision is impossible.

[Diffuse systemic sclerosis after occupational exposure to trichloroethylene and perchloroethylene]. / Sclérodermie systémique diffuse liée à une exposition professionnelle au trichloroéthylène et perchloréthylène.

BACKGROUND: Diffuse systemic sclerosis (DSS) is an autoimmune disease that is most often endogenous but which can also be induced by exogenous substances of occupational origin. PATIENTS AND METHODS: We report a case of DSS involving prolonged intermittent occupational exposure to solvents (trichloroethylene [TCE] and perchloroethylene [PCE]). The disease was rapidly fatal with cardiac arrest secondary to myocardial fibrosis. DISCUSSION: In the event of exposure to TCE/PCE, we suggest more systematic prevention and diagnosis of DSS.

Transformed mycosis fungoides: clinicopathological features and outcome.

BACKGROUND: Transformation of mycosis fungoides (T-MF) occurs in 8-55% of MF patients. Its early histopathological diagnosis is of tremendous importance to better define the management and to establish the prognosis. Recent studies have demonstrated that advanced-stage MF at diagnosis of transformation is the predominant risk factor of poor outcome. The 5-year survival rates for stage IIB and IV MF are 26.9% and 10.6%, respectively. The prognostic value of the immunophenotypic characterization of the infiltrate has not been thoroughly studied in the literature. OBJECTIVES: To retrieve clinical, histological and immunophenotypic features of T-MF in our patient population and analyse their prognostic value. PATIENTS AND METHODS: A register-based retrospective study was performed including all patients with cutaneous T-cell lymphoma (CTCL) registered in our two departments from January 2000 to December 2005. Among 208 patients with CTCL, 17 patients with proven transformation of their MF were studied. Clinical features and staging as well as immunophenotypic and pathological findings at the time of the initial diagnosis of MF and of the diagnosis of T-MF were analysed. RESULTS: Our results, in accordance with previously published material, indicate that the main clinical prognostic factor in T-MF is the stage of the initial disease at the time of the transformation. Patients with stage IIB-IV MF have a poor prognosis. In our study, strong expression of CD30 is linked to a better prognosis. CONCLUSIONS: We believe that pathological and immunopathological documentation of progressive MF is important in order to identify T-MF early; however, the differential diagnosis is sometimes difficult. Aside from already acknowledged prognostic factors such as older age, advanced initial disease and short delay to transformation, the CD30 immunophenotype could be regarded as a useful additional prognostic marker in T-MF.

[Pachydermatodactyly associated with plantar pachydermy]. / Pachydermodactylie associée à une pachydermie plantaire.

INTRODUCTION: Pachydermodactyly is a superficial fibromatosis located on the proximal portion of fingers'phalanges and interphalangeal joints. Several types of this disease have been described depending on topography, etiology and pathological associations. We report a typical observation of pachydermodactyly associated with a plantar pachydermy. CASE-REPORT: A 19 year-old man was followed for a psychotic disease, associated with mental retardation. On clinical examination, he showed a typical pachydermodactyly, predominantly located on the second, third and fourth fingers of both hands, associated with recent acrocyanosis. A pachydermic aspect was also observed on the external part of the feet sole. Histopathological analysis was identical on digital and plantar lesions with collagen swelling. These lesions extended into the subcutaneous fat only in the feet. DISCUSSION: This observation is compatible with the most classical form of pachydermodactyly which involves several fingers of both hands, usually in men. Furthermore, the association with a psychiatric disorder and the notion of repeated traumatisms have been frequently reported in the literature. However, acrocyanosis and the important disability observed in our case are unusual. Association with plantar pachydermy has never been reported. The identical histological aspect on finger and feet sole suggests that pachydermatodactyly should be integrated in framework of acral pachydermy.

[Photo-induced sarcoidosis]. / Sarcoïdose photodéclenchée.

INTRODUCTION: Among the multiple forms of clinically atypical cutaneous sarcoidosis, lesions limited to light-exposed areas are rare. We describe a fourth observation. CASE REPORT: A phototype V 51-year-old woman, born in Tunisia presented with papular erythema on the face. Treatment with topical steroids did not result in any improvement. Biopsy specimen revealed a non caseating granuloma according with sarcoidosis. Assessment for systemic sarcoidosis was negative. Phototesting showed no abnormalities. The patient was successfully treated with chloroquine. DISCUSSION: Only three similar observations are reported in the literature. The role of light exposure despite the negative phototesting seems to be real. The tallying of these four observations permits one to isolate photo-induced sarcoidosis as a single entity.