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.

Comparative histological analysis of drug-induced maculopapular exanthema and DRESS.

BACKGROUND: Cutaneous adverse drug reactions frequently present as a benign maculopapular exanthema (MPE) with a rapid healing. Sometimes systemic signs are present, which could represent a more severe or systemic MPE (sMPE) or even be the initial phase of a drug reaction with eosinophilia and systemic symptoms (DRESS). Histopathology associated with MPE, sMPE and DRESS has not been well characterized. OBJECTIVES: To study the cutaneous histopathological changes associated with MPE, sMPE and DRESS. METHODS: A retrospective clinicopathological analysis of 13 cases of MPE, 13 of sMPE and 45 of DRESS, collected in one centre from 2005 to 2013. RESULTS: The number of histopathological changes per section increased gradually from MPE to sMPE and DRESS. Prevalence of spongiosis, dermal lymphocytes, eosinophils and neutrophils did not differ between MPE, sMPE and DRESS. Keratinocyte damage, rare in MPE, was regularly found in sMPE and frequent in DRESS. The density of the inflammatory infiltrate increased progressively from MPE to sMPE and DRESS. Atypical lymphocytes were absent in MPE, present in sMPE and more frequent in DRESS. Deep dermal involvement and leukocytoclastic vasculitis were only observed in DRESS. LIMITATIONS: This was a retrospective study. CONCLUSIONS: Numerous histopathological changes per section in drug-induced exanthema should alert for a more severe form of cutaneous adverse drug reactions, i.e. DRESS.

High prevalence of psychiatric disorders in patients with skin-restricted lupus: a case-control study.

BACKGROUND: Psychiatric disorders have been extensively documented in patients with systemic lupus erythematosus (SLE). However, the prevalence of psychiatric disorders in patients with skin-restricted lupus (SRL) remains unknown, although SRL is more common than SLE. OBJECTIVES: To assess current and lifetime prevalence of Axis I psychiatric disorders among outpatients with SRL and to examine the factors associated with psychiatric disorders among such patients. METHODS: A multicentre case-control study involving outpatients with SRL and controls matched for sex, age and education level. The Mini International Neuropsychiatric Interview was used for psychiatric evaluation. RESULTS: We evaluated 75 patients and 150 controls. Of these, 49% of patients vs. 13% of controls fulfilled the criteria for at least one current psychiatric disorder (P < 0·001). The following disorders were significantly more frequent among patients than controls: current and lifetime major depressive disorder (9% vs. 0%, P < 0·001 and 44% vs. 26%, P = 0·01), generalized anxiety disorder (23% vs. 3%, P < 0·001 and 35% vs. 19%, P = 0·03), panic disorder (7% vs. 0%, P = 0·004 and 21% vs. 3%, P < 0·001), current suicide risk (24% vs. 7%, P = 0·003), alcohol dependence (7% vs. 0%, P = 0·004) and lifetime agoraphobia (20% vs. 9%, P = 0·01). Lupus duration and lupus past treatment by thalidomide were significantly higher among patients with current psychiatric disorders. CONCLUSIONS: This study demonstrates a high prevalence of several psychiatric disorders (anxiety, depression, suicide risk, alcohol dependence) in patients with SRL.

Drug reaction with eosinophilia and systemic symptoms (DRESS): clinicopathological study of 45 cases.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and severe adverse drug reaction. Large detailed studies of histopathological features of DRESS are sparse and suggest an association between keratinocyte damage and the severity of visceral involvement. OBJECTIVES: To describe the dermatopathological features in a large series of DRESS and their possible association with clinical features and the severity of the disease. METHODS: A retrospective analysis of the clinicobiological and dermatopathological features in a monocentric cohort of patients with DRESS. RESULTS: From January 2005 to January 2013, 45 patients were validated as probable or definite cases of DRESS. The median age was 64 years (range 3-87). The most frequent clinical and biological features included: fever ≥38.5°C (95%), facial oedema (72%), enlarged lymph nodes (51%), visceral involvement (75%), blood eosinophilia (97%) and atypical lymphocytes (82%). Severe DRESS occurred in 24% and a fatal outcome in 6% of patients. Histopathological analysis showed that no specific histopathological pattern was characteristic for DRESS. However, several changes in different cutaneous compartments were observed in 2 of 3 of cases. Spongiosis (55%) and keratinocyte damage (53%) were the most common epidermal changes. Spongiosis was associated with non-severe DRESS (P = 0.041) whereas confluent keratinocyte necrosis correlated with severe DRESS (P = 0.011). Vascular changes were frequent (88%). A moderate dermal perivascular lymphocytic infiltrate was invariably present, containing eosinophils, neutrophils and/or atypical lymphocytes in 57% of cases. CONCLUSIONS: Epidermal changes are indicative for the severity of DRESS.

[Mid-dermal elastolysis after insertion of a pacemaker]. / Élastolyse du derme moyen après pose d'un stimulateur cardiaque.

BACKGROUND: Mid-dermal elastolysis (MDE) is a rare acquired disease of elastic tissue histologically characterized by focal loss of elastic fibres within the mid-dermis. While the mechanisms leading to MDE remain unknown, increased degradation of elastic fibres may be involved. Many factors potentially triggering such degradation have been suggested. PATIENTS AND METHODS: A 58-year-old man consulted for an asymptomatic reticulated eruption that began in the area of a pacemaker implanted six weeks earlier. The eruption consisted of erythematous polycyclic and coalescing macules with a wrinkled centre. Histopathology with orcein staining revealed focal loss of elastic fibres in the superficial reticular dermis only. Hypersensitivity reaction to any components of the pacemaker was ruled out by means of allergy exploration. Laboratory investigations including autoimmunological and haematological factors were unremarkable. A diagnosis was made of a reticular variant of MDE following insertion of a pacemaker. DISCUSSION: We report the second case of MDE following the insertion of a pacemaker, which could have triggered an inflammatory response directed specifically towards the elastic fibres.

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.

[Successful use of combined corticosteroids and rituximab in the treatment of recalcitrant epidermolysis bullosa acquisita]. / Succès de l'association rituximab-corticoïdes généraux dans le traitement d'une épidermolyse bulleuse acquise récalcitrante.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease; it is potentially serious and is often refractory to conventional treatments, including corticosteroids. We report a new case of successful treatment of EBA using rituximab (anti-CD20 antibody) without relapse after 1 year of follow-up. CASE REPORT: A 76-year-old man was seen for blisters of the skin and mucosa, atrophic scars and milia on areas of friction. The diagnosis of EBA was made on the basis of histological and immunohistochemical criteria. The patient was unsuccessfully treated with topical steroids, dapsone, topical tacrolimus, systemic steroids, mycophenolate mofetil, doxycycline and methotrexate. Four weekly infusions of rituximab of 375 mg/m(2) body area were performed, combined with systemic steroids: they proved beneficial within 3 weeks, with a noticeable improvement and no further blisters at 7 months. After 1 year of follow-up, the skin disease is still stable with 5 mg/day of prednisone alone being given. DISCUSSION: This is the eighth reported case of treatment of EBA with rituximab and the sixth successful therapeutic outcome, with good steroid sparing effect and undeniable improvement in quality of life within several months and good tolerability at 12 months of follow-up. This treatment may be proposed early in cases of EBA refractory to conventional treatments. However, clinical observation is necessary to study potential long-term adverse effects.

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.