[Prurigo pigmentosa during pregnancy]. / Prurigo pigmentosa au cours de la grossesse.

INTRODUCTION: Prurigo pigmentosa is a rare inflammatory dermatosis characterized by pruritic and reticulate papules on the trunk leaving hyperpigmentation. This dermatosis has been rarely described outside Asia. The pathophysiology remains obscure. CASE REPORT: We report the case of a 21-year-old North-African woman presenting with a highly pruritic eruption with numerous erythematous macules and papules coalescing in a reticular pattern on the trunk. The eruption occurred during the first trimester of pregnancy, which was marked by severe vomiting resulting in weight loss of 13kg with ketonemia and ketonuria. Taking into account the characteristic pattern of the eruption, the absence of differential diagnosis, and the histological examination, we concluded on a diagnosis of prurigo pigmentosa. Progression of the disease exhibited phases of decreased inflammation and of pruritus alternating with episodes of inflammatory flares triggered by relapses of ketonemia. The symptoms finally resolved during the 2nd trimester of pregnancy after vomiting ceased. Secondary reticulated hyperpigmentation was observed. CONCLUSION: Although rare, the highly evocative clinical presentation of the eruption should help clinicians in diagnosing prurigo pigmentosa. While pathophysiology remains undetermined, prurigo pigmentosa was reported in cases of intense fasting, anorexia, type-1 diabetes, and in two other cases of pregnancy with severe vomiting. Our case underlines the need to screen for and treat underlying ketonemia to achieve control of the inflammatory flares of prurigo pigmentosa.

Epstein-Barr Virus-Infected Plasma Cells Infiltrate Erosive Oral Lichen Planus.

Epstein-Barr virus (EBV), in addition to its transforming properties, contributes to the pathogenesis of several inflammatory diseases. Here, we investigated its involvement in oral lichen planus (OLP), a common autoimmune-like disease of unknown etiopathogenesis that can display a malignant potential. EBV-infected cells (EBV+ cells) were sought in a large series of clinically representative OLPs ( n = 99) through in situ hybridization to detect small noncoding EBV-encoded RNAs. Overall, our results demonstrated that EBV was commonly found in OLP (74%), with significantly higher frequency (83%) in the erosive form than in the reticular/keratinized type mild form (58%). Strikingly, many erosive OLPs were massively infiltrated by large numbers of EBV+ cells, which could represent a large part of the inflammatory infiltrate. Moreover, the number of EBV+ cells in each OLP section significantly correlated with local inflammatory parameters (OLP activity, infiltrate depth, infiltrate density), suggesting a direct relationship between EBV infection and inflammatory status. Finally, we characterized the nature of the infiltrated EBV+ cells by performing detailed immunohistochemistry profiles ( n = 21). Surprisingly, nearly all EBV+ cells detected in OLP lesions were CD138+ plasma cells (PCs) and more rarely CD20+ B cells. The presence of EBV+ PCs in erosive OLP was associated with profound changes in cytokine expression profile; notably, the expression of key inflammatory factors, such as IL1-ß and IL8, were specifically increased in OLP heavily infiltrated with EBV+ PCs. Moreover, electron microscopy-based experiments showed that EBV+ PCs actively produced EBV viral particles, suggesting possible amplification of EBV infection within the lesion. Our study thus brings conclusive evidence showing that OLP is commonly infiltrated with EBV+ PCs, adding a further puzzling element to OLP pathogenesis, given that PCs are now considered to be major regulatory immune cells involved in several autoimmune diseases (ClinicalTrials.gov NCT02276573).

Papillomavirus genotyping on formaldehyde fixed paraffin-embedded tissues in vulvar intraepithelial neoplasia.

PURPOSE: Few studies have described the epidemiology of human papillomavirus (HPV) in vulvar intraepithelial neoplasia (VIN). The aim of this study was to genotype HPV on formalin fixed paraffin-embedded tissues in VIN lesions. METHODS: A 5-year retrospective study was conducted by including all patients attending the teaching hospital of Nice with a diagnosis of VIN between 1st January 2010 and 31st December 2014. For all patients, HPV genotyping was performed with the PapilloCheck® microarray kit, routinely used on cervical cytology samples, and optimized for formaldehyde fixed paraffin-embedded tissues in VIN. RESULTS: Forty patients were included in the study: 39 patients had usual VIN and one presented with differentiated VIN. Among the 39 patients with usual VIN, the prevalence of HPV was 90% (35/39). Thirty-two patients had high grade VIN (82%) and seven low grade VIN (18%). In high grade VIN, the most represented HPV types were: HPV 16 (21/32 66%), HPV 56 (3/32 9%) and HPV 33 (2/32 6%). In low grade VIN, the most represented HPV types were: HPV 16 (4/7 57%) and HPV 6 (3/7 43%). Interestingly, 5/39 (13%) of patients diagnosed with usual VIN also had co-existing lichen sclerosus. CONCLUSIONS: We have optimized a HPV genotyping technique, routinely used on cervical cytology samples, and on paraffin fixed embedded tissue showing VIN. Moreover, we have identified five patients with lichen sclerosus co-existing with usual VIN. This association has rarely been reported and proves that these two entities can coexist.

Glomus tumors of the upper limb: Single-center retrospective study of clinical and functional outcomes.

Glomus tumors are rare and benign hamartomas, arising from neuro-myo-arterial proliferation and deriving from mesenchymal origin. As they have a long-term impact on the individual's quality of life, the primary complaint is unbearable pain. The aim of this study was to assess the clinical and functional outcomes of their surgical treatment, and to review their clinical, radiological and therapeutic features. We performed a retrospective study over a 16-year period including 31 patients with an upper limb glomus tumor. Epidemiologic, diagnostic, therapeutic and follow-up data were collected and a functional outcome questionnaire was filled out postoperatively. Thirty-one patients underwent surgery with safe macroscopic resection margins. The glomus tumor was located on the fingers in 77.4% of cases, with predominance in the ring finger (41.9% of the cases). Patient age at surgery ranged from 22 to 80 years old (mean: 54.6) with a sex ratio of 0.48. Upon clinical suspicion, magnetic resonance imaging and ultrasound were done in most cases. Immediate pain relief was obtained in 18 cases. Only one patient underwent a second surgery for incomplete removal and persistent pain. The QuickDASH questionnaire was completed by 24 patients, resulting in a mean score of 1.61, with a mean follow-up time of 88.8 months (range: 3-171 months). Seven patients were lost to follow-up. These subcutaneous, mostly subungual, nodules, with predominance on the ring finger, have a disproportionate negative impact despite their small size. The long-term outcomes after microscope-assisted surgery indicate obvious improvement in the quality of life and the patient's satisfaction, with a very low rate of recurrence.

Hypochromic vitiligo: delineation of a new entity.

BACKGROUND: Hypochromic vitiligo is a rare entity that has been reported only twice under the term 'vitiligo minor', with an absence of clear delineation. OBJECTIVES: To delineate hypochromic vitiligo through a case series of patients with typical bilateral hypopigmented lesions affecting the face and trunk. METHODS: This is a retrospective multicentric evaluation study conducted in eight departments of dermatology in France, Belgium, Senegal and Saudi Arabia. RESULTS: Twenty-four cases of hypochromic vitiligo were identified. Fourteen were men and 10 women. The mean age at diagnosis was 35·4 years (range 8-66). Strikingly, all patients were dark skinned, with skin types V and VI. The pattern of distribution was highly similar in most of the patients (18 of 24), with involvement of the face and neck area predominating on seborrhoeic areas associated with multiple isolated hypopigmented macules involving predominantly the scalp. The retrospective nature of this study is its main limitation. CONCLUSIONS: Hypochromic vitiligo is not yet part of a conventional classification. The disease seems to be limited to individuals with dark skin types. Hypopigmented seborrhoeic face and neck involvement associated with hypopigmented macules of the trunk and scalp is the hallmark of the disease.

[Atypical histiocytoma in a child]. / Histiocytofibrome atypique chez une enfant.

BACKGROUND: Histiocytoma (HC) is a very common benign tumour generally seen in the lower limbs of adults, particularly women. There are, however, atypical forms of HC that behave like locally aggressive tumours, occasionally with relapse or even metastasis. Herein we report a case of locally aggressive HC in a child, which, on account of its clinical extension, required seven surgical procedures to achieve complete excision. PATIENTS AND METHODS: A 13-year-old child consulted for a hard purplish papule measuring 8 mm in diameter located in the right lumbar region. Punch biopsy revealed a poorly delineated dermal-hypodermic tumour comprising randomly distributed moderately pleomorphic fusiform cells, arranged in bands or with storiform architecture, certain of which were multi-nucleated. The mitotic index was high (11 mitoses in 10 fields at high magnification). There was no expression by the tumour of melanocytic markers (PS100, Melan-A), histiocytic markers (CD68) or CD34. FISH analysis showed the absence of COL1A1-PDGFB fusion gene. Based on these immunohistochemical and molecular findings, a diagnosis was made of atypical HC with high cellular density. Since the lower margins of the section showed tumoural foci, surgical excision was performed with 5-mm margins. Because the lateral and vertical limits were reached in all cases, a series of five further procedures (the last of was preceded by multiple peripheral biopsies) was necessary to achieve complete excision. These multiple excision procedures resulted in total excision of 25 cm across the longest side. No clinical relapse was seen after 25 months. DISCUSSION: Cellular or atypical forms of HC carry a high likelihood of post-surgical relapse. They are characterised by marked pleomorphism and high cellular density. In our patient, the extent of the lesion had been greatly underestimated initially, resulting in the need for several surgical procedures in order to achieve complete excision. It is thus important to highlight the predictive factors for this type of tumour in order to enable sufficiently extensive excision, or excision guided by previous biopsies, to be contemplated from the outset. These predictive factors are: young patient age, unusual location (trunk, face, neck), high cellularity, marked mitotic activity and deep extension.

[Localized eruptive juvenile xanthogranuloma]. / Xanthogranulome juvénile éruptif localisé

BACKGROUND: Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytosis of young children characterized by solitary or multiple yellowish cutaneous nodules. Atypical skin lesions such as lichenoid eruptions, and pedunculated, maculopapular, plaque-like or linear lesions have been described. We report a case of eruptive XGJ en plaque in the left leg in an infant. PATIENTS AND METHODS: A 13-month-old child presented asymptomatic eruptive, yellowish papules of the leg measuring 5 to 10mm since the age of 2months. There was no cutaneous infiltration between the lesions. Darier's sign was negative. Histological examination confirmed the diagnosis of JXG. The course of the disease comprised a gradual decrease in the number of active lesions with slight residual pigmentation. DISCUSSION: Our case was suggestive of JXG en plaque. Only 7 cases have been reported in the literature, all appearing before the age of 5months. The lesions corresponded mostly to an asymptomatic erythematous plaque studded with small yellowish/red nodules of variable localisation. Spontaneous involvement was noted in all cases. No systemic involvement was found. Herein we present a unique case of localised multiple JXG without evident clinical infiltrating plaque progressing with self-resolving flares.

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

BACKGROUND: 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. OBJECTIVES: To describe the confocal aspects of benign and malignant mucosal pigmented macules with histopathological correlations. METHODS: We retrospectively reviewed the confocal images of 56 labial or genital pigmented macules including 10 macular melanomas. According to the retrospective nature of the study, we evaluated the recorded images chosen by the physicians that performed the RCM examination for each case. RESULTS: In benign macules, the most frequently observed pattern was a ringed pattern characterized by round or polycyclic papillae, with a hyper-reflective basal layer; another pattern was characterized by sparse bright dendritic cells in the basal layer, the basal epithelial cells being otherwise less reflective. Roundish cells, a high density of dendritic cells with atypias and intraepithelial bright cells were clues to the presence of malignancy. CONCLUSIONS: Reflectance confocal microscopy seems to be a valuable tool to noninvasively differentiate benign from malignant mucosal pigmented macules and target biopsies in cases of equivocal features.

Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular CD4(+) /CD56(+) haematodermic neoplasm (CD4/CD56 HN), is a rare distinct form of lymphoma-like entity known of dermatologists because of its marked predilection for cutaneous involvement, and its aggressive behaviour. Moreover, the association or the evolution to an acute leukaemia entity that still expresses CD4 and CD56 markers is almost systematic. This new described entity of 'CD4(+) /CD56(+) leukaemia' or 'leukaemia of plasmacytoid dendritic cell lineage' has a poor prognostic and may lead to include haematopoietic stem cell transplantation in the treatment strategy as early as possible. REPORT OF CASES: We report here four cases presenting with skin lesions and haematological signs. One of the patients underwent allogeneic stem cell transplantation, with a relapse-free survival of 40 months. We discuss the diagnosis features as well as the treatment options. CONCLUSION: A collaborative work between dermatologists and onco-haematologists is essential to give patients the best chance of complete and long-term response.

[Optimizing biopsies of the oral mucosa]. / Optimisation des biopsies des muqueuses buccales.

We had for aim to describe and illustrate the artefacts observed in biopsies of the oral mucosa, as well as the impact of sending non-representative histological material to a laboratory. This article was based on an international literature review, as well as on our experience. We analysed the problems raised, for the pathologists and the histology lab-technicians, by these artefacts as well as their impact on the pathology report patient management. We suggest simple solutions.