[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.

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.

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.

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.

[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.

[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.

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.

[Study of skin retraction applied to the treatment of skin tumors. Mapping of the human body]. / Étude de la rétraction cutanée appliquée à la prise en charge des tumeurs cutanées. Cartographie du corps humain.

SUBJECT: Skin, the main organ of the human body, is equipped with own biomechanical characteristics, highly variable depending on intra-individual factors (location, weight status, dermatological diseases…) and interindividual (age, sex…). Despite some recent cutometric studies, our review of the literature shows that there is no currently reliable analytical model representing the biomechanical behavior of the skin. Yet, this is a central issue in dermatology surgery, especially in the treatment of skin tumors, for the proper observance of surgical margins. PATIENTS AND METHODS: We studied prospectively on 75 resection specimens (about 71 patient(s)), for the treatment of skin lesions tumor suspicious or known malignant or benign. Room dimensions were measured before and 5 minutes after excision, leading us to calculate a ratio of retraction of the skin surface. This retraction was correlated with age, gender, tumor type, and anatomic location of the site of excision. RESULTS: The power of retraction of the skin varies significantly by region of the body. It is maximum in the upper limb (hand excluded) and in the cervical region. At the cephalic region, skin of the ear and periorbital skin have capacities of important early retraction. Unlike the lower limb (foot excluded), the back skin of the nose and face appear to be a minimum of shrinkage. Age also seems to change on that capacity shrinkage, sex would have no influence. CONCLUSION: Our study confirms the variations in the ability of skin retraction based on a number of factors. In dermato-oncology, that power retraction could cause significant differences between clinical surgical margins and final pathologist margins. We believe it must be taken into account by the couple surgeon-pathologist, especially in the context of invasive and/or recurrent tumors.

Histopathological characterization of primary cutaneous melanoma using infrared microimaging: a proof-of-concept study.

BACKGROUND: The diagnosis of malignant melanoma is based upon the histological evaluation of the lesion. As such, the morphological interpretation relies on the expertise of a dermatopathologist. Infrared microimaging is emerging as a new powerful tool to investigate tissue biochemistry. Infrared spectra probe the biochemical constitution of the sample and are real tissue-specific spectroscopic fingerprints. OBJECTIVES: To assess the potential of infrared microimaging to aid in the analysis of tissue sections from primary cutaneous melanomas. METHODS: Ten samples of melanoma sections from the main histological subtypes were investigated using infrared microimaging combined with multivariate statistical analyses. RESULTS: This methodology yielded highly contrasted colour-coded images that permitted to highlight tissue architecture without any staining. It was possible to discriminate tumour areas from normal epidermis automatically, and intratumoral heterogeneity as revealed by our approach was correlated with the aggressiveness of the tumour. CONCLUSIONS: This proof-of-concept study shows that infrared microimaging could help in the diagnosis of primary cutaneous melanoma.

Drug rash with eosinophilia and systemic symptoms due to telaprevir.

We report a case of drug rash with eosinophilia and systemic symptoms (DRESS) due to telaprevir (VX-950), a specific inhibitor of the hepatitis C virus (HCV) serine protease. A 57-year-old woman with chronic hepatitis C was included in a phase 2 rollover study of VX-950. She received VX-950 in combination with pegylated interferon alfa-2a and ribavirin. Six weeks later, she developed a generalized pruritic maculopapular exanthema with malaise, fever, dyspnoea and lymph node swelling. She had an eosinophilia (up to 2.7 × 109 cells/l), large activated lymphocytes and increased concentrations of aminotransferases. Histological examination of a cutaneous biopsy was consistent with a drug rash reaction. The HCV treatment was stopped, and she was treated with topical and oral steroids. Cutaneous and systemic symptoms disappeared within 1 month. Telaprevir was considered the culprit drug. We report to our knowledge the first case of DRESS syndrome due to telaprevir. The safety data of telaprevir is derived mainly from the PROVE1, PROVE2 and PROVE3 studies. They showed a high frequency of cutaneous side effects reported under the imprecise terms of pruritus and rash, leading to an increased rate of treatment discontinuation. Telaprevir, due to its efficacy, is probably on the way to obtaining regulatory approval in the near future. It is therefore important to be aware of the high incidence of cutaneous side effects and better describe them. Our observation suggests that potentially severe hypersensitivity reactions may belong to the spectrum of rashes induced by this drug.

[Efficacy of intravenous immunoglobulin in the treatment of scleromyxoedema]. / Efficacité des immunoglobulines intraveineuses dans le traitement d'une mucinose papuleuse généralisée.

BACKGROUND: Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG). CASE REPORT: A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease. DISCUSSION: The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.