The keystone flap and its simplified version for malignant skin tumor defects of the lower limbs: A review of 25 cases.

BACKGROUND: Skin tumors commonly occur on the legs and are treated in first line by surgery. Several techniques are available to repair lower limb defects: secondary-intention healing, partial closure, primary closure with or without an s-plasty, or a skin graft. The lack of tissue laxity of the surrounding skin does not allow several local flaps (advancement, rotation, or transposition). Closing large skin defects at this site may be challenging. PATIENTS AND METHODS: We retrospectively reviewed a series of consecutive patients undergoing malignant tumor wide excision on lower limbs, with a keystone flap or its simplified technique (releasing incision) for closure of a skin defect. RESULTS: Twenty-five patients, 17 women and 8 men, ranging from 19 to 95 years old (mean age: 70 years) were included. Keystone flap reconstruction on the lower limbs was performed in 19 cases and the simplified technique in 6. The excised tumors were as follows: squamous cell carcinoma (n=6), basal cell carcinoma (n=9), melanoma (n=9) and Bowen's disease (n=1). Three local complications were observed. No cases of recurrence were observed. CONCLUSION: Keystone flap is a reliable surgical method for reconstruction of lower limb skin defects. Aesthetic results are better than when a skin graft is used, complications are uncommon, and prolonged operative time is avoided.

[Haemorrhagic complications following ibrutinib intake after dermatological surgery]. / Complication hémorragique secondaire à la prise d'ibrutinib après chirurgie dermatologique.

INTRODUCTION: Ibrutinib is a selective oral inhibitor of Bruton's tyrosine kinase. It is used in haematology to treat lymphoid B disorders. Haemorrhagic complications in dermatological surgery are occasionally associated with the use of anti-platelet and/or anticoagulant medication. Herein, we report a case of haemorrhage under ibrutinib following skin surgery. PATIENTS AND METHODS: A 70-year-old male patient began treatment with ibrutinib for chronic lymphocytic leukaemia had 2 basal cell carcinomas of the face. The next day he had a persistent haemorrhage lasting more than 48h, with no effects on the final scarring result. DISCUSSION: Ibrutinib is a tyrosine kinase inhibitor whose mechanism of action plays a role in platelet adhesion. It is known to cause haemorrhaging, either spontaneously or following invasive procedures, especially at the beginning of treatment. In the case of low-risk haemorrhagic procedures in which bleeding may be controlled by mechanical haemostasis, ibrutinib should be discontinued 3 days before and after surgery. In the event of recent initiation of ibrutinib and in the absence of urgent dermatological management, it is preferable to schedule any surgical procedures 3 months after the start of ibrutinib.

[Atypical Sézary syndrome in a young subject]. / Syndrome de Sézary atypique chez un sujet jeune.

INTRODUCTION: Sézary syndrome accounts for 5% of cutaneous T-cell lymphomas, with mean age of onset of 60 years. Erythroderma associated with palmoplantar keratoderma and lymphadenopathy is the usual clinical presentation, but the disease has potentially confusing polymorphic clinical features. PATIENTS AND METHODS: We report the case of a 27-year-old patient with no notable disease history, presenting generalized non-pruritic dermatosis for 3 months, with erythema and papules, and follicular distribution, localized to the limbs, the trunk and the face. Palmoplantar keratoderma was associated with acral edema. The clinical presentation was initially evocative of pityriasis rubra pilaris. Laboratory tests showed hyperlymphocytosis with Sézary cells in the blood. A diagnosis of grade IVA Sézary syndrome was made based on the skin biopsy results and the PET scan. Screening for KIR3DL2 on T-cells in blood was positive. Extracorporeal photochemotherapy was initiated but cutaneous relapse occurred, leading to combined treatment with bexarotene, which proved ineffictive. Despite numerous chemotherapies (cyclophosphamide, doxorubicin, vincristine, etoposide and prednisone, then dexamethasone, oxaliplatin and cytarabine, associated with brentuximab, vedotin, and, ultimately, clofarabine and endoxan), the patient died after 9 months. DISCUSSION: Our case illustrates an atypical clinical presentation of cutaneous lymphoma in a young patient. With a fatal outcome in 9 months despite 5 different lines of treatment, our case highlights the aggressive nature of Sézary syndrome as well as the difficulties involved in treating this disease. CONCLUSION: A diagnosis of Sézary syndrome must be considered in the event of atypical dermatosis in patients of all ages. The presence of lymphomatous clonal cells and Sézary cells in the blood, immunophenotyping of lymphocytes in blood and marrow, and a second reading of the cutaneous biopsy results enabled us to make a diagnosis of Sezary syndrome.

[Tularemia: A case report]. / Un cas de tularémie.

BACKGROUND: Tularaemia is a zoonotic disease caused by inoculation with the Gram-negative coccobacillus Francisella tularensis. It was first described in the United States in 1911 and is a rare disease with an annual reported incidence in France between 2002 and 2012 of 0.07 cases per 100,000 habitants. Reporting of the disease in humans has been mandatory in France since 2003. PATIENTS AND METHODS: Herein we report a case of tularaemia following a tick bite in a patient in the north of France. DISCUSSION: Tularaemia is a rare form of zoonosis that should be sought in the event of unexplained adenitis. Clinical presentations vary, and in certain cases only dermatological signs are manifest. Diagnosis is confirmed by bacterial serology. Rapid initiation of suitable antibiotics produces a favourable and benign outcome in most cases. However, the offending organism, which is potentially lethal, is classed as a potential bioterrorism agent.

[Vemurafenib-induced radiation recall dermatitis]. / Dermite de rappel induite par le vémurafénib.

INTRODUCTION: Radiation recall dermatitis is an uncommon inflammatory reaction of the skin appearing after several days to several years at the site of previous irradiation; it is precipitated by the use of triggering drugs, although rarely by BRAF or MEK inhibitors. PATIENTS AND METHODS: We report an unusual case of recall dermatitis induced 3 months after initiation of vemurafenib and cobimetinib therapy. DISCUSSION: Radiation recall dermatitis is a cutaneous reaction that must be known and which in rare cases such as ours may occur a long time after the end of radiotherapy.

[Characteristics of chronic wounds in substance abuse: A retrospective study of 58 patients]. / Caractéristiques des plaies chroniques chez les toxicomanes : étude rétrospective de 58 patients.

BACKGROUND: Drug addiction causes chronic wounds (CW) responsible for severe complications. Very few studies are available on this topic. The aim of our study was to describe the demographic, clinical and etiological characteristics as well as the course of CW in drug addicts. PATIENTS AND METHODS: This was a retrospective and prospective multicenter study including all drug addicts with CW. RESULTS: We included 58 patients (17 prospectively), 84.5% of whom were male, of median age 43 years, presenting multiple CW as a result of intravenous (78.2%), inhaled (41.1%) and/or snorted (20%) drug abuse. Addiction to opioids (68.4%), cocaine (47.4%) and/or cannabis (40.4%) was ended and/or treated through substitution in 79.3% of patients. CW were fibrinous and necrotic (42.9 to 53.6%), recurrent (54.2%), and in some cases had been present for more than 1 year (61.5%). Intravenous drug addiction was associated with large, fibrinous, ulcers in a setting of venous and lymphatic insufficiency (74%). Only 23% of these wounds involved the upper limbs. Necrotic ulcers associated with clinical arteriopathy were described mainly with inhaled addiction. Abscesses (50%) and erysipelas (29.3%) were the most common cutaneous complications. After 3 months, 50% of CW were improved and 29.2% of patients were lost to follow-up. DISCUSSION: Drug abuse-related CW occurred preferentially in young men with history of intravenous abuse. For the most part, CW were seen on the legs and were associated with venous and lymphatic insufficiency, and the resulting major risk for cutaneous infection increased morbidity and mortality in this population in whom medical follow-up is inherently complicated.

[Superficial temporal artery pseudoaneurysm: 2 cases]. / Pseudo-anévrisme de l'artère temporale superficielle : deux cas.

BACKGROUND: Pseudoaneurysm of the superficial temporal artery causes tumefaction in the temporal region. Herein, we report two cases. PATIENTS AND METHODS: Case 1 : a 32-year-old man presented with a slightly pulsatile nodular formation measuring 2 cm in the right temporal region that had appeared nine months after traumatic injury. A diagnosis of superficial temporal artery pseudoaneurysm was considered. Excision was performed with ligation of the afferent and efferent artery. The clinical diagnosis was confirmed by histopathology. Case 2 : a 24-year-old man presented with a nonpulsatile subcutaneous tumefaction on his left temple. Surgery was proposed based on a supposed epidermal cyst. However, the perioperative aspect suggested a lesion of arterial origin and excision was performed following ligation of the afferent and efferent artery. CONCLUSION: Pseudoaneurysm of the superficial temporal artery must be considered for all temporal cutaneous formations, particularly when there is a history of trauma. The clinical diagnosis may be confirmed by Doppler ultrasound. Surgery is the treatment of reference.

[Angiosarcoma in primary lymphoedema: A rare complication]. / Angiosarcome sur lymphœdème primaire du membre inférieur : une complication rare.

BACKGROUND: Chronic lymphoedema is classically complicated by recurring episodes of cellulitis. Degeneration to the angiosarcoma form (Stewart-Treves syndrome) is much less common. It occurs mainly in the upper limbs following surgery or radiotherapy for mammary neoplasia. Herein we report a rare case of Stewart-Treves syndrome (STS) of the lower limb as a complication of congenital lymphoedema. PATIENTS AND METHODS: A 69-year-old woman treated for bilateral lower-limb oedema present for 30years developed painful necrotic lesions in her left lower limb. A diagnosis of angiosarcoma was made based on biopsy of a nodular lesion. Since surgical excision was precluded by the extent of the lesions, chemotherapy was initiated with paclitaxel 175mg/m2 every 21days. The outcome was rapidly unfavourable and the patient died at her home during the third course of treatment. DISCUSSION: Angiosarcoma is an extremely rare complication of primary lymphoedema; treatment is poorly codified and the prognosis is unfavourable.

Factors associated with the choice of the first biologic in psoriasis: real-life analysis from the Psobioteq cohort.

BACKGROUND: Decision-making is a complex process. The aim of our study was to assess factors associated with the choice of the first biological treatment in patients with moderate-to-severe psoriasis. METHODS: Data on all patients included in the French prospective, observational, cohort, Psobioteq and initiating a first biologic prescription between July 2012 and July 2016 were analysed. Demographic information and clinical features were collected during routine clinical assessments by the dermatology team at the recruiting centres using a standardized case report form. The primary outcome was the nature of the first biologic treatment. Four groups were identified as follows: adalimumab, etanercept, ustekinumab and infliximab groups. Factors associated with the choice of the first biological agent were determined by a multinomial logistic regression model adjusted on year of inclusion. RESULTS: The study population included the 830 biological-naïve patients who initiated a first biological agent. The mean age was 46.6 years (±SD 13.9), and 318 patients (38.3%) were female. The most commonly prescribed biologic was adalimumab: 355 (42.8%) patients, then etanercept (n = 247, 29.8%), ustekinumab (n = 194, 23.4%) and infliximab (n = 34, 4.0%). In the multinomial logistic regression analysis, patients were significantly more likely to receive adalimumab if they had a severe psoriasis as defined by baseline PASI or if they had psoriatic arthritis compared to etanercept (aOR, 0.42; 95% CI, 0.16-1.07) and ustekinumab (aOR, 0.15; 95% CI, 0.04-0.52). Patients were significantly more likely to receive ustekinumab (aOR, 2.39; 95% CI, 1.04-5.50) if they had a positive screening for latent tuberculosis compared to adalimumab. Younger patients were also more likely to receive ustekinumab. Patients with chronic obstructive pulmonary disease were more likely to be prescribed ustekinumab or etanercept compared to adalimumab. There was a trend in favour of etanercept prescription in patients with cardiovascular comorbidities, metabolic syndrome and in patients with a history of cancer. CONCLUSION: We identified patient- and disease-related factors that have important influence on the choice of the first biological agent in clinical practice. Clinicians appear to have a holistic approach to patient characteristics when choosing a biological agent in psoriasis.

[Amyopathic dermatomyositis (DM) with anti-MDA5 antibodies, associated with bullous pemphigoid, Sjögren syndrome and gastric MALT lymphoma]. / Dermatomyosite amyopathique avec anticorps anti-MDA-5, associée à une pemphigoïde bulleuse, un syndrome de Sjögren et un lymphome de type MALT.

BACKGROUND: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement. PATIENTS AND METHODS: A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption. Based on the investigations performed, a diagnosis was made of bullous pemphigoid. At the same time, amyopathic dermatomyositis was discovered together with interstitial lung disease. Systemic steroids were introduced in combination with rituximab. A favourable outcome was achieved. DISCUSSION: Anti-MDA5 dermatomyositis must be considered systematically in all cases of pulmonary involvement associated with cutaneous signs of dermatomyositis, in which no muscular involvement is generally seen. This condition accounts for up to 7% of DM and carries a severe prognosis due to pulmonary involvement.

[Developments in pyoderma gangrenosum therapy in 2015]. / Actualité sur la prise en charge du pyoderma gangrenosum en 2015.

Diagnosis of pyoderma gangrenosum in 2014 continues to be made on clinical grounds, with the main form comprising an ulcer having poorly delineated purplish edges. The diagnostic criteria have been recently modified so as to rule out differential diagnoses (10% of retrospective series of pyoderma patients). The condition is commonly associated with other diseases (75%) such as emerging endocrine diseases (36.7%), and classical gastrointestinal, rheumatological and haematological diseases. The new concepts of PAPA and PASH syndromes, belonging to the category of auto-inflammatory diseases, provide new therapeutic perspectives. Corticosteroids continue to be the first-line therapy, along with immunosuppressant drugs in the event of steroid dependence. Second-line treatments consisting biotherapies show promise and interleukin-1 inhibitors have exhibited potential against pyoderma syndrome.

[Advancement cheek flap for nasal sidewall defects]. / Lambeau d'avancement-rotation jugal pour pertes de substance latéronasale et paranasale moyenne.

BACKGROUND: The nasal sidewall and adjacent cheek are frequent sites of cutaneous tumour. The aim of this study is to present an advancement-rotation cheek flap technique for nasal sidewall and paranasal cheek defects. PATIENTS AND METHODS: This is a retrospective, non-comparative study of patients undergoing a cheek advancement-rotation flap for nasal sidewall and paranasal cheek defects between September 2008 and October 2012. Sex, age, histological type of the excised lesion, method of anaesthesia, size of the defects, any occurrence of complications and results were compiled from the medical record. RESULTS: Eleven patients with a mean age of 66 years were included. For 10 patients, the tumour was a basal cell carcinoma and in one case was a squamous cell carcinoma. The defects were located in the nasal sidewall and paranasal cheek in four cases, the paranasal cheek/lower eyelid in five cases and in the nasal sidewall, anterior cheek and lower eyelid in two cases. All but one had surgery under a local anaesthetic. No patients presented post-surgical complications. One patient had a subcutaneous granuloma which subsequently subsided completely. The aesthetic result was good: two of the 11 patients had a marked nasolabial fold, one patient had a mild cutaneous deformity due to flap movement under eyelid. No recurrence of carcinoma was observed during follow-up of between 14 and 40 months. CONCLUSION: This flap should be considered for defects measuring under 3 cm on all or part of the nasal sidewall, paranasal cheek and lower eyelid fold.

[Blueberry Muffin Baby and Langerhans' congenital cell histiocytosis]. / Histiocytose langerhansienne congénitale et « Blueberry Muffin Baby ¼

BACKGROUND: Blueberry Muffin Baby is a rare neonatal cutaneous syndrome for purpuric lesions reflective of extramedullary hematopoiesis. Many causes are known, examples are congenital infections, malignancy and hematologic disorders. Langerhans' cell histiocytosis is a clonal proliferation of dendritic histiocytes. This has very rarely been associated with a Blueberry Muffin Baby presentation. CASE REPORT: We report the case of a newborn presenting with Blueberry Muffin Baby syndrome related to congenital Langherans' cell histiocytosis. At birth, he had multiple purpuric lesions on the trunk, limbs and face. Skin biopsy showed a dermal proliferation of histiocytes staining positive for S100 and CD1a. Chest and bone radiographs, and abdominal ultrasound were normal. Skin lesions have resolved in 8 weeks, the patient is in complete remission at 18 months of follow-up. DISCUSSION: A Blueberry Muffin Baby syndrome may reveal neonatal Langerhans' histiocytosis.

[Vemurafenib-induced toxic epidermal necrolysis]. / Nécrolyse épidermique toxique au vemurafenib.

BACKGROUND: Herein we report the first case of toxic epidermal necrolysis (TEN) occurring with use of vemurafenib. PATIENTS AND METHODS: A 75-year-old female patient was being treated with vemurafenib for stage IV melanoma with BRAF V600E mutation. She suddenly presented fever, diffuse pruriginous maculopapular erythema, palpebral edema, palmar bulla, conjunctivitis, cheilitis and mucosal ulceration. The condition progressed towards detachment affecting 50% of the skin area. Cutaneous biopsy revealed lichenoid dermatosis, chiefly vesicular with numerous eosinophils. Direct immunofluorescence (IFD) was negative. Vemurafenib was the only drug to which the reaction was ascribable and we concluded on vemurafenib-induced TEN. DISCUSSION: To our knowledge, this is the first reported case of vemurafenib-induced TEN, but this adverse effect, although already described in the BRIM-3 study, appears rare in clinical practice. Other severe skin reactions have been described in the literature. These include a case of Stevens-Johnson syndrome in a female patient treated with vemurafenib and previously receiving ipilimumab. A more common occurrence is cutaneous reactions involving efflorescence of benign hyperkeratotic lesions, occasionally accompanied by authentic epidermal carcinoma or keratoacanthoma, and requiring regular dermatological monitoring of patients treated with vemurafenib. CONCLUSION: If maculopapular exanthema occurs under vemurafenib, continuation of this treatment should be reassessed since the risk of progression to a more serious condition such as TEN, as seen in the present case, cannot be ruled out.

[Blastic plasmacytoid dendritic cell neoplasm revealed by ecchymotic lesions on the face]. / Tumeur à cellules plasmocytoïdes dendritiques blastiques révélée par des lésions ecchymotiques du visage.

BACKGROUND: Cutaneous CD4+CD56+ malignant tumor proliferation was previously called "CD4/CD56 hematodermic neoplasm". However, the most recent studies have shown that the disease develops from plasmacytoid dendritic cells and the tumor has been renamed "Blastic Plasmacytoid Dendritic Cell Neoplasm" (BPDCN). It is an aggressive disease with a poor prognosis and behaves like acute leukemia in the short to moderate term. PATIENTS AND METHODS: A 65-year-old man with no particular history consulted for a left laterocervical lesion of ecchymotic aspect that had appeared one year earlier. Topical corticosteroid therapy had been unsuccessful. Examination of biopsies with lymphocyte typing enabled a diagnosis of BPDCN to be made. At the histopathological level, biopsy showed an infiltrate comprising medium to large cells. Immunohistochemical examination was remarkable for the absence of expression of markers of T- and B-cell lines. However, these tumor cells expressed CD4, CD56 and TCL1. Staging of the disease was normal. Treatment with chemotherapy was initiated in collaboration with a team of hematologists. Autologous bone marrow transplant was then performed. DISCUSSION: BPDCN is a rare malignant blood dyscrasia. It is distinguished by inaugural skin involvement, with systemic manifestations occurring much later. Histopathological examination of a skin biopsy with immunostaining establishes the diagnosis. In terms of phenotype, the tumor population is highly characteristic. The cells are negative for antigens of T- and B- cell lines. However, these cells express CD4, CD56 and TCL1, which are markers of plasmacytoid dendritic cells. The disease carries a poor prognosis and evolves in the short to middle term in the same way as acute leukemia. First-line treatment consists of the chemotherapy regimens used in aggressive lymphoma or acute leukemia. A bone marrow graft is sometimes performed at the time of initial relapse. Average survival is 12 months for chemotherapy alone and 30 months for transplant after first relapse. Early bone marrow transplantation has been shown to improve survival.