[A new case of Rowell's syndrome]. / Un nouveau cas de syndrome de Rowell.

INTRODUCTION: This article introduces a new case of Rowell's syndrome, a controversial entity defined by the association of lupus erythematosus and erythema multiforme. OBSERVATION: A 43-year-old woman was diagnosed with lupus erythematosus induced by esomeprazole. Because her eruption did not improve after withdrawal of the drug, hydroxychloroquine was administered. Two weeks later, the patient described new annular lesions on her chest and arms, both erosive and crusted, and some had a target-like appearance. The oral mucosa was also affected. Histology revealed sub-epidermal blistering with keratinocytic necrosis, strongly suggesting erythema multiforme. Screening for other causes of erythema multiforme proved negative. A positive outcome was achieved with corticosteroids and hydroxychloroquine. One year later, the patient was in complete remission for both lupus erythematosus and erythema multiforme. DISCUSSION: The association of lupus erythematosus and erythema multiforme first described in 1963 is known as Rowell's syndrome. While diagnostic criteria have been established in the literature, the reality of this entity is still contested. The annular lesions of subacute lupus erythematosus may be confused with the lesions of erythema multiforme. As suggested in the above section, other authors consider Rowell's syndrome to be a singular entity. Indeed, our patient developed lesions distinct from those initially suggesting subacute lupus erythematosus, in particular: the target-like aspect of the elementary lesions, mucosal involvement, a distinct histological aspect, and dissociated outcomes. Ultimately, the definition of Rowell's syndrome remains highly debated.

[A second case of white lentiginosis]. / Un second cas de lentiginose blanche.

INTRODUCTION: White lentiginosis is characterized by white guttate macules with a typical histological structure: lentiginosis hyperplasia with hypopigmentation. OBSERVATION: A 23-year-old woman, from Algeria, presented with white macules from 5 years ago. Few women in her family had the same trouble. Cutaneous examination revealed diffused white little macules. Histological examination showed a lentiginous hyperplasia of the epidermis, with elongated club-shaped rete ridges and an unusual loss of pigmentation. DISCUSSION: Different diagnosis should be discussed with disseminated leucoderma macules. Nevertheless, the only one which corresponds with our case is the white lentiginosis. To our knowledge, it is the first case described since Grosshans et al. in 1994. Our patient is a descendant of the original case's family. This new case appears to confirm the genetic origin of the disorder and suggests autosomal dominant or X-linked inheritance.

[Skin necrosis during long-term fluindione treatment revealing protein C deficiency]. / Nécroses cutanées tardives sous fluindione révélant un déficit en protéine C.

BACKGROUND: Cutaneous necrosis is a rare complication of vitamin K antagonist therapy. It presents as cutaneous hemorrhagic necrosis and usually occurs at the start of treatment. We describe an atypical case of recurrent skin necrosis after two years of treatment with fluindione. CASE REPORT: A 70-year old woman with a history of venous thromboembolism and obesity presented with a large haemorrhagic necrosis of the abdominal wall. She had been treated with fluindione for two years. Genetic protein C deficiency was discovered. Resumption of vitamin K antagonist therapy was followed by recurrence of skin necrosis despite concomitant administration of heparin. Treatment with vitamin K antagonists could not be continued. DISCUSSION: This observation is unusual due to the late onset of skin necrosis. The condition usually begins shortly after initiation of vitamin K antagonist therapy, generally between the third and the sixth day of treatment. It is due to a transient hypercoagulable state in patients with protein C deficiency or, in rare cases, protein S deficiency. This late-onset skin necrosis, occurring many years after initiation of anticoagulant therapy, may be explained by a sudden worsening of pre-existing protein C deficiency due to infectious and iatrogenic factors.

[Blasch-kolinear psoriasis revealed by infliximab therapy]. / Psoriasis blaschko-linéaire révélé par un traitement par infliximab (Remicade).

BACKGROUND: Blaschko-linear psoriasis is a rare disease about which only a few publications have appeared in the literature. This form of psoriasis poses problems of differential diagnosis with regard to other forms of inflammatory Blaschko-linear dermatoses. Herein, we report an original case, the linear nature of which was revealed by treatment with infliximab. CASE REPORT: A 29-year-old man presented chronic psoriasis present for 17 years and resistant to various forms of systemic therapy. Treatment with infliximab 5mg/kg given on D1, D15 and two-monthly, thereafter resulted in practically complete resolution of all skin lesions after the fourth infusion. The only remaining lesions were psoriatic erythematous-squamous, non-pustular lesions with a Blaschko-linear pattern, limited to one side, on the left arm and left leg. These lesions persisted after 10 courses of infliximab, although no other lesions reappeared. DISCUSSION: This case was original in terms of the revelation of Blaschko-linear lesions during treatment with infliximab, despite the complete disappearance of diffuse psoriatic plaques, thus suggesting the existence in this patient of two cell populations, each having a different response to biotherapy.

Destabilization and intracranial fragmentation of a full metal jacket bullet.

We report a case with an atypical entrance wound as a result of a destabilized full metal jacket bullet penetration. The destabilized bullet by an impact with the dorsal hand experiences a yawing to tumbling motion in flight. The large angle of yaw induces a larger presenting profile upon impact that contributes, associated to a rapid deceleration, to a greater mechanical force on the projectile structure and a fragmentation into core and jacket. Forensic pathologists have to be aware that the metal jacket bullet could tend to break up outside or inside the body particularly after a shooting through a target. This phenomenon induces atypical entrance wounds and atypical X-ray presentation.

[Two cases of subcutaneous panniculitis-like T-cell lymphoma (CD4- CD8+ CD56-)]. / Lymphome T sous-cutané (CD4- CD8+ CD56-) à type de panniculite: deux cas.

BACKGROUND: Subcutaneous T-cell lymphoma is a rare disease and diagnosis is often difficult. We report two cases of subcutaneous T-cell lymphoma simulating panniculitis and initially treated with systemic steroids. PATIENTS AND METHODS: Case No. 1. A 75-year-old woman, otherwise asymptomatic, presented with plaques and nodules of the legs present for 4 months. Histological and immunohistochemical analysis revealed subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Improvement was observed with systemic steroids followed by radiotherapy. Case No. 2. A 53-year-old woman presented with a 10-year history of recurrent infiltrated plaques. Lupus panniculitis was evoked but not confirmed. In the context of new panniculitis lesions, histological examination showed a dense lymphocytic infiltrate involving the fat lobules. Lymphocyte immunophenotyping and genotyping led to a diagnosis of subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Molecular analysis showed T-cell monoclonality (alpha beta). The lesions improved after few months of systemic corticosteroids. Although new nodular lesions appeared from time to time, further therapy was not deemed necessary. DISCUSSION: A distinction is currently made between two types of subcutaneous T-cell lymphomas and the prognosis and therapeutic consequences differ widely. In our two patients, subcutaneous CD8+ T-cell lymphoma was diagnosed. The clinical course was indolent in both cases. Differential diagnosis with regard to lupus panniculitis can be difficult because of its slow progression. In such cases of low-grade lymphomas, first-line therapy may be limited to systemic corticosteroids.

[Chromium-induced vasculitis-like purpuric allergic contact dermatitis]. / Dermite de contact purpurique au chrome simulant une vasculite.

INTRODUCTION: Purpuric allergic contact dermatitis is a rare and poorly understood condition. CASE REPORT: A 27-year-old male patient with a personal history of atopic dermatitis since childhood consulted for chronic papular-purpuric rash present for 7 years. Moderate pruritus was seen. Profuse lesions were observed on the palms and soles and on the upper and lower limbs, with sparing of the trunk. These lesions consisted of purpuric papules, in some cases with crusts, forming large plaques. The clinical picture was initially suggestive of vasculitis, but this diagnosis was ruled out by histological examination and laboratory tests. Skin patch tests were evocative of chromium-induced contact dermatitis. Retrospective directed history-taking confirmed the relevance of the latter test since it revealed regular wearing of leather clothing. Lasting cure was achieved following eradication of the allergen. DISCUSSION: Reports of contact purpuric dermatitis are rare. This condition has been described principally for allergens consisting of rubber or dyes used in clothing. Our case was notable on account of the severity of the lesions, mimicking vasculitis, as well as the novelty of the incriminated allergen, chromium, found in leather garments. It underlines the value of routine skin patch tests in the event of chronic non-specific dermatitis. To our knowledge, this is the first reported case of chromium-induced purpuric allergic contact dermatitis.

[Hypersensitivity to fluindione (Previscan). Positive skin patch tests]. / Syndrome d'hypersensibilité à la fluindione (Préviscan). Positivité des tests épicutanés.

INTRODUCTION: Fluindione (Previscan) is an oral anticoagulant belonging to the vitamin K antagonist class and is very widely used in France. While bleeding is a common complication, severe immunoallergic reactions are less frequent. The authors report a case of drug-induced hypersensitivity syndrome. CASE REPORT: A 75 year-old woman was hospitalized for diffuse erythematous papular rash associated with facial oedema. These symptoms appeared 3 weeks after the beginning of treatment with fluindione, allopurinol and perindopril. Laboratory tests showed hyperleukocytosis, mixed hepatitis and moderate renal failure, with the entire picture being evocative of drug-induced hypersensitivity reaction. The eruption was associated with eosinophilia, hepatic cytolysis with cholestasis, and acute renale failure. While allopurinol and perindopril were stopped definitively, fluindione was only suspended temporarily following overdosage. On reintroduction, rapid recurrence of clinical and biologic signs was observed with increased severity. The skin rash resolved completely on withdrawal of the drug. Patch tests performed later were positive for fluindione and negative for allopurinol and perindopril. DISCUSSION: These manifestations were consistent with the diagnosis of drug-induced hypersensitivity syndrome due to fluindione. Very few cases have been described with fluindione despite widespread prescription of the treatment is in France. While there may be no skin involvement, immunoallergic signs such as fever, hepatitis and acute tubular interstitial nephritis have been described with fluindione and these may be related to this syndrome (DRESS - Drug Reaction with Eosinophilia and Systemic Symptoms). Skin patch testing, which is easily performed, can be extremely helpful in determining a causal relationship with medication.

Fatal blunt pancreatic trauma secondary to assault and battery: a case report.

The authors report on an fatal case of closed trauma of the pancreas in a context of violence. A 55-year-old man was found unconscious on the sidewalk and died a short time after being taken to the hospital. He had been hit with several punches to the face and abdomen 6 h before. The post-mortem examination showed numerous bruises over the whole body, a haemoperitoneum, a fissuration of the spleen and a massive peripancreatic haemorrhage associated with a complete dilaceration of the pancreas head. Histological examination of the pancreas revealed a massive necrosis associated with a subtotal disappearance of the acini, numerous sites of cytosteatonecrosis and a large haemorrhagic suffusion of the peripancreatic tissue. This case illustrates the possibilities of pancreatic injuries induced by blunt force aimed at the abdomen in a context of violence. Even if this occurs as an isolated injury it can result in rapid death because of the particular type of pancreatic fracture which is frequently involved. In postmortem situations, the pancreas should be systematically checked at necropsy and a histological examination should be carried out at the slightest doubt of a pancreatic lesion or suspicion of blunt force abdominal injuries.

Leucoderma associated with flares of erythrodermic cutaneous T-cell lymphomas: four cases. The French Study Group of Cutaneous Lymphomas.

We describe four patients with erythrodermic cutaneous T-cell lymphomas (two with erythrodermic mycosis fungoides, and two with Sézary syndrome) who presented with extensive hypopigmented lesions that occurred during flares of their cutaneous disease. These cases must be distinguished from previously described hypopigmented mycosis fungoides where hypopigmented lesions were the sole manifestation of the lymphoma. In two cases a biopsy was performed on hypopigmented skin, showing an infiltrate of atypical lymphocytes with epidermotropism and absence of melanocytes, as in vitiligo. It is suggested that the hypopigmentation could be due to the cytotoxicity of tumour or reactional lymphocytes directed against melanocytes.

Pilotropic cutaneous T-cell lymphoma without mucinosis. A variant of mycosis fungoides? French Study Group of Cutaneous Lymphomas.

BACKGROUND: In the course of mycosis fungoides, pilofollicular manifestations without mucinosis (papules, keratoses, comedones, or epidermal cysts) are rare (15 cases reported). Therefore, histological and clinical diagnoses may be difficult. The clinical course and histopathological and immunohistochemical findings in 9 patients are described. OBSERVATIONS: Pilofollicular lesions were present at the onset (n = 3), before (n = 3), or during a relapse of mycosis fungoides (n = 3). Comedones and epidermal cysts were most frequent (n = 5). They disappeared with lymphoma therapy (n = 4), therapy with isotretinoin (n = 3), or spontaneously (n = 1), or they persisted (n = 1). Clues to the histopathological diagnosis consisted of pilotropism of the infiltrate with minor alteration of the hair follicle walls. The infiltrate was monomorphous and composed of sezariform CD4+ lymphocytes. Pilotropic or peripilofollicular infiltrates, or the absence of infiltrate, were detected in consecutive biopsy specimens obtained from the same patient. The keratinocyte expression of intercellular adhesion molecule type 1 was observed in the hair follicle bulb in front of the pilotropic infiltrate but not in the epidermis. No staining was observed in biopsy specimens of 6 of 7 patients with follicular mucinosis, of folliculitis lesions, or of normal hair follicles. CONCLUSIONS: Our findings indicate the role of adhesion molecules in pilotropism leading to mechanical obstruction of the follicle by tumoral cells followed by hyperkeratosis and cyst formation. It remains to be determined if the expression of intercellular adhesion molecule type 1 is the cause or the consequence of pilotropism. By becoming more aware of it, this variant of mycosis fungoides is probably not so rare.

[AIDS-related eosinophilic folliculitis. Efficacy of high dose topical corticotherapy]. / Folliculites à éosinophiles associées au SIDA. Efficacité d'une corticothérapie locale forte.

INTRODUCTION: A chronic pruriginous eruption of eosinophil-rich follicular papules and pustules is observed in AIDS patients. The pathogenesis of this disease, termed eosinophil folliculitis, is poorly understood and treatment is debated. CASE REPORT: A 30-year-old woman with AIDS developed highly pruriginous lesions of 5 month duration localized on the face, the trunk and upper limbs. There were papulo-pustules and excoriated papules. The elementary lesion was a follicular pustula. The eosinophil count was normal. The pathology examination revealed a rich eosinophil infiltration around the hair follicles and sebaceous glands as well as follicular spongiosis. Search for demodex, pityrosporons and a large number of infectious agents was negative. Oral minocyclin was uneffective. Local high-dose corticosteroids produced a remarkable effect and led to complete remission in 9 months. DISCUSSION: Most cases of eosinophil folliculitis associated with AIDS have been reported in men, but rarely in Europe. The remarkable efficacy of the local corticosteroid in this case was exceptional. This condition could result from inappropriate inflammatory reaction in AIDS induced by various factors including demodex and pityrosporon. Several therapeutic approaches have been proposed to eradicate the triggering factors and others to modify the immune response. The exceptional response to the short local treatment with corticosteroids would suggest that this approach could be proposed as first intention treatment in eosinophil folliculitis associated with AIDS.

[Adenoid cystic carcinoma or cylindroma of the breast. General review in light of one case report]. / Carcinome adénoïde kystique ou cylindrome du sein. Revue générale à propos d'une observation.

Cystic adenoid carcinoma or cylindroma of the breast is a rare tumour, and until new only 140 cases have been described in the literature. The clinical criteria are not very specific: tumour in a woman of 50 years of age, with slow growth, unilateral, well delimited, firm, peri-areolar, painless and not adherent to the skin nor the deep tissues. The diagnosis is made by histological examination which shows the presence of pseudo-cysts encased in cellular masses heaped up, composing epithelium and myo-epithelial elements. These are sometimes visible with optic microscopy, and if necessary confirmed by electron microscopy and using immunohistochemical techniques. It is now realised that the myo-epithelial cell plays a role in the histogenesis of the cylindroma. The outcome is usually good after simple surgical removal which has to be sufficient to avoid local recurrences. All the same there have been rare cases of metastases in the literature which means that these cases should be followed up carefully.