[Acral melanoma in a patient with hereditary keratoderma of the palms and soles (mal de Meleda): A chance association?] / Mélanome acral chez un patient atteint de kératodermie palmoplantaire de type mal de Méléda : une association fortuite ?

INTRODUCTION: Development of acral malignant melanoma in Mal de Meleda is highly unusual. As far as we could ascertain, to date, only 10 previous cases have been published. Herein, we report a new case. OBSERVATION: A 64-year-old Algerian man was followed for familial Mal de Meleda. The diagnosis was based on clinical presentation as he had a non-syndromic hereditary foul-smelling and yellowish palmoplantar keratoderma transgrediens. After the failure of acitretin, which had not prevented retractile and mutilating progression of the palmoplantar keratoderma, he had undergone surgery with graft excision of both palms. At the age of 59 years, he presented a tumor on the dorsal aspect of the 1st phalanx of the 3rd finger of the right hand in a non-grafted area. The diagnosis of acral melanoma was confirmed histologically. The radiological findings showed a specific homolateral axillary adenopathy. He underwent digital amputation of the 3rd finger, with lymph node dissection and chemotherapy involving dacarbazine. Follow-up at 5 years showed complete remission of the melanoma. DISCUSSION: Mal de Meleda is a hereditary palmoplantar keratoderma due to mutation of the SLURP1 gene. Clinical diagnosis is based on the typical phenotype in adulthood. The occurrence of acral melanoma, which is a rare form of melanoma (1 to 7%), especially in the fingers, together with an unusual palmoplantar keratoderma in a subject of type IV phototype does not appear to be a chance event. This association seems to be the outcome of immune dysregulation rather than of chronic inflammation.

[Thrombotic skin gangrene: A rare extra-intestinal manifestation of ulcerative colitis]. / La gangrène cutanée thrombotique : une manifestation extradigestive rare de la rectocolite hémorragique.

BACKGROUND: Thrombotic cutaneous gangrene is a rare extra-intestinal manifestation of ulcerative colitis with a severe prognosis. CASE REPORT: A 35-year-old woman with a 7-year history of ulcerative colitis presented with extensive ecchymotic lesions that began a few hours earlier. On examination, she was febrile with multiple necrotic lesions. Skin biopsy showed multiple microthrombi in the dermal vessels. A diagnosis of thrombotic cutaneous gangrene was established. The patient was treated with heparin and systemic corticosteroids. The majority of cutaneous lesions showed improvement after 1 month. Thrombophlebitis of the left lower limb occurred subsequently. CONCLUSION: Thrombotic cutaneous gangrene is attributed to microvascular thrombosis, which arises from the hypercoagulability observed in ulcerative colitis. Complete blood and coagulation tests must be performed and early anticoagulation with heparin must be considered in order to prevent the progression of cutaneous infarction.

[Obstructive sleep apnea syndrome. Is it a risk factor for diabetic retinopathy?]. / Le syndrome d'apnée de sommeil. Est-il un facteur de risque de rétinopathie diabétique ?

PURPOSE: The goal of our study was to evaluate the prevalence of diabetic retinopathy in type 2 diabetic patients with obstructive sleep apnea syndrome. PATIENTS AND METHODS: We performed a comparative study including 60 type 2 diabetics separated into 2 groups: a 1st group composed of 20 patients with obstructive sleep apnea syndrome and a 2nd group composed of 40 patients without this syndrome. The two groups were matched for age and length of diabetes. All patients underwent a complete ophthalmic examination. RESULTS: In both groups, the mean patient age was 59 years, the diabetes had been present for an average of 10 years, and the HbA1c was 10%. Diabetic retinopathy was diagnosed in 11 patients from the 1st group (55%) and 6 cases from the 2nd group (15%) (P=0.03). Non-proliferative and proliferative retinopathy were found in 7 cases (35%) and 4 cases (20%), respectively, in the 1st group and in 3 cases (7.5%) in the 2nd group for both types (P=0.03 and P=0.042, respectively). Diabetic macular edema was observed in 4 patients (20%) in the 1st group and 2 patients (5%) in the 2nd group (P=0.04). CONCLUSION: Obstructive sleep apnea syndrome is an independent risk factor for the appearance and aggravation of diabetic retinopathy. This underscores the importance of systematic screening for, and timely management of, sleep apnea.

[Allopurinol-induced DRESS complicated by hemophagocytic lymphohistiocytosis]. / DRESS à l'allopurinol compliqué d'un syndrome d'activation macrophagique.

BACKGROUND: Drug hypersensitivity syndrome is among the most severe drug hypersensitivity reactions and in rare cases it may progress to hemophagocytic lymphohistiocytosis. Herein, we report a case of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. CASE REPORT: An 18-year-old girl presented with maculopapular rash associated with cervical lymphadenopahy appearing 3 weeks after treatment with allopurinol. Her hemodynamic status at admission was unstable. Cutaneous examination revealed an itchy maculopapular rash, which was purpuric at certain sites, together with facial edema. The diagnosis of drug hypersensitivity was suggested and was confirmed by histological examination of a skin biopsy. Allopurinol was stopped. Two weeks later, however, eosinophilia was noted. Further, four days after discontinuation of allopurinol, in view of the laboratory signs of bicytopenia, hyponatremia, hypertriglyceridemia and hyperferritinaemia, as well as the presence of hemophagocytosis in bone marrow, a diagnosis was made of lymphohistiocytosis hemophagocytic syndrome complicating a drug reaction with eosinophilia and systemic symptoms. Moreover, viral serology tests were negative. The patient was given intravenous immunoglobulin and the outcome was good. DISCUSSION: The literature contains only very few reports of drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. The incriminated drugs were vancomycin, lamotrigine and phenobarbital. To our knowledge, there has only been one report of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis.

[Epidemiology of open globe injuries in the Tunisian region of Cap Bon: Retrospective study of 100 cases]. / Épidémiologie des traumatismes oculaires à globe ouvert dans la région tunisienne du Cap Bon : étude rétrospective à propos de 100 cas.

PURPOSE: The goal of our study was to assess the epidemiological characteristics of ocular injuries in the Cap Bon region. PATIENTS AND METHODS: We retrospectively reviewed the records of 100 patients, hospitalized and treated in the department of ophthalmology at the Mohamed Taher Maamouri university medical center in Nabeul, Tunisia for an open globe injury between January 2006 and November 2013. The mean duration of follow-up was 15.9 months. RESULTS: The average age of the patients was 31.9 years. Domestic accidents accounted for 30% of cases of trauma, followed by violence (29%). The trauma was sharp in most cases (54%). The injury was penetrating in most cases (65.7%). The initial visual acuity was ≤ 1/10 in 69.8% of cases. The wound was isolated to the cornea in 59.7% of cases and scleral in 25% of cases. The most common associated ocular lesions were traumatic cataract (35%), followed by hyphema (32%). An intraocular foreign body was present in 4% of cases. At the final examination, 41.8% had a final visual acuity ≤ 1/10. CONCLUSION: Open globe injuries represent a real public health problem given their psychological and functional repercussions. The best treatment is preventative, involving better awareness in the family, school and work environment.