Cutaneous angiosarcoma is a rare and highly aggressive neoplasm with poor prognosis. Owing to its variable presentation, it may be a challenging clinical and histological diagnosis. Herein, we describe a particular case of cutaneous angiosarcoma of the head and neck in a 71-year-old male with skin type V who presented with solid, progressive eyelid edema. A histological examination of skin biopsies first concluded cutaneous metastasis of an undifferentiated carcinomatous tumor. Immunostaining was essential to reach the correct diagnosis. The treatment of cutaneous angiosarcoma remains unsatisfactory. We sincerely hope that the prognosis of cutaneous angiosarcoma will be improved with the use of targeted therapies based on current genetic studies as it has been for melanoma.
The literature insufficiently describes the etiologies of chronic penoscrotal edema, and even more so those of chronic permanent scrotal erythredema. Herein, we report the case of a sixteen-year-old male who presented, in addition to permanent and chronic scrotal erythredema, classical cutaneous manifestations of systemic lupus. The patient had received systemic corticosteroid therapy combined with hydroxychloroquine. The evolution was good, without new outbreaks or recurrences of penoscrotal edema. Apart from the usual urological emergencies, systemic diseases may be involved in scrotal erythredema, which requires long-term medical management.
Introduction: Cystic lymphatic malformations (CLM) are rare benign, slow-flowing congenital malformations composed of abnormal cystic dilatations. They can be microcystic, macrocystic or very rarely mixed. We describe a rare case of mixed CLM in a 12-year-old female patient. Observation: A 13-year-old girl, from a consanguineous marriage, who presented to our structure for a swelling of the right forearm which appeared 3 weeks previously and which was progressively increasing in size. Examination found a soft, depressible and painful subcutaneous mass in the right forearm associated with vesicular hemorrhagic lesions grouped into plaques in the ipsilateral arm initially treated as shingles without improvement. Diagnosis of mixed CLK was made clinically and confirmed on imaging. Therapeutic abstention was recommended with monitoring and local treatment. Discussion: The originality of our observation lies in the association of micro and macrocystic CLM, the late appearance of the lymphatic malformation, and clinical presentation of lymphangiectasias confused with shingles. Key words: Mixed lymphatic malformations, MLK, Vascular malformations
Sir, An eight-month-old girl was referred to our department with an extensive lingual ulceration. The parents noted that she had habitually bitten her tongue since the release of her first teeth at the age of six months. She was a poor feeder and did not sleep well because of the painful lingual ulceration. There was no family history of developmental disorders or congenital syndromes. Intraoral examination revealed a deep, circular, and extensive ulceration of the whole ventral surface of the tongue with intermittent bleeding in the tongue (Figs. 1a – c). An examination of the rest of the intraoral mucosa revealed that the lower central incisors had recently erupted. However, there were two other ulcerations of the palmar surface of the second and third fingers caused by nocturnal finger biting. Neurological examination noted a lack of pain sensitivity related to peripheral neuropathy diagnosed as congenital insensitivity to pain. Based on the clinical features and the particular site on the ventral surface of the tongue against the lower central incisors and ulcerative lesions of the fingers due to self-biting, the lesion was diagnosed as Riga–Fede disease. Because of the size of the ulceration, significant pain during feeding led to inadequate nutrient intake associated with permanent sleep disturbances. Radical treatment was chosen and the lower central incisors were extracted. Topical corticosteroids were prescribed to help with healing. The term Riga–Fede disease has been used to describe a traumatic ulceration that has occurred on the ventral surface of the tongue in newborn babies and infants. It is most commonly related to neonatal or natal teeth but may also occur in infants after the eruption of the primary lower incisors [1]. This benign ulceration occurs as a result of repetitive mechanical trauma caused to the oral mucosal surfaces by the teeth and is most commonly located on the ventral surface of the tongue against the teeth [1,2]. Riga–Fede disease may reveal an underlying developmental or neurologic disorder, including congenital insensitivities to pain [3]. The case of our patient was associated with congenital insensitivity to pain. Failure to diagnose may lead to dehydration and inadequate nutrient intake in the infant because of the significant pain during feeding. No biopsy is needed. The diagnosis of Riga–Fede disease is based on clinical characteristics [1,2]. Treatment should focus on eliminating the source of trauma. Conservative treatment is attempted at first by grinding the sharp edges of the teeth and placing composite resin in a dome shape or by placing a protective ring. If conservative treatment fails to heal the wounds, radical treatment may be necessary, such as extraction of the teeth [2,3]. We believe that Riga–Fede disease must be recognized by clinicians to avoid misdiagnosis and delayed treatment.
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