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Abstract

WHAT IS YOUR DIAGNOSIS? CASE REPORT White male patient, 44 years-old, reported the appearance 7 years ago of lesions in the plantar region, slow-growing, painful to the touch and which gradually caused difficulty in walking. Examination revealed erythematous-violaceous nodule with a smooth surface, 1cm in diameter, in the left plantar region, painful on palpation (Figure 1). Patient (smoker and drinker) denied familial cases. Ultrasound performed on left foot revealed a hypoechoic image containing a small, anechoic, irregular, circumscribed, area subcutaneously in the plantar region. No change in vascularization was revealed by color Doppler ultrasound. Surgical excision of the lesion was performed and subsequent histopat-hological examination revealed proliferation of blood vessels in the dermis surrounded by round cells with uniform nuclei in the stroma in a fibrous capsule (Figures 2). Performed actin which showed diffuse and intense smooth muscle expression (Figure 3). CASE REPORT 60 year-old male patient with asymptomatic nodules on the face, neck, chest and scrotum noted 3 months previously. Reported smoking, drinking and illicit drug use and complained of fatigue, heartburn and weight loss of 15kg over the 3-month period. Physical examination revealed erythematous-viola-ceous nodules on the scalp, face, neck, chest, upper limbs and scrotum. The nodules, some of them exulcerated, were of firm consistency, with smooth surfaces , measuring between 0.5 and 2.0 cm in diameter, asymptomatic. Palpable lymph nodes in the occipital and posterior cervical regions. Increased abdominal size was noted, with ill-defined mass (Figures 1 and 2). A biopsy of a lesion was performed and the histopathological examination revealed diffuse infiltrate in the dermis, with the presence of cells with clear cyto-plasm with nuclei compressed to the periphery, characterizing signet-ring cells (Figure 3). Digestive endoscopy showed nodular and ulcerated lesions in the antrum (also biopsied). A CT scan of the abdomen revealed an infiltrating lesion, with swelling in the antrum and gastric body. The chest scan was normal. RELATO DO CASO Dermatological examination of a male child, 2 years and 10 months old, revealed brownish macules (some keratosic and interspersed with hypopigmented patches) on the the face and with bleeding papulo-erythematous lesions on the upper lip, together with pinpoint hyperchromic, reddish-brown macular lesions on the abdomen and limbs (Figures 1 and 2). Clinical examination showed significant retardation of growth and development, microcephaly, generalized hypotonia with hyporeflexia, photophobia, hypogonadism and abdominal disten-sion. Head circumference 38.5 cm, chest 38 cm and abdomen 40 cm (Figures 2 and 3). Seizures experienced since birth. Anato-pathological exam of lip lesion showed pyogenic granuloma. Cranial CT scan showed right-sided open-lip schizencephaly, periventricular calcifications, absence of septum pellucidum and dilatation of the lateral ventricles. A brain MRI showed supratential dilatation, schizencephaly, agenesis of the septum pellucidum and septo-optic dysplasia. Ophthalmologic evaluation: positive and symmetric red reflex, poor response to light and changes in pupillary reflexes. Normal ECG and echocardiogram. Normal karyotype, 46XY. Upper GI endoscopy: sliding hiatal hernia. USG of the scrotum did not find the testicles and epididymis. No ectopic testis. Serology for HIV, herpes, toxoplasmosis and cytomegalovirus (CMV) negative. The clinical and laboratory tests were compatible with the DeSanctis-Cacchione syndrome.