Hyaline fibromatosis syndrome is the current name for clinical manifestations of
diseases previously known as “infantile systemic hyalinosis” and “juvenile
hyaline fibromatosis”. The authors report representative clinical cases of each
one of the above subtypes with emphasis on cutaneous manifestations and
difficulties for early diagnosis in this syndrome, essentially of
multidisciplinary approach.
A 57-year-old female showed bulky, loose tumors, which progressively spread to
her arms, anterior chest, and back. She reported dysphagia and dyspnea after
mild exertion. She denied alcohol consumption. CT scan of her chest showed no
internal lesions. Benign symmetric lipomatosis is a rare syndrome, clinically
described as multiple nonencapsulated lipomas of various sizes and symmetrical
distribution. This syndrome has three known phenotypes; in type 2
(Launois-Bensaude syndrome), lesions occur primarily on the shoulders, upper
arms, and chest, and is unrelated to alcoholism. It causes aesthetic deformities
and might block the upper airways. Mediastinal invasion might occur as well.
BackgroundAcral lentiginous melanoma is a melanoma with poor prognosis which is frequently diagnosed at an advanced stage. Since the thickness of tumour is one of the main prognostic factors, this case can exemplify how important complete histological analyses looking for focal invasiveness can be.Case reportA 77 year-old woman with a black spot with slow progressive growth on the left plantar region. She sought medical attention due to the expansion onto the dorsal surface of toes. The lesion had irregular borders and had spread to half the plantar surface. Histopathology confirmed the clinical suspicion of acral lentiginous melanoma Clark level IV and 2.6 mm Breslow thickness. The surgical specimen was entirely processed for histological evaluation, requiring 53 slides. Tumor dermal invasion was detected in only three out of 53 glass slides as the invasiveness was not identified by clinical, dermatoscopy or macroscopy exams.ConclusionSectioning through the entire lesion is considered very important to determinate the appropriate stage of the disease and the correct treatment and patient follow-up.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1513617994148349.
Dermatomyositis is a rare inflammatory disease, autoimmune, with proximal
myopathy associated with characteristic dermatological manifestations. In
adults, 20-50% of the cases are paraneoplastic manifestation, being mandatory
the workup for malignancy Herein we report a case of a woman with classic
dermatological presentation of dermatomyositis and newly diagnosed breast
cancer. In general, the clinical presentation of paraneoplastic dermatomyositis
is more exuberant and manifestations may precede, concur or succeed the
diagnosis of neoplasia. The prognosis of cases associated with breast cancer is
worse than the idiopathic form. Treatment is based mainly on the resolution of
the underlying disease, beyond immunosuppressants.
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