Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized by the evolution of circumscribed erythematous loose skin masses, especially in the body folds, and histologically by a loss of elastic fibers and granulomatous T‐cell infiltrates. This disease is often associated with preceding or successive lymphoproliferative malignancies, especially Mycosis Fungoides (MF) and Hodgkin’s Disease (HD). Whether Granulomatous Slack Skin Disease is a benign disorder, an unusual host reaction or a precursor of malignant lymphoma or an indolent Cutaneous T‐cell Lymphoma (CTCL) in itself, is still a controversy. This article reviews its literature on the etiology, clinical findings, and treatment of Granulomatous Slack Skin Disease. It also concentrates on its association with Hodgkin’s disease and its comparison with Mycosis Fungoides and Sezary Syndrome.
We describe an unusual case in which a 35-year-old man presented with skull base mucormycosis with osteomyelitis secondary to squamo us cell carcinoma of the temporal bone. We also review the literature on the clinical characteristics, diagnosis, and treatment of mucormycosis.
Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal-recessive anomaly that primarily affects the skin and the mucosa of the upper aerodigestive tract in children. It is caused by hyaline deposits in tissues. Hoarseness secondary to laryngeal involvement is frequently the first presenting feature. It is important to consider this disease in the differential diagnosis of hoarseness because it might lead to life-threatening airway compromise. We report a case of lipoid proteinosis in a 12-year-old girl who presented with hoarseness and skin lesions.
Patient presented with a congenital discharging branchial sinus. Pre-operative work up including a CT scan of the abdomen-pelvis revealed absence of the left kidney. Branchio-Oto-Renal (BOR) syndrome, a rare autosomal dominant disorder is characterized by branchial arch anomalies, otological and renal anomalies. Clinical manifestations tend to have considerable variability, But no case with branchial and renal abnormality sans otological defects has been described yet in the spectrum of BOR syndrome variants.
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