ABSTRACT
Bullous pemphigoid is most commonly seen in elderly patients, however, an increasing number of cases in children have been reported. In this article we present a case of a five-month-old boy who was admitted with red annular plaques over his entire body and vesicles and bullae on his hands and feet. Correct diagnosis was made by skin biopsy and after reviewing the literature high dose oral steroids therapy (1 mg/kg/day) was successfully initiated. The cause of the disease remains unknown. With correct treatment the prognosis is good with documented ten-year follow-up without relapse.
Subject(s)
Pemphigoid, Bullous , Glucocorticoids/administration & dosage , Glucocorticoids/therapeutic use , Humans , Infant , Male , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/pathology , Prednisolone/administration & dosage , Prednisolone/therapeutic useABSTRACT
A 60-year-old man with chronic lymphocytic leukaemia was admitted to our department with fever and hepatospleno-megaly. Laboratory findings revealed hyperferritinaemia of 40,300 microgram/l and both liver and renal dysfunction. A bone marrow biopsy showed haemophagocytosis consistent with haemophagocytic syndrome. The serology was compatible with acute Cytomegalovirus infection. The patient received therapy with ganciclovir, prednisolone and gamma globulin, and the acute renal failure was treated with haemodialysis. The patient responded well to the treatment and was discharged after a month with normal lever and renal function.
Subject(s)
Acute Kidney Injury/virology , Cytomegalovirus Infections/complications , Lymphohistiocytosis, Hemophagocytic/virology , Acute Kidney Injury/therapy , Cytomegalovirus/isolation & purification , Cytomegalovirus Infections/drug therapy , Humans , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lymphohistiocytosis, Hemophagocytic/drug therapy , Lymphohistiocytosis, Hemophagocytic/pathology , Male , Middle AgedSubject(s)
Lymphoma, T-Cell, Cutaneous/diagnosis , Lymphomatoid Papulosis/diagnosis , Skin Neoplasms/diagnosis , Skin/immunology , T-Lymphocytes, Cytotoxic/immunology , Biopsy , Diagnosis, Differential , Drug Substitution , Female , Humans , Immunohistochemistry , Immunosuppressive Agents/therapeutic use , Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/pathology , Lymphomatoid Papulosis/drug therapy , Lymphomatoid Papulosis/immunology , Lymphomatoid Papulosis/pathology , Methotrexate/therapeutic use , Middle Aged , Predictive Value of Tests , Skin/drug effects , Skin/pathology , Skin Neoplasms/immunology , Skin Neoplasms/pathology , Steroids/therapeutic use , T-Lymphocytes, Cytotoxic/drug effects , Treatment OutcomeABSTRACT
INTRODUCTION: Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised cutaneous amyloidosis (PLCA). The method of treatment was compared to the risk of local recurrence. METHODS: A literature search produced 77 articles with localised cutaneous amyloidosis, 23 articles were excluded; thus, a total of 54 articles were included. RESULTS: A total of 94 patients were included with a male:female ratio of 1.2:1.0. The median age was 57 years (range 24-87 years). The most common tumour localisation was in the head and neck region with a total of 38 lesions (34%), and 20 patients (22%) had two or more lesions in different locations. The nodular subtype was reported in 65 patients (69%). Only 29 patients received therapy with eight patients having two or more treatments (28%). Eight patients (9%) had local recurrence and all were nodular PLCA, which were mainly seen in males and localised in the face. One patient developed SA (1%); in fact, this was the only patient who was positive for monoclonal amyloid light chain amyloidosis by immunoelectrophoresis of the serum. CONCLUSION: Our review suggests that PLCA is a benign disease that has a good prognosis and that it is associated with a low risk of developing SA (1%). The risk of developing local recurrence or developing new lesions was 9%, and no significant differences were found when compared to the primary treatment.
