Multiple dermatofibromas is a rare entity consisting of more than fifteen lesions. Multiple clustered dermatofibroma is a distinct variant of multiple dermatofibromas and is defined as a well-demarcated plaque composed of individual dermatofibromas. We report a 16-year-old boy with multiple clustered dermatofibroma in a segmental distribution, which has previously not been reported in the literature.
Primary cutaneous lymphomas represent a heterogeneous group of T‐ and B‐cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T‐cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following (http://www.australasianlymphomaalliance.org.au).
Terminal osseous dysplasia with pigmentary defects (TOD) is an extremely rare X-linked dominant disorder, which is characterised by cutaneous digital fibromas, pigmentary skin defects and skeletal abnormalities. A single mutation in the last nucleotide of exon 31 of the filamin A gene (FLNA) has recently been identified as a cause of the disease. We describe a case of an 18-month-old girl with the clinical phenotype of TOD and the disease-specific FLNA mutation confirmed by genetic testing. This report highlights the importance of recognising this distinct phenotype that can present to a wide variety of health-care professionals, and reviews the spectrum of filamin A disorders.
A 24-year-old man with a long history of severe atopic eczema presented with a marked exacerbation requiring hospital admission. It emerged that his occupation as an animal house technician required him to work closely with laboratory animals, particularly mice and rats. Radioallergosorbent tests to mice allergens were markedly elevated. Avoidance of animal work, in conjunction with medical treatment, resulted in a marked improvement of his eczema.
We read with interest the recent article 'IgG4-related disease with cutaneous manifestations treated with rituximab: Case report and literature review 1 ' in Australian Journal of Dermatology and would like to report a case of extensive immunoglobulin type gamma 4 (IgG4)-related skin disease which responded extremely well to prednisolone and azathioprine.A 61-year-old woman presented at our clinic with a 3-year history of a large erythematous, nodular plaque on the left cheek ( Fig. 1a). Six months previously, similar lesions had developed on her forehead. Eight years prior, she had undergone a left parotidectomy and the histology revealed a non-specific lymphocytic infiltrate.A skin biopsy of the left cheek plaque demonstrated an inflammatory infiltrate of CD20-positive B-cells and CD4predominant T-cells, with a large number of IgG4 positive plasma cells. The parotid gland was re-examined, and histology demonstrated marked lymphoid hyperplasia with prominent germinal centres, polyclonal plasma cells and focal granulomas. Her serum IgG4 level was elevated at 2.24 g/L (0.03-2.01 g/L). A full body CT scan showed no systemic involvement. She was commenced on prednisolone and azathioprine with significant amelioration of the plaques (Fig. 1b).IgG4-related disease is a newly described systemic, autoimmune, fibro-inflammatory condition that can present with cutaneous features, with recent reports demonstrating the effectiveness of multiple agents including oral steroids, mycophenolate mofetil, azathioprine, methotrexate, thalidomide and rituximab. 1,2,3,4,5 Our case complements that of Jalilian and colleagues and again demonstrates a late diagnosis after a previous parotidectomy, possibly because the condition was not well described when the patient initially presented.
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