Complete skin remission of Sneddon-Wilkinson disease with acalabrutinib.

Sneddon-Wilkinson (SW) disease is rare condition which typically occurs in the context of a monoclonal immunoglobulin and treatment options have not been well studied. Here we present a case of a 65-year-old female with SW with underlying, otherwise indolent, lymphoplasmacytic lymphoma (LPL) with an IgA paraprotein, who achieved a complete skin remission with the administration of the Bruton Tyrosine Kinase inhibitor (BTKi), acalabrutinib.

CD30-positive lymphoproliferative disorders-An Australian Clinical Practice Statement from the Peter MacCallum Cancer Centre.

The CD30-postive lymphoproliferative disorders, including lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, account for up to 30% of all cutaneous T-cell lymphomas (CTCLs) and are the second most common form of CTCLs after mycosis fungoides. Both conditions differ in their clinical presentations; however, they share the expression of the CD30 antigen as a common immunophenotypic hallmark. There is a wide spectrum of management options depending on factors such as extent of disease, staging and treatment tolerability.

Excellent treatment outcomes from low dose radiation therapy for primary cutaneous CD4+ small/medium T-Cell lymphoproliferative disorder.

Background And Purpose: Primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder (PCSMLPD) is a benign behaving condition, typically manifesting as solitary head or neck papules, frequently creating cosmetic concerns. Optimal management of this rare disease is unclear. Herein, patterns of care and treatment outcomes are described, with particular focus on low-dose RT.

Mycosis fungoides and Sézary syndrome: Australian clinical practice statement.

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.

Terminal osseous dysplasia with pigmentary defects; Case and brief review of filamin A-related disorders.

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.

Multiple clustered dermatofibroma presenting in a segmental distribution.

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.

Occupational exposure to laboratory animals causing a severe exacerbation of atopic eczema.

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.