VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) for the dermatologist.

In 2020, Beck et al1 described a novel adult autoinflammatory syndrome entitled VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic), a newly-discovered disorder that connected previously unrelated inflammatory syndromes and a prototype for a new class of hematoinflammatory diseases.2 Eighty-nine percent of published cases have documented skin involvement, but despite the high incidence and diagnostic accessibility of skin manifestations, there has been little focus on the dermatological features of VEXAS syndrome thus far. A PubMed search of all published case reports of VEXAS syndrome to date was performed, with inclusion of all cases confirmed by genetic sequencing, and this review summarizes the reported dermatological signs. There have already been 141 confirmed published cases since original publication, 126 of which had documented cutaneous signs.1-34 A wide range of skin presentations are reported, including Sweet-like urticated and tender erythematous nodules, cartilaginous involvement with chondritis, cutaneous vasculitis, and periorbital angiodema.1-34 Many patients had been diagnosed with Sweet syndrome, relapsing polychondritis, polyarteritis nodosa, or erythema nodosum.1-34 Hallmarks of skin histopathology are a neutrophilic dermatosis with coexisting or exclusive leukocytoclastic vasculitis.1 The new classification therefore helps link previously disparate inflammatory skin conditions into a unifying pathophysiological pathway.

Multiparametric Sonography of Hematologic Malignancies of the Testis: Grayscale, Color Doppler, and Contrast-Enhanced Ultrasound and Strain Elastographic Appearances With Histologic Correlation.

Primary testicular lymphoma is rare and appears with nonspecific findings on grayscale and color Doppler sonography. We present 8 patients further examined with contrast-enhanced sonography, strain elastography, and histologic analysis after orchiectomy. Seven of 8 patients had a diagnosis of large B-cell lymphoma, and 1 of 8 had a diagnosis of granulocytic sarcoma, with solitary lesions (2 of 8), multiple lesions (3 of 8), or entire testicular involvement (3 of 8). Lesions appeared hypoechoic (7 of 8) or isoechoic (1 of 8), all with increased vascularity on color Doppler sonography and a nonbranching linear pattern of intratumoral vessels (7 of 8). Contrast-enhanced ultrasound (CEUS) confirmed this pattern and showed increased enhancement in all lesions. On strain elastography, all lesions were hard, with an elasticity score of greater than 4. Multiparametric sonography of testicular lymphoma identifies increased vascularity on color Doppler and contrast-enhanced ultrasound and increased lesion stiffness on strain elastography.

Multiparametric sonographic imaging of a capillary hemangioma of the testis: appearances on gray-scale, color Doppler, contrast-enhanced ultrasound and strain elastography.

We report a case of a lobular capillary hemangioma in a 66-year-old man, who presented with left testicular pain, with an asymptomatic incidental right testicular lesion found on ultrasonography. The sonographic examination demonstrated a heterogeneous mainly iso-echoic intratesticular lesion with marked vascularity on the color Doppler examination. Further evaluation with contrast-enhanced ultrasound and strain elastography was performed; the multiparametric imaging suggested a benign tumor. The multidisciplinary team decision with patient consent was to perform a radical orchiectomy with subsequent histopathology confirming a benign lobular capillary hemangioma.

Accuracy of classification of invasive lobular carcinoma on needle core biopsy of the breast.

Although the UK National Institute for Health and Care Excellence guidelines recommend that in patients with biopsy-proven invasive lobular carcinoma (ILC), preoperative MRI scan is considered, the accuracy of diagnosis of ILC in core biopsy of the breast has not been previously investigated. Eleven pathology laboratories from the UK and Ireland submitted data on 1112 cases interpreted as showing features of ILC, or mixed ILC and IDC/no special type (NST)/other tumour type, on needle core biopsy through retrieval of histology reports. Of the total 1112 cases, 844 were shown to be pure ILC on surgical excision, 154 were mixed ILC plus another type (invariably ductal/NST) and 113 were shown to be ductal/NST. Of those lesions categorised as pure ILC on core, 93% had an element of ILC correctly identified in the core biopsy sample and could be considered concordant. Of cores diagnosed as mixed ILC plus another type on core, complete agreement between core and excision was 46%, with 27% cases of pure ILC, whilst 26% non-concordant. These data indicate that there is not a large excess of expensive MRIs being performed as a result of miscategorisation histologically.

Invasive Lobular Carcinoma of the Breast.

Invasive lobular carcinoma of the breast is a distinct histologic type with specific clinical, radiologic, and microscopic features, which is increasing in incidence in the Western World. Routine pathologic prognostic factors, including grade, lymph node stage, and size are of significance in predicting outcome in this group of patients who, overall, have survival comparable to those with other subtypes of breast cancer. Variants, however, are seen and these, as well as other standard factors such as histologic grade, have prognostic significance and should be recorded in pathology reports.