RESUMEN
(1) Background: Various cutaneous adverse drug reactions (ADRs) are observed with the implementation of mRNA COVID-19 vaccines. To gain insight into the clinicopathologic features, we analyzed the correlation of histological and clinical data in 48 patients with these ADRs. (2) Methods: Single-center retrospective study in patients with ADRs after mRNA COVID-19 vaccination (mRNA-1273 and BNT162b2 vaccines). (3) Results: Distant generalized ADRs prevailed (91%), often appearing clinically as spongiotic dermatitis or maculopapular exanthema. Histopathological analysis revealed spongiotic changes (46%) and dermal superficial perivascular predominantly lymphocytic infiltrates (17%). Eosinophils were found in 66% of biopsies, neutrophils in 29%, and plasma cells only in 8% of biopsies. Most ADRs occurred after the second vaccine dose (44%). Histologically spongiotic changes were associated with clinical features of spongiotic dermatitis in only 50% of patients and maculopapular exanthema in the remaining patients. ADRs represented an aggravation of preexisting skin disease in 23% of patients. ADRs regressed within 28 days or less in 53% of patients and persisted beyond a month in the remaining patients. (4) Conclusions: Our study demonstrates a diverse spectrum of generalized ADRs, revealing correlations between histology and clinical features but also instances of divergence. Interestingly, in about half of our patients, ADRs were self-limited, whereas ADRs extended beyond a month in the other half.
RESUMEN
Diffuse dermal angiomatosis (DDA) is a rare reactive angioproliferation in the skin and considered to be a subtype in the group of cutaneous reactive angiomatoses. DDA is clinically characterized by livedoid patches and plaques with tender ulceration. Its histologic features are a reactive diffuse proliferation of bland endothelial cells and pericytes within the dermis, forming small capillary vessels. Previously described cases of DDA most commonly involved the limbs and were associated with a wide spectrum of predisposing comorbidities, especially advanced atherosclerotic vascular disease and arteriovenous fistula. However, several cases of DDA of the breast (DDAB) have been reported in recent years. In this study we present 2 additional patients with DDAB and review all 36 cases of DDAB published in the literature. We describe the clinical and histopathologic characteristics, hypothesized pathogenetic mechanisms, and predisposing conditions of this rare skin disorder and discuss treatment options. The breast is a more commonly involved site of DDA than previously believed. DDAB typically occurs in middle-aged women and is associated with macromastia, overweight or obesity, and probably smoking. Predisposing comorbid conditions differ from those of DDA involving other parts of the body, making DDAB a unique clinicopathologic entity in the spectrum of cutaneous reactive angiomatoses. Currently there is no consensus on the best therapeutic approach. Isotretinoin and other medical therapies have been used with limited success. Breast reduction surgery appears to be a viable treatment option for DDAB in women with macromastia and might provide definitive healing.
RESUMEN
Buschke-Ollendorff syndrome refers to the concomitant occurrence of connective tissue nevi, composed of elastic fibers in most cases, with osteopoikilosis. This autosomal dominant inherited disorder is caused by mutations in the gene LEMD3 on chromosome 12q14, which induces a rather heterogeneous clinical phenotype. Here, we report on the most proximal germline mutation found to date in the LEMD3 gene, p.Val94fs, in a three-generation Swiss family. Quantitative RNA analyses in affected and non-affected skin tissue from the proband demonstrate a comparable nonsense-mediated decay of mutant LEMD3 mRNA in both tissues; however, different levels of tropoelastin expression suggest that additional factors are involved in the development of the cutaneous lesions.
