To the Editor: Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis occurring in childhood. This entity is rare in adults. Clinically, it typically presents as asymptomatic solitary or multiple yellow-orange-brown firm papules or nodules, most commonly affecting the face, neck, and upper trunk. Most often, cutaneous JXG spontaneously involutes over several years. Extracutaneous sites have also been described, including oral, ocular, and lung lesions.1 Cutaneous JXG has only rarely been associated with dermal elastic fiber abnormalities, with only rare reports describing secondary anetoderma occurring after JXG resolution.2,3 In this article, we present a case of JXG exhibiting prominent elastophagocytosis on microscopy. The patient was a 9-year-old girl, with no significant medical history. She presented with an asymptomatic lesion of a few months' duration over the left axilla, consisting of a firm, rubbery erythematous yellowish slightly pedunculated papule (Fig. 1). The differential diagnosis included JXG, skin tag, nevus, and neurofibroma. Shave biopsy of the lesion revealed a well-circumscribed nodule composed of a dense dermal histiocytic proliferation, with numerous foamy giant cells, multinucleated giant cells of the Touton type, and scattered lymphocytes and eosinophils (Figs. 2A, B). Interestingly, prominent elastophagocytosis was also noted within the lesion (Fig. 2B), a finding also highlighted using elastic stain (Fig. 2C).FIGURE 1.: JXG presenting as a yellowish papule on the left axilla.FIGURE 2.: Histopathological evaluation showed classical JXG features with prominent elastophagocytosis (arrows) (hematoxylin and eosin, A ×40, B ×200); (C) elastic stain highlighted the elastophagocytosis (arrows) (×400).Elastophagocytosis is the phagocytosis of normal or degenerated elastic fibers by histiocytes, multinucleated giant cells, or both.4 These engulfed elastic fibers are typically seen in the cytoplasm of these cells. Elastophagocytosis can be primary or secondary with multiple underling theories.4 First, activated macrophages may phagocytose ultrastructurally normal elastic fibers leading to their degradation. Furthermore, secondary alteration of ultrastructurally normal elastic fibers may activate macrophages leading to elastophagocytosis. Alternatively, primary alteration of elastic fibers may lead to activation of macrophages leading to elastophagocytosis. It is important to note elastic fiber degradation does not necessarily lead to elastophagocytosis. An example of this is photoaged skin characterized by degenerated elastic fibers but rarely associated with elastophagocytosis.4 Elastophagocytosis has been reported in many conditions, including elastolytic (such as middermal elastolysis), inflammatory (such as lichen sclerosis and noninfectious granulomatous entities), infectious (such as leprosy), and neoplastic (such as granulomatous slack skin) conditions, among others.4,5 Among the histiocytic disorders, elastophagocytosis was rarely reported in Rosai–Dorfman disease and xanthoma disseminatum but not JXG.6,7 However, the mechanisms leading to elastophagocytosis in these histiocytic disorders have not been studied. Only rarely has cutaneous JXG been associated with dermal elastic fiber abnormalities. Anecdotal reports described secondary anetoderma occurring after JXG resolution.2,3 Anetoderma can be primary or secondary and is characterized by well-defined circular/oval macules that are bulging outward or slightly depressed. Classic pathologic findings include decreased dermal elastic fibers with shortening and fragmentation. The pathogenesis of anetoderma is still unknown; however, underlying pathologic mechanisms include inflammatory cells releasing elastolytic enzymes that lead to elastic fiber degradation and elastophagocytosis.2,3,8 Thus, linking our finding of elastophagocytosis in JXG and the reported secondary anetoderma post-JXG resolution allows us to postulate that elastophagocytosis may contribute to elastic fiber degeneration and loss in such cases, although other factors such as enzymes released by the neoplastic cells may also have a role. This needs to be clarified in future studies.
Livedoid vasculopathy (LV) is a chronic, recurrent, painful cutaneous disease manifesting as longstanding distal lower extremity ulcers that scar leaving stellate atrophic lesions known as ‘atrophie blanche’. A significant number of cases have been associated with thrombophilic abnormalities. In this study, we describe, to the best of our knowledge, the first report of LV only associated with sickle cell trait with significant improvement on aspirin.
Introduction: Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues. Anti-U1RNP myositis is a newly described entity characterized by myositis, arthritis, interstitial lung disease, and Raynaud phenomenon. We present a case of a unique combination of deep morphea in a patient with anti-U1RNP myositis. Case presentation: A 64-year-old male with 5-year history of proximal muscle weakness, polyarthritis, Raynaud phenomenon, and dyspnea on multiple immunosuppressives presented with localized infiltrated, tight, and hyperpigmented plaques over the posterior thighs and mid-to-lower back developing over the last 2 years and limiting his movement. Autoimmune workup revealed a positive ANA, anti-U1RNP antibody, anti-Jo1 antibody, and anti-Ro52 antibody. Further workup showed restrictive lung disease, kidney disease, and arthritis. Patient was diagnosed with anti-U1RNP myositis. Skin biopsy of the back lesion showed deep morphea. Discussion: Association of deep morphea with anti-U1RNP myositis is not described prior in the literature. Treatment of morphea is challenging since the patient is already on immunosuppressive medications. The patient failed methotrexate prior and is currently on Mycophenolate mofetil and Deflazacort which are reported as potential treatment for morphea. Therefore, physical therapy plus topical Tacrolimus were suggested as an initial measure to preserve the range of motion of his posterior thighs and back. This is a case of progressive deep morphea developing in a patient with a unique autoimmune profile on immunosuppressive drugs. Conclusion: Anti-U1RNP myositis is a challenging diagnosis and should be always thought of in patients with positive anti-U1RNP, myositis, interstitial lung disease, arthritis, kidney disease, and Raynaud phenomenon. Moreover, deep morphea treatment in immunosuppressed patients is challenging and different measures should be considered.
A number of cutaneous manifestations and adverse cutaneous reactions have been associated with COVID-19 infection and vaccine.A Lebanese national registry was established to characterize the dermatologic manifestations and adverse cutaneous reactions associated with COVID-19 infection and vaccination in a sample of the Lebanese population.An observational cross-sectional study was conducted via a web-based clinical form distributed to physicians wishing to report their cases from May 2021 till May 2022.In total, 142 patients were entered in the registry, of which 133 were adults and nine were pediatric patients. The main dermatological manifestations reported with COVID-19 infection in the adult group were urticaria (32.9%), telogen effluvium (21.4%), morbilliform (10%), and papulosquamous (8.6%) eruptions. Urticaria was the most common adverse cutaneous reaction to the vaccine (33%). Interestingly, herpes zoster was triggered in 12 patients post vaccination in our series with this finding more frequently seen in patients above the age of 41 (P = 0.013). In the pediatric group, the most reported dermatological findings associated with COVID-19 infection were malar erythema (25%) and telogen effluvium (25%). One 16-year-old patient developed lichen planus after one dose of a COVID-19 vaccine. No deaths were reported in both age groups.This Lebanese registry adds more robust evidence that clinical manifestations of the COVID-19 virus and vaccine are diverse. More studies are necessary to establish the pathophysiology of these dermatological findings in the context of COVID-19 infection and vaccination.
Cutaneous metastasis is the result of malignant cell spread from primary malignancy to the skin. This is not uncommon, and rates reported in the literature are as high as 10.4%. To the best of our knowledge, there are no studies assessing the epidemiologic, clinical, and histopathological features of cutaneous metastasis in our region.To assess the clinical and histopathological findings of all patients diagnosed with cutaneous metastasis at the American University of Beirut - Medical Center (AUB-MC) and to compare our findings with those published in the literature.Retrospective clinical and histopathologic evaluation of all cases diagnosed as cutaneous metastasis at AUB-MC between 1992 and 2010.A total of 72 patients (50 females and 22 males) were identified. The mean age at diagnosis was 55.2 years. The most common primary cancer was breast cancer in women and laryngeal cancer in men. The most common clinical presentation was a single nodule in 27% of cases followed by multiple nodules in 23%. Cutaneous metastasis lesions were asymptomatic in the majority. The chest was the most commonly affected site. On microscopy, the majority of metastatic cases were adenocarcinomas (74%).This is, to our knowledge, the first study characterizing the epidemiological, clinical, and histopathological features of cutaneous metastasis in the Lebanese population. The clinical and histopathological features observed were in concordance with the published literature, with minor differences.
