Rapidly Invasive Buschke-Löwenstein Tumor Associated With Human Papillomavirus Types 6 and 52.

Buschke-Löwenstein tumor, or giant condyloma acuminatum, represents a rare, sexually transmitted disorder, with a slow evolution and the tendency to infiltrate in the adjacent tissues associated with human papillomavirus (HPV). This article reports the first case of male Buschke-Löwenstein tumor associated with HPV6 and HPV52.

Familial papular epidermal nevus with "skyline" basal cell layer.

Papular epidermal nevus with "skyline" basal cell layer (PENS), a novel keratinocytic nevus, has recently been described as a mosaic condition with varying presentations. We herein describe typical PENS lesions, which usually occur sporadically, affecting two members of the same family. The concept of paradominant inheritance is proposed to explain the paradox of occasional transmission of normally sporadically occurring traits.

Pityriasis rosea and pityriasis rosea-like eruption after anti-SARS-CoV-2 vaccination: a report of five cases and review of the literature.

Only a few cases of pityriasis rosea (PR)/pityriasis rosea-like eruption (PRLE) after anti-SARS-CoV-2 vaccination have been reported. In the period May 2021- February 2022 we observed five cases of clinically typical PR that appeared 2 to 3 weeks after anti-SARS-CoV-2 vaccination with BNT162b2 (3 patients) or mRNA- 1273 (2 patients). In 4 patients PR appeared after the first vaccination; in one patient after the second one. In 3 patients a biopsy for histopathological examinations was carried out. Results were typical for PR. In all patients laboratory examinations were within normal ranges. All patients were treated with cetirizine. Complete remission was observed within 14-30 days. Four patients were subjected to the second vaccination, but no skin lesions appeared. All patients are currently in good general health. It is possible that a relationship between anti- Sars-CoV-2 vaccination and PR/PRLE exists; however, it is very rare, in consideration of millions of vaccinated subjects and the low number of reported cases of PR/PRLE. The pathogenesis of this relationship is unknown. However, some hypotheses may be advanced: PR/PRLE following anti-Sars-CoV-2 vaccination may be just a coincidence; anti-Sars-CoV-2 vaccines cause a reactivation of HHV-6 and/or HHV-7; vaccines can induce a delayed hypersensitivity response clinically similar to drug-induced PRLE.

Diffuse normolipemic plane xanthoma in a child with common variable immunodeficiency.

Diffuse plane xanthoma is extremely rare in children. Although it may be associated with systemic disorders, its etiology remains obscure in a number of patients. The case of a boy with common variable immunodeficiency and normal serum lipid levels, who developed diffuse plane xanthoma during treatment with intravenous immunoglobulins, is reported.

Granulomatous tattoo reaction induced by intense pulse light treatment.

Cosmetic tattooing involves implantation of pigments into the dermis in order to create a permanent makeup. Here, we report a case of sarcoidal granulomatous reaction to old cosmetic tattoos after an intense pulsed light (IPL) treatment for facial skin rejuvenation. We consider this case as a peculiar example of photo-induced reaction to tattoo. In addition, we hypothesize that an underlying immune dysfunction was present, and acted as a predisposing factor for this unusual response, as the patient had suffered from an episode of acute pulmonary sarcoidosis 15 years before. Overall, our observation suggests that IPL treatment should be used cautiously in patients with tattoos, especially when a history of autoimmune disease is present.

"Hairy" granular cell tumor.

The occurrence of a solitary subcutaneous granular cell tumor characterized by overlying hypertrichosis in a child is reported. Granular cell tumor should be included in the differential diagnosis of cutaneous neoplasms associated hypertrichosis in the pediatric patient.

Primary giant cell tumor of soft tissue of the groin - a case of 46 years duration.

BACKGROUND: Soft tissue giant cell tumor (GCT-ST) of low malignant potential is an uncommon neoplasm, considered the soft tissue counterpart of giant cell tumor of bone. GCT-ST mainly affects young to middle-age adults and presents as a painless growing mass mainly located in the lower extremities and trunk. Histologically, this tumor is characterized by a mixture of uniformly scattered osteoclast-like multinucleated giant cells intimately admixed with short fascicles of spindled cells. Complete excision with negative surgical margins is associated with a benign clinical course in most cases. METHODS: The authors report the clinicopathological and immunohistochemical features of an unusual GCT-ST of 46 years duration previously histologically misdiagnosed as Kaposi's sarcoma. RESULTS: Histologically, the tumor was characterized by a multinodular growth pattern with osteoclast-like multinucleated giant cells admixed with spindle cells partially arranged in a storiform pattern, fibrosis and foci of haemorrhage and mature bone. Immunohistochemistry revealed CD68 reactivity of the multinucleated giant cells. CONCLUSION: GCT-ST is a rare neoplasm characterized by benign clinical course if excised adequately, as shown by our case of exceptionally long duration. Emphasis is placed on the importance of differential diagnosis with other giant cell-rich soft tissue neoplasms because clinical behaviour, prognosis and treatment significantly differ.

Treatment of classic Kaposi's sarcoma-associated lymphedema with elastic stockings.

Lymphedema of the lower extremities is a frequent complication of Kaposi's sarcoma (KS). Compressive therapy is the basis of treatment for lymphatic disorders, but to the authors' knowledge, there are no controlled trials to evaluate its effectiveness in KS-related lymphedema. Sixty-five patients with classic KS-associated lymphedema limited to below the knee were studied. Fifty patients received below-knee elastic stockings, whereas the remaining 15 did not use any compressive device. Among treated patients, 60% (30/50) experienced a limb volume reduction, while 40% (20/50) had an increase of limb volume. In contrast, all patients (15/15) of the untreated group had an increase of limb volume. No correlation between lymphedema reduction and systemic or local chemotherapy was observed, supporting compressive therapy as the major strategy for the treatment of this condition. Our results suggest that elastic stockings may be important tools for the management of lymphedema associated to classic KS.

A Second Case of Gobello Nevus Syndrome.

An uncommon type of epidermal nevus characterized by hyperpigmented hyperkeratotic bands following a Blaschko-linear pattern and generalized follicular hyperkeratosis were observed in a 17-year-old male patient who additionally showed tufted hair folliculitis on the scalp and clinodactyly of the fifth finger of both hands. The combination of epidermal nevus with skeletal abnormalities was first described by Gobello et al. [Dermatology 2000;201:51-55] as a new epidermal nevus syndrome that was named after the first author of this work. Our case shows identical clinical and histopathological features and represents the second case of this rare syndrome reported in the literature.

Staging of classic Kaposi's sarcoma: a useful tool for therapeutic choices.

Three hundred patients with classic Kaposi's sarcoma (CKS) have attended our Department of Dermatology over a period of 20 years. Many of them have been treated by systemic chemotherapy with good responses. Due to the highly variable clinical evolution of the disease, it was, however, often difficult for us to decide whether or not to treat elderly patients. We therefore attempted to establish a new staging system based on objective criteria that more closely follow the clinical variability of CKS and make the therapeutic choices easier. The proposed staging system comprises 4 stages, each further divided according to the speed of disease evolution and presence of complications that can severely impair the quality of life. The application of this staging system to our patients has shown that evolution is prevalently slow in the maculo-nodular and infiltrative stages I and II and faster during the florid and disseminated stages III and IV. Complications are mainly present in the rapidly evolving florid and disseminated stages, with visceral involvement in the more aggressive forms. Based on these findings, we are employing systemic therapy in the florid and disseminated stages and in the infiltrative stage only in case of rapidly evolving or slowly evolving but complicated disease.

Chordoma cutis.

We report a case of an 85-year-old white man with a diffuse form of psoriasis, who showed a large asymptomatic subcutaneous tumour in the sacrococcygeal region. On cut section there was a subcutaneous neoplasia with a glistening, friable surface. Histologically, the deep dermis was infiltrated by cords and nests of pleomorphic cells embedded in an abundant mucinous stroma, and characteristic physaliphorous (multivacuolated) cells were observed. The neoplastic cells were immunohistochemically positive for cytokeratins (using CAM 5.2 and AE1/AE3), vimentin, S100 protein, and epithelial membrane antigen (EMA), but negative for carcinoembryonic antigen (CEA). Histological and immunohistochemical findings led us to the diagnosis of classic chordoma. Chordomas are rare, slow growing malignant tumours of the spinal axis originating from remnants of the notochord. Occasionally, a skin lesion is the first sign of a primitive or metastatic chordoma.

ERK-dependent downregulation of the atypical chemokine receptor D6 drives tumor aggressiveness in Kaposi sarcoma.

D6 is an atypical chemokine receptor acting as a decoy and scavenger for inflammatory CC chemokines expressed in lymphatic endothelial cells. Here, we report that D6 is expressed in Kaposi sarcoma (KS), a tumor ontogenetically related to the lymphatic endothelium. Both in human tumors and in an experimental model, D6 expression levels were inversely correlated with tumor aggressiveness and increased infiltration of proangiogenic macrophages. Inhibition of monocyte recruitment reduced the growth of tumors, while adoptive transfer of wild-type, but not CCR2(-/-) macrophages, increased the growth rate of D6-competent neoplasms. In the KS model with the B-Raf V600E-activating mutation, inhibition of B-Raf or the downstream ERK pathway induced D6 expression; in progressing human KS tumors, the activation of ERK correlates with reduced levels of D6 expression. These results indicate that activation of the K-Ras-B-Raf-ERK pathway during KS progression downregulates D6 expression, which unleashes chemokine-mediated macrophage recruitment and their acquisition of an M2-like phenotype supporting angiogenesis and tumor growth. Combined targeting of CCR2 and the ERK pathway should be considered as a therapeutic option for patients with KS.