Acrokeratosis of Bazex as a sign of thyroid cancer: first description and review of thyroid-associated paraneoplastic dermatoses.

OBJECTIVE: Bazex syndrome is a rare paraneoplastic skin disorder of unknown pathogenesis. Cutaneous findings are usually noticed before the diagnosis of the underlying malignancy, more frequently squamous cell carcinomas of the upper aerodigestive tract or metastasis to cervical lymph nodes. Association with other malignancies has been reported. CASE REPORT: Herein, we describe a case in course of metastatic papillary thyroid carcinoma and review the relevant literature. RESULTS: A bibliographic search was conducted and a total of 8 studies concerning the association were reviewed. CONCLUSIONS: Physicians be aware of unexpected cutaneous conditions as a possible sign of underlying tumors.

Ulcerative necrobiosis lipoidica: case report of an atypical presentation and literature review.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous degenerative skin disease by unknown causes, which is mostly associated with diabetes mellitus, usually presenting with typical plaques of the shins. Although less common, some ulcerative forms may be seen in clinical practice. The occurrence of an atypical presentation in one of our patients was the occasion to review the pertinent literature.

Primary melanoma of the bladder: a case report and review of the literature.

OBJECTIVE: Primary bladder melanomas are rare and aggressive neoplasms. We herein described a new case and performed a review of the literature. PATIENTS AND METHODS: We present the case of a 81-year-old woman with a primary mucosal melanoma of the bladder after a history of acral melanoma (KRAS mutated) and lentigo maligna of the forehead. Using PubMed, we found that in literature 38 cases were described. RESULTS: In our patients, during a transurethral resection (TURBT), two bladder lesions were detected. The histologic exam revealed a malignant melanoma, Mib1/ki67: 10-12%, PDL1 <1%. No BRAF, NRAS or KRAS mutations were detected. She subsequently underwent a transurethral revision of the trigone and a partial cystectomy of the dome with bilateral pelvic lymph node dissection. Microscopical findings showed a residual 5 mm non-muscle-invasive melanoma of the bladder, with negativity of the surgical margins and of the 17 pelvic lymph nodes. No adjuvant treatment was proposed. To date the patient is disease-free. CONCLUSIONS: Primary bladder melanoma carries a poor prognosis and poses a therapeutic challenge to clinicians who manage patients with this rare condition. In our experience the multidisciplinary approach for the diagnosis and management of this rare cancer is mandatory.

Radiotherapy in cancer and rheumathoid arthritis patients: cancer treatment or control of articular flares? We can achieve both.

OBJECTIVE: The study was aimed to investigate the role of radiotherapy (RT) as a risk factor for reactivation or worsening of symptoms in patients affected by rheumatoid arthritis (RA) PATIENTS AND METHODS: This is a single-center retrospective observational study on RA patients who developed cancer requiring RT during the course of the disease. The control group consisted of RA patients with cancer who did not undergo RT. In both groups, the disease activity was evaluated at baseline and at 6 and 12 months through the DAS28 index. A relapse was defined as an increase of >20% in DAS28. A radiotherapist evaluated total and daily doses and timing of radiation. Acute and late toxicity was defined as events occurring within 90 days from the start and more than 90 days after the completion of RT, respectively. RESULTS: Seventy-two RA patients (38F/34M; mean age: 70±9 years; mean disease duration: 13±9 years), 29 (40.2%) of whom received radiotherapy (mean age 72.9±9 years), were enrolled. The most frequent malignancies were breast (27.2%), thyroid (9.8%), and skin (7%). Between radio-treated and non-radio-treated patients, no significant differences in RA reactivation (6/29 vs. 17/43; p=0.12) or mean exacerbation time (6.7 ± 4.9 months compared to 6.4 ± 4.1 months; p=0.78) were found. Overall, RT was well tolerated with low rates of both acute and late toxicity. CONCLUSIONS: In RA patients, RT was well tolerated and not associated with an increased risk of articular flares. Properly designed prospective clinical studies with a larger number of patients should be performed to confirm these data.

Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Literature up to September 2020 shows that clear evidence exists only for some of the mentioned associations, while in the majority of cases, data appear contrasting. The aim of this review is to summarize the available studies on the topic and draw possible conclusions. Further clinical and laboratory studies are needed to assess the real plausibility and relevance of these associations, as well as the possible role of Helicobacter pylori with the underlying pathogenic mechanisms.

Possible role of Helicobacter pylori in diseases of dermatological interest.

Helicobacter pylori is a gram-negative, flagellate, microaerophilic bacterium identified for the first time about 30 years ago, as a pathogenic factor of gastritis and peptic ulcer. Soon after, it was linked to several gastrointestinal and extra-gastrointestinal diseases (hematological, cardiovascular, neurological, pulmonary and ocular diseases, obesity, diabetes mellitus, growth retardation and extragastric MALT lymphoma). Association and possible cause-effect correlation with H. pylori infection were suggested in diseases of dermatological interest such as chronic urticaria, rosacea, Henoch-Schoenleins purpura, idiopathic thrombocytopenic purpura, cutaneous and oral lichen planus, atopic dermatitis, recurrent aphthous stomatitis, systemic sclerosis, psoriasis, Sjögrens syndrome, Behçet's disease, pruritus, alopecia areata, primary cutaneous marginal zone B-cell lymphomas, vitiligo, chronic prurigo, multiformis, prurigo nodularis, leukocytoclastic vasculitis, prurigo pigmentosa, eczema nummulare, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, Sweet's syndrome, cutaneous T-cell pseudolymphoma and pemphigus vulgaris. A critical review of the literature up to May 2017 shows clear evidence of H. pylori involvement only for some of the above purported associations, while in the majority of cases data appear contrasting and/or obtained on a not adequately large study population. Further clinical and laboratory research, with more adequate methodological and statistical basis, is required to assess the actual existence and relevance of many purported associations, as well as the possible role of H. pylori and the underlying pathogenic mechanisms.

Hard to face: cutaneous malignant melanoma.

An 88-year-old Caucasian man recently sought medical attention due to recrudescence of skin disease in the area of previously excised nodule (in 2002) on the nose dorsum. The original lesion was clinically considered as a pigmented basal cell carcinoma, after which histological examination revealed a superficial spreading melanoma in vertical growth face (Clark level IV; Breslow thickness 2.1 mm) arising from a pre-existing nevus. The adjacent skin also showed significant actinic damage both in the epidermis and in the dermis. A sentinel node was positive (micrometastases of melanoma) and radical lymph node dissection of the neck was performed. Other lymph nodes did not contain metastasis. Computed tomography scans of brain, chest, abdomen and pelvis performed at that time showed no evidence of systemic disease.

Laser treatment of post-thyroidectomy scar.

A good aesthetic result in thyroid surgery is one of the main goals, as this procedure usually involves young women affected in clearly visible anatomical areas. To minimize the detrimental effects of scarring outcome, several therapeutic options have been employed. Lasers may be an alternative choice for prevention and treatment of post-surgical thyroidectomy scar. This paper reviews literature and the current knowledge on this topic. A comprehensive search in the Cochrane Library, MEDLINE and PUBMED databases was performed to identify relevant literature investigating the role of laser therapy in both prevention and treatment of unappealing scarring after thyroid surgery. Laser treatment of post-thyroidectomy scar is emerging with promising clinical outcomes. The greatest efficacy has been seen with vascular-selective and ablative sources. Laser therapy should be taken into account as it represents a valid and safe treatment option.

Pilomatrixoma of the breast in a patient with type 1 myotonic dystrophy: successful surgical approach.

Malherbe’s calcifying epithelioma is an uncommon cutaneous tumour that originates from the matrix cells of hair follicle. It was initially described by Malherbe as a benign calcifying epithelioma. Several ultra-structural and electron-microscopic studies later demonstrated its origin from matrix cells and the term pilomatrixoma was introduced. The treatment of this tumour remains mainly surgical. Malignant cases with post-surgical recurrences have been described in literature and recurrences have been related to an incomplete surgical treatment or tumour aggressiveness. We present the case of 31-year-old female patient with pilomatrixoma of the breast, which was very similar to fibroadenoma, in terms of size and other clinical features. We successfully treated this patient surgically, and the aesthetic results were good, despite the proximity of the tumour to the areola-nipple complex. Fifteen months later, the patient is doing well, free of any clinical local recurrence.

Langerhans cell sarcoma: an unusual microscopic presentation.

A 70-year-old Caucasian male presented to our clinic for a pruritic eruption progressing over several months. He complained of fatigue with a 20-pound weight loss over the past year. On presentation, the patient had browny-yellow to violaceous, purpuric, macular and papular lesions on the legs, arms, lower abdomen and back. Initial biopsy showed an angiocentric infiltrate with a suggestion of intraluminal proliferation; CD31 and Fli-1 positivity suggested either reactive angioendotheliomatosis or an unusual intravascular histiocytosis. Further excisional biopsies demonstrated perivascular collections of cells with ample cytoplasm, prominent nuclear pleomorphism and mitotic activity. The nuclei demonstrated nuclear folding, grooves and indentations. The atypical cells were S100, CD1a and CD56 positive with immunohistochemistry. A diagnosis of Langerhans cell sarcoma (LCS) was made. LCS is a rare, aggressive malignancy that can involve multiple organs including the skin, lymph nodes, lung, bone marrow, spleen, heart, and brain. The skin and lymph nodes are commonly involved, and the cutaneous presentation varies greatly. Immunohistochemistry characteristically shows CD1a and S100 positivity. CD56 expression is uncommon and often portends a poor prognosis. There is no established treatment of LCS due to its rarity. Surgery, radiation, and chemotherapy have been used with varied outcomes. Our patient was treated with prednisone with improvement of cutaneous disease. He did not develop systemic involvement, but died 1.5 years later from complications associated with heart failure. Langerhans cell sarcoma should be considered when faced with an unusual angiocentric infiltrate in which initial immunohistochemical staining results may be misleading.