Michela Tabanelli

Cutaneous granulomatous reaction to injectable hyaluronic acid gel: Another case

We report a case of a granulomatous reaction in the melolabial folds, occurring 10 days after treatment with Restylane®. The patient, who had previously been treated with the same product in the last 2 years without any adverse effect, developed an unusual early fibrotic reaction that we hypothesized related to hypersensitivity after repeated use. The lesions slowly disappeared with topical steroid therapy. An improved knowledge of the modality of these uncommon adverse effects is necessary to assess the long‐term safety and efficacy of this product.

Treatment of refractory pemphigus vulgaris with anti‐CD20 monoclonal antibody (rituximab): Five cases

Background: Pemphigus vulgaris is an autoimmune disease characterized by blisters and widespread erosions, involving skin and mucous membranes, caused by autoantibodies to desmoglein 1 and 3. This pathology is associated with increased morbidity and mortality if untreated. The treatment of pemphigus vulgaris requires multiple immunosuppressive agents, but often it is particularly resistant. Objective: To evaluate the efficacy and safety of rituximab therapy in refractory pemphigus vulgaris. Methods: Five patients diagnosed as having pemphigus vulgaris were treated with anti‐CD20 monoclonal antibody (rituximab). Each patient was treated with rituximab intravenously at a dosage of 375 mg per square metre of body surface area once weekly for 4 weeks. Results: All the patients presented clinical resolution. No adverse effects were observed. It is important to observe the clinical evolution in the future, but our experience is still limited to a short lifetime and follow‐up. Conclusion: In our experience rituximab has been an effective and safe treatment for refractory pemphigus vulgaris.

Acute generalized exanthematous pustulosis induced by pentoxifylline

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Topical corticosteroids versus “wait and see” in the management of solitary mastocytoma in pediatric patients: a long‐term follow‐up

Management of patients affected by mastocytoma (MS) includes avoiding triggering factors of mast cell degranulation, and administration of symptomatic treatment. We evaluated topical steroid treatment efficiency on the clinical course of MS in a group of patients, comparing the results with another untreated group.We retrospectively evaluated clinical data of 176 patients under 15 years of age, affected by MS and referred to our Dermatological Pediatric Service from 1996 to 2010. Ninety‐one of 176 children were treated with topical steroids. Follow‐up was possible in 130 of 176 patients and lasted for 56.3 months on average. We compared 62 treated and 68 untreated patients. There was not statistic difference between the two groups: (i) in the number of healed or partially improved cases; and (ii) in the time of partial regression, although it is quicker with therapy. The time of healing is 16.4 months (on average) with treatment, and 34.7 months (on average, p = 0.001) without any treatment. The resolution of MS is independent of therapy administration, but the time of healing is statistically faster using the local steroids. An appropriate treatment with them is effective and safe, considering the long time needed for spontaneous resolution.

Prurigo nodularis as the first manifestation of a chronic autoimmune cholestatic hepatitis

autoimmune cholestatic hepatitis A 54-year-old white woman was referred to us because of an itching dermatosis. It had started eight months before our first consultation and was characterized by excoriated papules and nodules located on the extensor surfaces of the limbs, abdomen, and back (Fig. 1). The patient already had a histologic examination that was consistent with a prurigo nodularis (PN) (Fig. 2). She had been treated initially with topical and systemic cortico-steroids without improvement, and subsequently with oral cyclosporine, 2.5 mg/kg per day, with very good results. However, when the dose was reduced it led to relapse of the disease. At the beginning of treatment, a limited set of blood tests was performed, including blood urea nitrogen, creatinine, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and full blood count. AST (40 UI/L) and ALT (49 UI/L) were very slightly elevated, and total bilirubin was normal (0.51 mg/dL). We repeated the blood tests and found very high levels of alkaline phosphatase (AF) (1080 UI/L), high levels of gamma-glutamyl transferase (GGT) (628 UI/L), and slightly elevated AST (59 UI/L) and ALT (80 UI/L). Liver echography and abdominal computed tomography showed normal intrahepatic and extrahepatic biliary ducts and some lymph node involvement at the hepatic hilum. Autoimmune screening demonstrated positive antimitochondrial antibodies type M2, with a titer 1/640 and positive antinuclear antibodies (ANA), with a titer 1/640, nuclear dots pattern. Biliary acids were elevated (45.5 lmol/L). The patient started ursodesossicolic acid 300 mg three times daily and tried to taper cyclosporine, but with a dose lower than 2 mg/kg daily, pruritus reappeared. A liver biopsy was performed, and histology showed features of chronic portal and interface hepatitis, with associated bile duct lesions. Signs of primary biliary cirrhosis were absent, and the more reliable diagnosis was chronic autoimmune hepatitis type 1 with cholestasis, even though an overlap syndrome of autoimmune hepatitis and primary biliary cirrhosis was not excluded. The cyclosporine was stopped, and a combination therapy of oral prednisone 5 mg daily and oral azathioprine 50 mg daily was started with immediate excellent results. At follow-up (2, 4, and 6 months), the PN was completely healed, and the patient did not report itching. The latest laboratory tests showed improvement: liver function was normal, ANA was negative, and antimitochondrial antibody titer remained stable (1/640). PN has been associated with many different diseases. Laboratory and instrumental investigations need to be performed to exclude such associations that include obstructive biliary disorders. We report a case of chronic autoimmune cholestatic hepatitis type 1 manifesting as PN. Figure 1 Excoriated papules and nodules on the back, with secondary hyperpigmentation of healed lesions Figure 2 Hyperorthokeratosis and parakeratosis. Prominence of rete ridges. In the superficial dermis, slightly dilated vessels and slight perivascular inflammatory infiltrate, constituted by lymphocytes, with rare mast cells and eosinophils. (hematoxylin and eosin, original magnification ·20)

Benign myoepithelioma in the interdigital space of the foot.

To the Editor: Myoepithelioma is a rare dermal neoplasm mainly localized in the subcutaneous and subfascial soft tissue of the extremities of children and adults. The degree of myoepithelial differentiation varies and the stroma ranges from hyalinized to condromyxoid. Only a small number of case reports of myoepithelioma, primarily occurring in the dermis, have been described in the literature. A 49-year-old woman referred to us for evaluation of an ovular nodule, 2 3 1.5 cm in diameter, with smooth, fleshcolored surface, located in the interdigital space between the fourth and fifth toes of the left foot. The nodule appeared 4 years before consultation and had always been stationary and asymptomatic, but over the last 5 months it had become ingrown and painful, causing the patient some discomfort (Fig. 1). An excision of the whole nodule was performed. Histology showed a circumscribed nodular intradermal proliferation, characterized by rounded ovoid cells with pale cytoplasm focally arranged in a striking trabecular fashion and distributed in a mixoid stroma. Immunohistochemical study showed staining for cytokeratin CAM 5.2, pankeratin, desmin, p63, and calponin, with focal immunostaining for glial fibrillary acidic protein (GEAP). On the basis of these histological and immunohistochemical findings, a final diagnosis of myoepithelioma of skin was made (Figs. 2, 3). Myoepitheliomas are included in the ‘‘mixed tumor/parachordoma family’’ neoplasm of the soft tissues (WHO classification), but their definitive classification is still a matter of debate; some Authors consider them as one extremity of the wide spectrum of mixed tumours (monophasic variant), while others separate them from parachordomas, owing to the lack of epithelial differentiation in the latter group and a slightly worse prognosis. Although the majority of morphologically benign or low-grade myoepithelial neoplasm of soft tissue behave in a benign fashion, there is an approximately 20% risk for a local recurrence. In addition myoepitheliomas may reach considerable size and mimic other soft tissue tumors, such as condroid lipoma when myxoid stroma is particularly evident; the latter, however, is deeper in localization, and does not show ‘‘myoepithelial areas’’ and myoepithelial-associated antigens. For all these reasons a close followup is suggested and an enlargement of the excision is advisable in cytological more aggressive cases.

Diagnosis and treatment of post-Caesarean scar endometriosis.

Sir, Endometriosis is a common disease that involves approximately 10–15% of women of reproductive age. It is defined as an ectopic implantation of endometrial tissue (glands and stroma) outside the uterus, causing dyspareunia, dysmenorrhoea, menstrual irregularities and infertility. It is frequently detected in the pelvic organs, such as the peritoneum, the ovaries, the pouch of Douglas and the uterosacral ligaments (1). Extra-pelvic endometriosis is a rare condition. It is reported most frequently in the gynaecological and surgical literature (2). The possible sites are, in order of frequency, the abdominal wall, the bowel, the peritoneum, the omentum, the umbilicus, the hernia sacs, the bladder, the kidney, the lungs, the pleura and the extremities (1, 3, 4). The most common locations of abdominal wall endometriomas are old surgical scars from obstetric or gynaecological procedures. Scar endometriosis presents clinically as a painful, palpable subcutaneous mass, associated with cramps and bloating during menses. It is easily confused with other conditions, such as keloids, haematoma, stitch granuloma, abscess, inguinal and incisional hernia (5). Caesarean scar endometriosis is the most frequently reported form of this disorder and is usually benign, although a malignant transformation has been reported (4).

Erythematous papules on the parasternal region in a 76‐year‐old man

A 76-year-old man presented with a diffuse eruption that had started a few months previously. He had a history of several years of muscle weakness and fatigue associated with mild joint pain and weight loss. He reported itching, abundant salivation and excessive sweating. Over several months, he had developed confusional episodes with disorientation, visual and auditory hallucinations, complex behaviour during sleep and progressive nocturnal insomnia. Before presentation to us, a diagnosis of Morvan’s syndrome had been made at the Neurological Clinical Department, University of Bologna. Physical examination revealed many erythematous papules on the trunk, especially on the parasternal region. (Fig. 1), and the patient reported mild itching and pain. An excisional biopsy was obtained from one of the lesions (Fig. 2).

Multiple acquired pigmented lesions in a patient affected by melanoma

A 64-year old Caucasian man had a nodular cutaneous melanoma on his back with a Breslow thickness of 5.7 mm, mitotic rate 2/mm 2 and no ulceration. He attended our melanoma outpatient clinic for scheduled follow-up visits every three months. The patient underwent a sentinel lymph node biopsy with intradermal injection of a radiotracer and methylene blue, and a single macrometastasis was identifi ed in his left axilla. The following complete level I–III axillary dissection was negative for other metastases and no adjuvant therapy was prescribed by the oncologists. The patient underwent annual check-ups with lymph node and abdominal sonography as well as total body positron emission tomography/computed tomography (PET/CT) [ 1 ] . Multiple acquired pigmented lesions in a patient affected by melanoma Case for Diagnosis

Multiple perianal ulcers due to suppositories

We report a case of long‐standing inexplicable perianal ulcers. After exclusion of an inflammatory, infectious or neoplastic origin, a thorough personal history revealed that for many years the patient had been using analgesic suppositories containing indomethacin, caffeine, and prochlorperazine dimaleate, four to five times a week, for migraine. On stopping the suppositories, there was complete healing within 12 weeks. We hypothesize that vasoconstriction and vascular damage were the pathogenetic mechanisms behind the perianal ulcers.