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Background: Acrokeratosis paraneoplastica is an infrequent paraneoplastic disease in which cutaneous lesions appear and develop in a context of an underlying malignancy. Upper aerodigestive tract carcinomas are the most common neoplasias associated with this syndrome. The aim of this report is to raise awareness of this syndrome, since the recognition of the skin lesions could allow suspicion and diagnosis of the underlying neck malignancy at an early curable stage. Methods: We report a case of a 62-year-old life-long smoking male who presented a several months evolution of multiple skin lesions, affecting both ear lobes, hands and feet. He was referred for appropriate diagnosis and treatment of the lesions. Results: After further examinations and study of the patient, the diagnosis concluded in the detection of a laryngeal squamous cell carcinoma (SCC) causing the paraneoplastic skin-affecting syndrome (acrokeratosis paraneoplastica). Conclusions: A correctly oriented clinical suspicion of the malignancy was possible due to the detection of suggestive skin lesions affecting ear lobes and other locations. Keywords: Acrokeratosis paraneoplastica, Bazex syndrome, Laryngeal carcinoma, Skin lesions, Paraneoplasia

Background. Sebaceous gland tumors are a rare type of neoplasm. In some cases they have been associated with visceral tumors in patients with Muir-Torre syndrome, a hereditary form of nonpolyposis colorectal cancer. The aim of this study was to review the diagnosis and follow-up of a series of patients with sebaceous gland tumors to assess how many met the criteria for Muir-Torre syndrome. Patients and methods. A search was performed of records from 1990 to 2005 in the database of the Department of Dermatology of the Consorcio Hospital General Universitario de Valencia in Valencia, Spain, to identify patients with sebaceous gland tumors. The biopsy material was reviewed to confirm the diagnosis. We also searched the patient histories for information suggestive of a diagnosis of Muir-Torre syndrome; when the histories were incomplete, we contacted the patients by telephone. Results. We identified 20 patients diagnosed with sebaceous gland tumors, but after reviewing the biopsy material diagnosis was only confirmed in 12. Two patients belonged to a family with a history of visceral tumors that met the clinical criteria for hereditary nonpolyposis colorectal cancer syndrome. Follow-up was not uniform in all patients and not all underwent the same tests. Conclusions. It is essential to rule out the presence of Muir-Torre syndrome in patients with sebaceous gland tumors. The use of new techniques such as immunohistochemistry or detection of microsatellite instability may help to identify families at increased risk of Muir-Torre syndrome.

We present the cases of 2 regular cocaine users aged 30 and 37 years who developed a cutaneous condition with an identical presentation and a similar clinical course. The lesions first appeared as eruptions on the back and then spread to other parts of the body. They consisted of painful inflammatory nodules that developed into abscesses and ulcers that grew outwards. They had a granulomatous base and irregular violaceous edges. The results of histopathologic studies were compatible with the clinical diagnosis of pyoderma gangrenosum, and additional tests ruled out underlying diseases. After several therapeutic failures with usual treatments, the patients responded quickly to infliximab after interrupting their cocaine consumption. The association between pyoderma gangrenosum and cocaine use was clear as both suffered clinical relapses at the same time as they started using the drug again.

Pemphigus vulgaris is a potentially fatal autoimmune bullous disease. High doses of immunosuppressive drugs are used in managing severe cases of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, has proven to be effective in patients with refractory pemphigus vulgaris and pemphigus foliaceus. We review cases of pemphigus vulgaris and pemphigus foliaceus not associated with lymphoma that were treated with rituximab, and we report a new case of severe refractory pemphigus vulgaris successfully treated with rituximab.

Cutaneous fistulas and sinuses in the maxillofacial region secondary to osteomyelitis rarely appear in clinical practice. The most frequent cause of mandibular osteomyelitis is a dental infection, but it may also be hematogenic in origin. The diagnostic criteria for bacterial osteomyelitis are suppuration and osteolytic changes in the radiological study. The differential diagnosis of an ulcerative lesion in the mandibular area includes several pathologies, such as a fistula of dental origin, a reaction to a foreign body, a deep mycotic infection, a pyogenic granuloma or a congenital malformation.

Papillary dermal elastolysis similar to pseudoxanthoma elasticum is an elastolytic disorder characterized by cutaneous lesions on the neck and in the supraclavicular region that are clinically similar to pseudoxanthoma elasticum, with no systemic complications. The histological examination shows a loss of elastic fibers in the papillary dermis. We report a case in a 76-year-old woman with typical lesions on the neck.

We present the case of a 26-year-old Ecuadorian woman who presented with multiple hypertrophic scars along the lower left limb, secondary to a cutaneous process that she had had for 11 years, previously diagnosed and treated in her country as cutaneous leishmaniasis. It had been clinically worsening for 9 months with suppuration through the scars in the groin and thigh, associated with local pain and systemic symptoms. As we suspected cutaneous tuberculosis, we performed a number of complementary examinations to confirm the diagnosis and rule out bone involvement. Finally, a lymph node culture confirmed that it was scrofuloderma.

A 42-year-old woman presented with a 12-year history of extensive yellow and erythematous plaques, round and oblong with irregular configuration and glossy atrophic central areas on the pretibial aspects of both legs. Her 45-year-old sister presented with a 7-year history of a single plaque with erythematous margins, abundant telangiectases, and an atrophic center in the lower portion of the left leg. There was no family history of type-1 or type-2 diabetes mellitus. Both patients had normal fasting glucose concentration, oral glucose tolerance test, and glucose overload test. Different treatment options including topical corticosteroids were unsuccessful. Treatment with oral fumaric acid esters was attempted but the medication was discontinued because of intolerable side effects (flushing and gastrointestinal discomfort). At present, after a follow-up of 2 years, the plaques remain unchanged. These two cases should be added to the few cases of familial nondiabetic necrobiosis lipoidica previously reported.

A 62-year-old woman developed pruritus, oedema, erythema and vesiculation, well demarcated at the application sites of nitroglycerin TTS on the trunk and upper arms (Fig. 1). Lesions healed with post-inflammatory hyperpigmentation. Two weeks before, the patient had started treatment for coronary insufficiency with daily nitroglycerin transdermal patches (Dermatrans 5®, Recordati group, Madrid, Spain), and 6 days later she noted pruritus. The symptoms started 12 h after rechallenge. Although the patches were substituted with another brand, the pruritus and eczema did not decrease. Nitroglycerin TTS was withdrawn and the patient was treated with topical corticosteroids and oral antihistamines for a week. Later, patch tests were carried out with adhesives and nitroglycerin 1%, 0.5% and 0.1% in petrolatum (Fig. 2). All the nitroglycerin patch tests were positive at 48 h (stronger) and at 72 h, but the adhesive proved negative. The woman subsequently received sublingual nitroglycerin without adverse effects. Furthermore, the patient had been on isosorbide mononitrate orally for the last year because she had high blood pressure, but no cross-sensitivity allergic reaction occurred.