Subcutaneous Sarcoidosis during Pegylated Interferon Alfa and Ribavirin Treatment for Chronic Hepatitis C.

Interferon is used to treat hepatitis C virus infection and its cutaneous side effects are well known. Recently, interferon-induced sarcoidosis has been reported. We report a new case of sarcoidosis during pegylated interferon alfa and ribavirin treatment with an unusual presentation in a woman with previous episodes of erythema nodusum and nodular vasculitis related to HCV.

[Chronic graft-versus-host disease presenting as bullous lesions]. / Lesiones ampollosas como forma de presentación de una enfermedad de injerto contra huésped crónica.

Graft-vs-host disease is still the leading cause of morbidity and mortality in patients undergoing bone marrow transplantation. It is important to start treatment early to reduce the severity and consequences of this complication. Cutaneous lesions are often the presenting compliant of graft-vs-host disease and presage visceral involvement. We present the case of a 45-year-old woman with multiple myeloma who underwent autologous and subsequently allogeneic bone marrow transplantation with hematopoietic precursors. She developed bullous lesions with fluid elimination on the abdomen and legs. Biopsy findings were compatible with graft-vs-host disease and immunosuppressive therapy was increased. She subsequently presented oral lichenoid lesions and sicca syndrome. The bullous lesions progressed to painful ulcers that healed leaving highly sclerodermatous skin with substantial hyperpigmentation. Bullous lesions are a rare form of presentation of chronic graft-vs-host disease. In such cases, the diagnosis may not be suspected initially, particularly when the lesions are isolated and internal organs are not involved.

[Bilateral pseudo-Kaposi sarcoma in upper limbs]. / Pseudosarcoma de Kaposi bilateral en miembros superiores.

Acroangiodermatitis or pseudo-Kaposi sarcoma is an angioproliferative, self-limited entity that includes a group of diseases, congenital or acquired, with cutaneous lesions similar to Kaposi sarcoma (KS). This term can lead to confusion because it comprises several entities that are completely different, nonetheless, it has an important clinical value as it guides the diagnosis and management of these patients. We report the case of a 67-year-old patient with lesions of acroangiodermatitis in both forearms secondary to arteriovenous shunts from hemodialysis. Doppler ultrasound showed a former arteriovenous fistula in addition to the one already known. Immunohistochemical study showed CD34+ staining in endotelial cells and absence of HHV-8 expression.

[Fatal Sweet syndrome associated to chronic idiopathic systemic inflammatory response syndrome]. / Síndrome de Sweet de evolución fatal asociado a síndrome de respuesta inflamatoria sistémica crónico idiopático.

Sweet syndrome is one of the cutaneous processes more frequently associated to systemic diseases. Its association to the systemic inflammatory response syndrome has rarely been described. We report a case of chronic and relapsing Sweet syndrome associated to a chronic and idiopathic systemic inflammatory response syndrome that lasted seven years and proved fatal to the patient. Among the rare cases of Sweet syndrome associated to a systemic inflammatory response syndrome that have been described there have not been any fatal cases as occurred with our patient.

Metastatic tuberculous abscesses in an immunocompetent patient.

The decreased incidence of infectious diseases in developed countries may make their diagnosis difficult. Cutaneous tuberculosis is an example of this fact. A 44-year-old man presented with two painful abscesses on his lower extremities, which developed into chronic ulcers. A cutaneous biopsy revealed necrotizing granulomas in the dermis. Ziehl-Neelsen and periodic acid-Schiff stain were negative. Mantoux test was positive. Tc-99m scintigraphy showed increased uptake in the bone tissue of the left ankle and right tibiae, without direct relation to cutaneous lesions. Chest X-ray showed micronodular, apical, bilateral infiltrates, reduced volume of the right lung, and cavitation of the right superior lobe. Mycobacterium tuberculosis was grown from sputum and skin biopsy samples. Isoniazid, rifampin and pyrazinamide treatment for 2 months, followed by isoniazid and rifampin for 12 months, resulted in complete resolution. The clinical features of cutaneous tuberculosis in our patient were characteristic of tuberculous abscesses. Some uncommon findings, such as the low number of lesions, negative acid-fast resistant stains in cutaneous biopsy samples and his preserved general state of health, may be explained by a higher competence of the immune system than is usual in this clinical subset of disseminated tuberculosis. Cutaneous tuberculosis should be included in the differential diagnosis of cutaneous abscesses in immunocompetent patients.

Linear immunoglobulin A bullous dermatosis induced by gemcitabine.

OBJECTIVE: To report a case of linear immunoglobulin (Ig) A bullous dermatosis (LABD) induced by gemcitabine. CASE SUMMARY: A 59-year-old man was diagnosed with squamous-cell carcinoma of the lung in T4N2M0 stage and treated with cisplatin, vinorelbine, and gemcitabine. Twenty-four hours after the administration of gemcitabine, a symmetric, bullous, herpetiform eruption appeared on his trunk and upper limbs. Histopathologic examination and direct immunofluorescence test were consistent with IgA bullous dermatosis. Cutaneous lesions resolved two weeks after the drug was withdrawn and topical steroid treatment was instituted. DISCUSSION: Drug-induced LABD is a variant of classic or idiopathic LABD. Vancomycin is the most frequently implicated drug, but other agents have been reported to cause LABD. According to the Naranjo probability scale, the relationship of gemcitabine treatment with cutaneous eruption in our patient is possible. CONCLUSIONS: We report the first case of gemcitabine-induced LABD. Clinicians should monitor patients receiving this drug for signs of LABD.

Dermatomyositis and mucinosis.

BACKGROUND: Mucin deposition is a common feature in autoimmune collagen diseases including dermatomyositis. Nevertheless, clinical manifestations of mucinosis are uncommon in patients with dermatomyositis. Two cases of mucinosis associated with dermatomyositis are reported. PATIENTS: A 53-year-old woman presented with symmetrical plaques on the upper limbs formed by the coalescence of small, violaceous papules. In addition, she showed the typical cutaneous and muscle features of dermatomyositis. A 44-year-old woman with dermatomyositis of 5 years' evolution developed linear, flesh-colored papules across the flexural creases of her palms and fingers. RESULTS: Skin biopsy of the upper limb lesions in the first patient showed epidermal changes compatible with dermatomyositis and dermal mucin deposition. Histopathologic examination of the palmar lesions of the second patient showed less intense epidermal changes of dermatomyositis and dermal mucin deposition. CONCLUSIONS: Mucin deposition in patients with dermatomyositis may have an unusual clinical presentation, and it should be considered in the differential diagnosis of atypical cutaneous lesions in these patients.

Two cases of segmental multiple glomangiomas in a family: type 1 or type 2 segmental manifestation?

Several autosomal dominant skin diseases may manifest cutaneous mosaicism. Two types of segmental arrangement can be distinguished: type 1 is characterized by segmental lesions with similar severity to that observed in the diffuse phenotype, the remaining skin being normal; type 2 is characterized by segmental lesions showing a major degree of severity and milder lesions diffusely arranged. Multiple glomus tumours have recently been included in the group of genodermatoses showing type 2 segmental involvement. A family with 2 cases of multiple glomangiomas arranged in a segmental fashion is reported. A 12-year-old girl presented multiple nodular glomangiomas on her right buttock and thigh, in a band-like distribution. A sister of her paternal grandfather showed plaque-like multiple glomangiomas on her left thigh and various glomangiomas on her right buttock and arm. No other family members were known to be affected. Two new cases of familial segmental multiple glomangiomas are reported, with the particularity that one of these exhibited type 2 segmental manifestation and the other type 1 from a clinical point of view.

[Malignant diffuse mesothelioma: contribution of 23 cases]. / Mesotelioma difuso maligno: aportación de 23 casos.

Malignant diffuse mesothelioma is a tumour related to asbestos exposure, more common in males and located primarily in the chest cavity. Its diagnosis requires ruling out other tumours with pleural or peritoneal metastases, a biopsy showing a morphological pattern consistent with mesothelioma and in many cases to perform immunohistochemical markers to rule out an adenocarcinoma. We report here 20 cases of diffuse pleural mesothelioma and three cases of peritoneal mesothelioma in 20 males and three cases of peritoneal mesothelioma in 20 males and three females. Asbestos exposure was observed for 31% of cases. The most common clinical manifestations included headache and dyspnea; interestingly, three cases had hydropneumothorax with poor response to drainage. The diagnostic confirmation was obtained mainly with thoracotomy or laparoscopy biopsies and to rule out an adenocarcinoma immunohistochemical stainings were performed.

Cytomegalovirus causing necrotizing laryngitis in a renal and cardiac transplant recipient.

BACKGROUND: Cytomegalovirus (CMV) infection is a common opportunistic pathogen in transplant recipients; however, cytomegaloviral laryngitis is extremely rare. METHODS: We present a case of disseminated CMV infection and ulcerating necrotizing laryngitis situated in the subglottis of an immunocompromised patient with a renal and cardiac transplant. The patient was initially seen with discomfort in the throat. RESULTS: Physical examination of the head and neck, fiberoptic laryngoscopy, and CT scan of the larynx were normal. Histopathology of a laryngeal ulcer in the subglottic region showed intranuclear and intracytoplasmic bodies. Immunoperoxidase stain and in situ hybridization studies were positive for CMV. CONCLUSIONS: In immunosuppressed patients with persistent throat symptoms, the systematic exploration of larynx and bronchus is necessary to rule out the presence of opportunistic infections.

[Corticoid-resistant rejection and humoral rejection. The diagnostic criteria and therapeutic management]. / Rechazo corticorresistente y rechazo humoral. Criterios diagnósticos y manejo terapéutico.

The standard treatment of rejection episodes post-heart transplantation is based on high dose of steroids followed by monoclonal or policlonal antibodies directed against T lymphocytes like ATG or OKT3. When this treatment is insufficient it can be used other drugs like metothrexate, ciclofosfamide and another procedures such as total lymphoid irradiation, plasmapheresis or photopheresis. Cardiac allograft rejection is defined by histologic features that include the presence of lymphocytic infiltration of the myocardium with or without necrosis and this histologic pattern is termed cellular rejection. However there is a second form of acute rejection, associated with capillary injury, usually mediated by humoral immune response and without interstitial infiltrates which has been named vascular or humoral rejection. Vascular allograft rejection is commonly resistant to standards forms of immunosuppressive therapy and may result in irreversible allograft dysfunction and reduced patient survival.

[Asymmetric polyarthritis in Sézary's syndrome]. / Poliartritis asimétrica en el síndrome de Sézary.

We present the case of a patient with negative serology asymmetric polyarthritis and erythematous-squamous cutaneous lesions of two years evolution, initially attributed to psoriasis, which histopathology (hyperkeratosis, epidermic infiltration due to atypical CD4+ lymphocytes with formation of Pautrier's microabscesses) together with hematological findings (persistent lymphocytosis with cells with cerebriform nucleus, 97% CD4+) allowed the diagnosis of Sézary's syndrome. To our knowledge, this is the eight documented case of arthritis in Sézary's syndrome and the first one with asymmetric chronic polyarthritis.

Cystic giant solitary trichoepithelioma.

A case of a giant solitary trichoepithelioma is reported. The tumor was located on the thigh, extending from the deep dermis to the subcutaneous tissue with no epidermal contact, and showed a large central cystic cavity that measured 9 cm x 4 cm. We review the cases published under this and other names.