Lichenoid pseudovesicular papular eruption on nose: A papular facial dermatosis probably related to actinic lichen nitidus or micropapular polymorphous light eruption

Background: Facial papules are a feature of several clinical conditions and may present both diagnostic and therapeutic challenges. Aim: To describe a grouped papular eruption on the nose and adjoining cheeks that has not been well characterized previously. Materials and Methods: A series of consecutive patients with a papular eruption predominantly involving nose and cheeks were evaluated, treated and followed up prospectively at tertiary care centers. Demographic details, clinical features, histopathology and response to treatment were recorded. Results: There were five men and six women (mean age 29.9 ± 6.9 years) who had disease for a mean duration of 17.3 ± 11.1 months. All patients presented with a predominantly asymptomatic eruption of monomorphic, pseudovesicular, grouped, skin colored to slightly erythematous papules prominently involving the tip of nose, nasal alae, philtrum and the adjoining cheeks. A total of 15 biopsies from 11 patients were analyzed and the predominant finding was a dense, focal lymphoid infiltrate restricted to the upper dermis with basal cell damage and atrophy of the overlying epidermis. The eruption ran a chronic course from several months to years. Limitations: Direct immunofluorescence could not be performed except in one case. Immunohistochemical stains for CD4 and CD8 could not be done owing to nonavailability. Phototesting was undertaken in one patient only. Conclusion: Small grouped papules on the nose and adjoining skin with a lichenoid histopathology appear to represent a distinct clinicopathological entity. It may be related to actinic lichen nitidus/micropapular variant of polymorphous light eruption.

Angiomyomatous hamartoma of lymph nodes: Clinicopathological study of 6 cases with review of literature.

Angiomyomatous hamartoma (AMH) is a rare disease with predisposition for inguinal and femoral lymph nodes. Histologically, it is characterized by replacement of lymph nodal parenchyma with irregularly distributed thick walled blood vessels, haphazardly arranged smooth muscle cells, variable amount of fat and fibrous tissue in a sclerotic lymphatic stroma. Few cases have also been reported in popliteal and sub ‑ mandibular location. The exact pathogenesis is still not known. Although this entity is very rare, its recognition is important in discriminating it from other benign and malignant vascular lesions of lymph nodes.

Synchronous existence of granular cell tumor and small cell carcinoma of lung: An unusual entity.

Granular cell tumor (GCT) is a rare benign mesenchymal tumor that uncommonly occurs in the lung and tracheobronchial tree. Small cell carcinoma of lung is a centrally located malignant neoplasm that commonly occurs in elderly smokers. Concomitant existence of both the neoplasm in lung is extremely rare with only one reported case in the literature. Few rare combinations of GCT with other primary bronchogenic carcinomas have also been reported. Clinical symptoms depend upon the site and size of the tumor. Definitive diagnosis is by histopathological and proper immunohistochemical analysis. Identification of this entity is important as treatment requires individual therapy protocols that depend on the presence of metastasis, location of the tumors, and type of bronchogenic carcinoma.

Sclerosing liposarcoma of the anterior mediastinum: An unusual case.

Liposarcomas are extremely rare in the mediastinum. Patients usually present late due to the compressive effect of the tumor on the adjacent structures. Severity of the symptoms depend mainly on the size of the tumor and the structure it infiltrates. Well differentiated slow growing liposarcomas are the most common ones in the mediastinum followed by dedifferentiated and poorly differentiated ones. These tumors have bad prognosis because of incomplete surgical excision due to its inaccessible location. Hence these patients should be kept under close follow up because of high recurrent rates. Here we are presenting a rare case of anterior mediastinal sclerosing liposarcoma in a 77 year old male.

Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report.

Myxomas are the most common benign cardiac tumors constituting approximately 75% of all the cardiac tumors. Rest 25% are malignant and sarcomas being the commonest. Among the sarcomas primary cardiac malignant peripheral nerve sheath tumors are extremely rare. They usually arise in relation to the branches of vagus or phrenic nerves, 5‑42% being associated with neurofibromatosis type 1. Clinical signs and symptoms depend on the location and extent of involvement. Complete resection is the treatment of choice but local recurrence is common.

Primary pulmonary leiomyosarcoma of lung: An unusual entity with brief review.

Primary pulmonary leiomyosarcomas are rare and diagnostically challenging group of neoplasms approximately constituting 0.2-0.5% of all primary lung malignancies. They originate from the smooth muscle cells of the bronchial wall, blood vessels or from the pulmonary interstitium. Here we present a case of 45 year old male with history of chronic cough, breathlessness and chest pain for few months. Clinical and radiological workup showed a left upper lobe lung mass. Endobronchial ultrasound guided (EUS) needle biopsy of the mass on histopathology confi rmed leiomyosarcoma. Further through evaluation ruled out the possibility of metastasis. A fi nal diagnosis of primary leiomyosarcoma of lung was made and patient was planned for surgical management.

Intralesional radiofrequency ablation for nodular angiolymphoid hyperplasia on forehead: A minimally invasive approach.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an idiopathic acquired condition characterized by erythematous papulo-nodular lesions with a predilection for the head and neck. The lesions are cosmetically disfiguring, resistant to most medical and surgical therapies and tend to recur. We report the novel use of radiofrequency equipment in the management of nodular ALHE on forehead of a 53-year-old man. Intra-lesional radiofrequency ablation was done using a modified 18 gauge intravenous cannula and three sittings over a period of four years yielded cosmetically acceptable results with no recurrence and minimal side effects.

Tracheobronchopathia osteochondroplastica: A rare cause of difficult intubation.

Tracheobronchopathia osteochondroplastica is a rare benign airway disorder which is characterised by submucosal nodules projecting into the tracheo-bronchial lumen usually involving the cartilaginous portions of the tracheo-bronchial tree or larynx. The condition is usually asymptomatic but can rarely present with difficulty during endotracheal intubation or rarely with obstructive airway complications. Bronchoscopic appearance is usually sufficient to make the diagnosis, and tissue biopsies are seldom required. No specific treatment is required in asymptomatic patients. However, interventional bronchoscopy procedures or surgery may be helpful in symptomatic cases.

Desmin-related restrictive cardiomyopathy in a pediatric patient: A case report.

Restrictive cardiomyopathies in the pediatric population have diverse etiologies, including storage diseases like hemosiderosis, glycogenoses and desmin with its associated proteins. Desmin-related myopathy is a rare familial disorder of the cardiac and skeletal muscle characterized by intrasarcoplasmic accumulation of desmin-reactive deposits in the muscle cells. The patients commonly present with cardiac involvement such as conduction blocks and/or restrictive cardiomyopathy. Diagnosis of desmin cardiomyopathy depends on light microscopic evaluation of endomyocardial biopsy, where abnormal deposition of desmin can be documented on immunohistochemistry and ultrastructural examination. The index report presents the clinical, light microscopic and ultrastructural fi ndings of desmin cardiomyopathy.