Case Report- Ichthyosis bullosa of Siemens: Response to topical tazarotene

In 1937, Siemens described a Dutch family with superficial blistering, flexural hyperkeratosis, and characteristic mauserung appearance. Since then, less than 20 kindreds with this condition have been described in the English dermatologic literature. A 14-year-old boy presented with history of recurrent blistering and peeling of skin since the age of 1 month, predominantly seen over limbs and trunk, often associated with secondary infection. His mother also had similar symptoms from childhood. On examination, the child had typical mauserung peeling of the skin and dirty gray hyperkeratosis in a rippled pattern over flexures. Skin biopsy from the boy showed intracorneal blistering with epidermolytic hyperkeratosis in the upper spinous layers. The typical history and clinical features along with characteristic histological findings confirmed our diagnosis of ichthyosis bullosa of Siemens. It must be differentiated from other conditions with epidermolytic hyperkeratosis and skin peeling, such as bullous ichthyosiform erythroderma of Brocq and peeling skin syndrome. Our patient responded well to 0.05% topical tazarotene gel over four weeks

Spot the diagnosis

A 24-year-old male presented with hoarseness of voice and asymptomatic papular eruption on the face since infancy. He was the second child in the family, born to consanguineous marriage. His younger sister had similar disease. He had no history of photosensitivity or blistering, seizures. He had multiple yellowish waxy papules on the borders of his eyelids giving it a beaded appearance (Figure 1). Similar lesions were also seen on forehead, arms and upper trunk. The skin over the face and neck was shiny and infiltrated with areas of pock like scarring. He also had vegetating plaques in the intertriginous areas. The lips were thickened, infiltrated and everted. The buccal mucosa had a distinct cobblestone appearance. His tongue was firm and enlarged. Systemic examination did not reveal any abnormalities

Dermatomyositis associated with adenocarcinoma of the lung: A case report

The association of dermatomyositis and underlying malignancies, especially small cell and squamous cell carcinoma of the lung, has been recognized a long time ago. We report the case of a 63-year-old male chronic smoker with adenocarcinoma lung associated with dermatomyositis. The occurrence of dermatomyositis should be considered as a potential presentation of paraneoplastic syndromes and physicians encountering similar clinical situations should perform extensive diagnostic work-up to exclude underlying neoplastic lesions