Bo-Kyung Koh

Recurrent primary cutaneous adenoid cystic carcinoma of the scrotum

A 60‐year‐old man had asymptomatic erythematous nodule on the right inguinal area, which had progressively grown for 2 years. Excision was performed at the Department of Urology of our hospital. Two months later, there was local recurrence on the excision site, and it extended locally to make several tender erythematous nodules on the right inguinal area and firm plaque on the scrotum ( Fig. 1 ). Histopathologic examination of the excision specimen revealed large cell masses with tubular or cribriform pattern and many small, solid epithelial islands unattached to the overlying epidermis. The tumor was confined to the dermis and subcutaneous tissue. Round spaces formed by malignant epithelial cells and containing amorphophilic basement membrane‐like material occured inside the tumor masses. The tumor cells were basaloid cells with scanty cytoplasm and deeply stained round‐to‐oval nucleus ( Fig. 2 ). There was clear perineural involvement by the tumor cells. A substance in the pseudoglandular spaces, cystic spaces and the stroma was stained strongly with periodic acid‐Schiff (PAS) and alcian blue (pH 2.5). A PAS‐positive substance was digested by diastase pretreatment. These findings suggested the substance to be a neutral mucopolysaccharide. Immunoperoxidase staining for S‐100 protein and cytokeratin were strongly positive with the tumor cells but not with carcinoembryonic antigen. A histopathologic diagnosis of ACC was made. Salivary glands, bronchi, esophagus, and other organs were examined, but there was no evidence of neoplasm in other organs. A diagnosis of primary cutaneous adenoid cystic carcinoma was made but the patient refused all the treatment, including surgery.

A case of melanonychia caused by Fusarium solani

SIR, The ichthyoses are a heterogeneous group of skin disorders of epidermal differentiation, with both inherited and acquired forms. This cornification disorder may be found isolated or in association with other genetic defects. In 1998, five siblings with congenital ichthyosis, follicular atrophoderma, hypotrichosis and hypohidrosis were described as a new genodermatosis by Lestringant et al. We report a 17-year-old Turkish patient with ichthyosis vulgaris, follicular atrophoderma, woolly hair and hypotrichosis as a second report on this syndrome. A 17-year-old-girl was admitted to our hospital because of woolly hair, sparse eyelashes and eyebrows, and a very dry skin. She was born at term after an uncomplicated pregnancy. Ichthyosis and baldness were present at birth, but there was no history of a collodion baby. She had almost no scalp hair until she was 4 months old. In the early childhood period, funnel-shaped round follicular depressions had appeared on the dorsal aspects of the hands. She stated that her scalp hair had improved and straightened with age. The patient was otherwise healthy and detailed ophthalmological, neurological and audiometric examinations were normal. There was no history of atopy. There was no family history of similar skin problems. Both her parents and paternal grandparents were first cousins. There was no maternal history of drug intake during pregnancy. On examination, there was diffuse ichthyosiform scaling sparing the major flexures and face as in ichthyosis vulgaris (Fig. 1). The ichthyotic skin was hypohydrotic but the axilla, palms and soles sweated normally. Follicular atrophoderma was observed on the backs of her hands (Fig. 2). She had diffuse and patchy non-scarring hypotrichosis with a receding frontal hairline. Her hair was normal in length, but was light brown in colour, coarse, curly and unruly, in contrast to the straight black hair of the rest of her family. Eyelashes and particularly eyebrows were sparse (Figs 3–4). Routine haematological, biochemical, immunological, thyroid and radiological investigations were normal. Osteopoikilosis was not present on the X-rays. Echocardiography and ECG were normal. Hair microscopy was normal apart from curling. Biopsy from ichthyotic skin showed orthokeratosis with focal hypogranulosis (Fig. 5). Electron microcopy showed normal tonofilaments. Based on clinical and laboratory findings a diagnosis of ichthyosis vulgaris associated with follicular atrophoderma, hypotrichosis and woolly hair was made. The patient was prescribed 10% urea cream and salicylic acid ointments. The ichthyotic lesions resolved within a few weeks. Ichthyosis is a feature of several genetic disorders. These are rare disorders and the associated ichthyosis may be mild. The following syndromes with ichthyosis may be considered