Igloo‐like prepuce: a peculiar aspect of smooth‐muscle hamartoma of the genitalia?

We report the case of a 16-year-old boy with 'igloo-like prepuce' and hypospadia, successfully treated by asymmetrical antero-posterior postectomy. Histopathological examination consisting of classical microscopy, immuno-histochemistry and electronmicroscopy of the surgical material provided insights into the etiopathogenesis of this rare disease.     

Marginal acrokeratoderma and psoriasis: is there an association?

Case 1 A 70‐year‐old housewife presented to us with multiple, asymptomatic, depressed, crateriform plaque lesions over the radial margin of the index finger and thumb and thenar and hypothenar eminences of both hands of 25 years' duration ( Fig. 1 ). There was no history of prolonged sun exposure or repeated trauma to the hands and there were no such lesions on the soles. None of her other family members were affected. We made a provisional diagnosis of keratoelastoidosis marginalis. Skin biopsy revealed an unremarkable epidermis, with minimal lymphocytic infiltration in the upper dermis and hyalinization in the reticular dermis, extending to the deeper dermis. Two years later she returned with well‐defined, erythematous, scaly, mildly itchy plaque lesions over the instep of both feet and the center of both palms. A clinical diagnosis of palmoplantar psoriasis was made.

Figure 1 Open in figure viewer PowerPoint Case 1. Crateriform plaque lesions on the hands. Note the palmoplantar psoriasis     

A case of pachyonychia congenita with unusual manifestations: an unusual type or a new syndrome?

A 30‐year‐old man presented with lesions on his oral mucosa and soles. There were no similar complaints in his family members. The dermatological examination revealed follicular hyperkeratosis on his trunk and upper extremities and flesh‐colored, firm cystic lesions on his axillae. He had focal, painful, hyperkeratotic areas sited particularly on both his soles and palms. In addition to these, leukokeratosis and ulcerative areas on buccal, labial mucosa, tongue, and at corners of the mouth, and complete loss of teeth was observed. The proximal layering was revealed on all of his nails. The laboratory investigations produced normal results except the deficiency of immunoglobulin A. The psychiatric examination revealed mild mental retardation. Keratin gene (KRT6a, KRT6b, KRT16, and KRT17) mutations for pachyonychia congenita were negative. He got removable dental prosthesis because of inadequate alimentation. Squamous cell cancer developed on lower lip mucosa during follow‐up. We present an individual who had different nail dystrophy, epidermal cysts, mental retardation, blepharitis, complete loss of teeth, and negative keratin gene mutations for pachyonychia congenita and developed squamous cell cancer on the oral leukokeratosis lesions. We think that the present case may be an unusual new type of pachyonychia congenita.