We report the exceptional finding of bone metaplasia in the setting of a case of NFD.

Schnitzler's syndrome (SS) is a rare entity characterized by the association of chronic urticaria and monoclonal IgM gammopathy. Usually, intermittent fever, arthralgia and elevation of erythrocyte sedimentation rate also occur. We report a patient with the same symptoms, but with monoclonal IgG instead of IgM paraproteinaemia. Histological examination of the urticarial lesions showed signs of leucocytoclastic vasculitis. After 20 years of therapeutic failure, cyclosporin has achieved a total clearance of urticarial lesions in our patient. Two previous similar cases with clinical features of SS and monoclonal IgG immunoglobulin have been described. We suggest our case also represents a variant of SS with IgG gammopathy.

Superficial mucoceles are subepithelial extravasations of sialomucin that occur at the epithelial-connective tissue interface and are directly related to minor salivary glands. They have been described in association with oral lichen planus and, exceptionally, with chronic graft versus host disease. Three patients who underwent an allogeneic bone marrow transplantation for a chronic myelogenous leukaemia presented multiple superficial mucoceles and an oral lichenoid graft versus host disease.

Takayasu’s arteritis (TA) is a chronic inflammatory and fibrosing arteriopathy that can also involve cutaneous vessels. The disease typically presents with a prepulseless phase that overlaps or is followed by the characteristic pulseless stage. In both phases of TA, cutaneous manifestations may be present. Lesions considered to be ‘specifically’ associated with TA have been described most frequently simulating erythema nodosum, erythema induratum and pyoderma gangrenosum. We report 2 Caucasian patients with TA and nodular cutaneous lesions. Nine skin biopsies from these patients were studied. A necrotizing vasculitis was present in 5 biopsies. We review those patients with TA and well-documented cutaneous manifestations in the English literature, with special interest in nodular lesions, the most frequent cutaneous manifestation of TA in Caucasian patients. Biopsies from lesions with similar morphology frequently show different histological findings.

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