Case reports about vasopressin-induced cutaneous necrosis are not frequent. Here we report a further case, of which skin manifestations included not only mottling, cyanosis, ecchymosis, bullae and gangrene, but also amber-like change in focal areas. Besides, intermittent paling of the skin with or without deep pain sensation of the limbs over non-injection sites was observed that might be a warning sign of impending skin necrosis. Based on the literature about vasopressin-induced skin necrosis we discuss the possible role of coagulation enhancement of this molecule.

We demonstrated that loss of function might not be the only way for mutated LMX1b to cause haploinsufficiency as the main pathogenic mechanism for NPS. LMX1b-R198X has less nuclear localization and higher stability than the wild-type protein; consequently, it might function as a competitor to sequester other effectors by protein-protein interaction to interfere with downstream transcriptional events.

A 36-year-old woman presented with a 3-month history of pruritic blisters on her trunk and legs, which had appeared 4 months after she gave birth. She was breast-feeding and was not taking any medication. On physical examination, scattered erythematous crusted patches were seen on her trunk. Grouped vesicles and bullae had developed on well-defined erythematous patches on both lower legs ( Fig. 1a). Vesicles arranged in a herpetiform configuration were also found ( Fig. 1b). Nikolsky sign was negative. No mucosal lesions were present. Laboratory data, including blood cell counts and biochemistry, were unremarkable. The anti-nuclear antibody titre was undetectable. Histopathological findingsSkin biopsy of the lesion over the anterior shin disclosed intraepidermal vesicles, showing significant spongiosis and abundant neutrophil infiltration, with micro-abscess formation (Fig. 2a). No acantholytic cells were identified. Direct immunofluorescence (DIF) showed intercellular deposition of IgG and C3 throughout the epidermis (Fig. 2b). Figure 1 (a) Erythematous, herpetiform vesicles on the lower legs, with crusting and hyperpigmentation in some areas; (b) vesicles in a herpetiform configuration on three partially overlapping patches. ª