Progression of IgA gammopathy to myeloma following infliximab treatment for pyoderma gangrenosum.

Pyoderma gangrenosum (PG) may be associated with inflammatory disorders and haematological conditions such as monoclonal gammopathy of uncertain significance (MGUS). We report the case of a 53-year old man who had PG and MGUS. After treatment with infliximab for the PG, he developed myeloma. The course of events in this case suggests that infliximab facilitated the progression from MGUS to myeloma.

Nicorandil-induced leg ulceration without mucosal involvement.

We report a case of leg ulceration occurring in a patient without mucosal ulcers, in whom nicorandil appeared to be the main aetiological factor. Having failed to heal on compression therapy, the ulcer rapidly improved and healed after the discontinuation of nicorandil. Most cases of nicorandil-induced ulcers reported in the literature develop on mucosal surfaces, including oral, vulval, perianal and peristomal ulcers. There are rare reports of cutaneous ulceration attributable to nicorandil, occurring concurrently with mucosal ulcers. To our knowledge, this is the first case of nicorandil-induced leg ulceration affecting the skin without mucosal involvement.

Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency.

Germline mutations of the fumarate hydratase (FH, fumarase) gene are found in the recessive FH deficiency syndrome and in dominantly inherited susceptibility to multiple cutaneous and uterine leiomyomatosis (MCUL). We have previously reported a number of germline FH mutations from MCUL patients. In this study, we report additional FH mutations in MCUL and FH deficiency patients. Mutations can readily be found in about 75% of MCUL cases and most cases of FH deficiency. Some of the more common FH mutations are probably derived from founding individuals. Protein-truncating FH mutations are functionally null alleles. Disease-associated missense FH changes map to highly conserved residues, mostly in or around the enzyme's active site or activation site; we predict that these mutations severely compromise enzyme function. The mutation spectra in FH deficiency and MCUL are similar, although in the latter mutations tend to occur earlier in the gene and, perhaps, are more likely to result in a truncated or absent protein. We have found that not all mutation-carrier parents of FH deficiency children have a strong predisposition to leiomyomata. We have confirmed that renal carcinoma is sometimes part of MCUL, as part of the variant hereditary leiomyomatosis and renal cancer (HLRCC) syndrome, and have shown that these cancers may have either type II papillary or collecting duct morphology. We have found no association between the type or site of FH mutation and any aspect of the MCUL phenotype. Biochemical assay for reduced FH functional activity in the germline of MCUL patients can indicate carriers of FH mutations with high sensitivity and specificity, and can detect reduced FH activity in some patients without detectable FH mutations. We conclude that MCUL is probably a genetically homogeneous tumour predisposition syndrome, primarily resulting from absent or severely reduced fumarase activity, with currently unknown functional consequences for the smooth muscle or kidney cell.

Guidelines for the management of bullous pemphigoid.

These guidelines have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines and a brief overview of epidemiological aspects, diagnosis and investigation. The guidelines reflect data available from Medline, Embase, the Cochrane library, literature searches and the experience of the authors of managing patients with bullous pemphigoid in special and general clinics for over 10 years. However, caution should be exercised in interpreting the data obtained from the literature because only six randomized controlled trials are available involving small groups of patients.

An interesting response to diphencyprone (DPC) sensitization on facial warts: review of DPC treatment for viral warts.

BACKGROUND: This paper highlights the sometimes impressive effect of diphencyprone (DPC) sensitization on warts resistant to other treatments and is interesting in view of the fact that all the warts apparently responded, despite only a very small area being treated. METHODS: A 31-year-old woman with a 5-year history of widespread facial plane warts that had proved resistant to repeated treatments with cryotherapy and topical preparations was sensitized to diphencyprone. RESULTS: After application of DPC to the warts within only a 1-cm(2) area of the face, all the facial warts became inflamed and resolved, including those not actively treated. Complete clearance occurred with no recurrence. CONCLUSION: DPC appears to be a valuable, safe and well-tolerated treatment for resistant viral warts and can be considered as a first line treatment. We review its use and action in this paper

Scleredema associated with paraproteinaemia treated by extracorporeal photopheresis.

Scleredema is a rare disease characterized by extensive cutaneous woody, non-pitting induration that spreads throughout the body. We describe a patient with scleredema associated with paraproteinaemia who was successfully treated by extracorporeal photopheresis.

The use of topical glycopyrrolate in the treatment of hyperhidrosis.

The use of an aqueous solution of 0.5% topical glycopyrollate was effective in the treatment of hyperhidrosis of the scalp and forehead after other treatments had proved ineffective; this appears to be the first report of its use in this condition.