Pyoderma gangrenosum (PG) is a chronic inflammatory disease of unknown cause that presents as an inflammatory and ulcerative disorder of the skin. PG is often associated with an underlying systemic disease. However, the frequencies of the underlying diseases are unclear in Japanese patients. In this retrospective, observational study, all patients diagnosed with PG who visited dermatology departments of nine regional hospitals in and around Ibaraki Prefecture were collected from 1982 to 2011 or 2014. The diagnoses of PG were based on the characteristic clinical and histological appearances and ruling out of infection. Sixty-two PG patients, including 29 males and 33 females, were identified. The ages of onset were 16-89 years, and the mean age was 50.2 years. Fifty (80%) of the 62 patients presented with an ulcerative PG, and the lower leg was the most common site (74%). Forty-six (74%) PG patients had underlying diseases. The most frequent was ulcerative colitis (32%), followed by myelodysplastic syndrome (11%), rheumatoid arthritis (6%) and aortitis syndrome (5%). For treatment, 54 cases (87%) received systemic corticosteroids and 10 received additional treatment with cyclosporin. There was no significant correlation between underlying diseases and response to the initial treatment. Multivariate analysis revealed that the number of affected sites negatively correlated with successful initial treatment. Fifteen (24%) of the 62 cases relapsed. In conclusion, ulcerative colitis and hematological disorders were frequently associated with PG while approximately a quarter of the cases were idiopathic.

No abstract

strongly indicates that lipo-PGE 1 treatment of livedoid vasculitis with cryoglobulinaemia is effective and safe.There are two known mechanisms by which cryoglobulins can result in disease. The first is by precipitation within the vascular lumen, typically cold induced, with hyaline plug formation and minimal early-phase inflammation. Typical clinical lesions would be minimally inflammatory cutaneous infarction with or without associated livedo reticularis, or noninflammatory retiform purpura. The second mechanism is that of immune complex vasculitis. As nearly all type II (monoclonal component, usually IgM; polyclonal component, usually IgG) and III (one or more polyclonal components) cryoglobulins are immune complexes, they should all be capable of inducing an immune complex vasculitis, although many do not. Histological examination of an early purpuric lesion in our patient clearly showed cryoglobulin thrombus formation within the dermal vessels. The scarcity of polymorphonuclear leucocytes and leucocytoclasia among the histopathological findings argue against a primary vasculitic process. On the other hand, histology of a recurrent painful nodule revealed leucocytoclastic vasculitis. This is considered to be a secondary event rather than the primary event involved in the pathogenesis. These features favour a thrombo-occlusive vasculopathy rather than a vasculitis per se. Cryoglobulins have been implicated in intravascular thrombotic processes with secondary leucocytoclastic vasculitis that can result in skin infarction and necrosis.In conclusion, we believe that lipo-PGE 1 treatment combined with low-dose oral corticosteroid effectively eliminated cryoglobulins and improved leg purpura and skin ulcers in our patient with livedoid vasculitis. Further studies will be needed to clarify the clinical application of lipo-PGE 1 , but we expect that lipo-PGE 1 treatment will be effective among selected patients with cryoglobulinaemic vasculitis. References1 Acland KM, Darvay A, Wakelin SH, Russell-Jones R. Livedoid vasculitis: a manifestation of the antiphospholipid syndrome? Br J Dermatol 1999; 140:131-5. 2 Boyvat A, Kundakci N, Babikir MO, Gurgey E. Livedoid vasculopathy associated with heterozygous protein C deficiency. Br J Dermatol 2000; 143:840-2. 3 Dispenzieri A, Gorevic PD. Cryoglobulinemia. Hematol Oncol Clin North Am 1999; 13:1315-49. 4 Dammacco F, Sansonno D, Piccoli C et al. The cryoglobulins: an overview. Eur J Clin Invest 2001; 31:628-38. 5 Browning CE, Callen JP. Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia. Arch Dermatol 2006; 142:75-8. 6 Ahn JH, Kim MR, Kim HC. Therapeutic effect of lipoprostaglandin E 1 on sudden hearing loss. Am J Otolaryngol 2005; 26:245-8. 7 Koga T, Az-ma T, Yuge O. Prostaglandin E 1 at clinically relevant concentrations inhibits aggregation of platelets under synergic interaction with endothelial cells. Acta Anaesthesiol Scand 2002; 46:987-93. 8 Gruss JD. Effects of adjuvant PGE 1 therapy following profundaplasty in pa...

A 28-year-old Japanese woman with hereditary complement (C9) deficiency and dermatomyositis is reported. She had a 3-year history of facial erythema and a 1-month history of progressive muscle weakness. Clinical and laboratory findings were suggestive of dermatomyositis; muscle biopsy confirmed an inflammatory myopathy. An unexpected finding, however, was the low titre of serum haemolytic complement (CH50). Treatment with prednisolone resulted in marked clinical improvement but did not affect the CH50 titre. Further investigation revealed a selective and total absence of the ninth complement component (C9), with direct DNA sequence analysis revealing a non-sense mutation at Arg95 of the C9 gene. This case demonstrates that the muscle lesions of dermatomyositis can occur in the presence of a complement defect that would prevent the formation of the C5b-9 membrane attack complex.

No abstract