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Superficial granulomatous pyoderma: who are you?

In this issue, Thami et al . describe a case of a superficial, sterile, ulcerative process with a granulomatous pathology. They report this case as superficial granulomatous pyoderma (SGP), but also suggest a new name for this rare and enigmatic condition of the skin. What is SGP? Does it have a specific clinical and pathological definition and why such a long and cumbersome name? First ‘named’ by Wilson-Jones and Winkelmann, 1 this process most likely coexisted within the spectrum of sterile pyodermatous processes, most often pyoderma gangrenosum (PG) but also perhaps lumped together with others. The authors felt it important to separate it from classic PG due to the variance in clinical pattern, characteristic histopathological changes, lack of serious associated diseases and a favourable prognosis with conservative treatment. Since its original description other cases have been reported, but it remains an uncommonly reported variant of PG. Although it shares several clinical features with classic PG, there are some notable differences, including a much slower progression, less tendency for undermining of the edges and lack of the purplish advancing rim of PG. Both conditions are marked by pathergy, and SPG seems to have a particular propensity for surgical wounds and perhaps injured hair follicles. The cribiform healing and scarring pattern are common to both. The ‘zonal’ granulomatous pattern microscopically together with plasma cell inflammation has been said to be characteristic for this condition. Although characteristic this pattern is not specific as plasma cells can actually comprise a good portion of the infiltrate in several disorders, including connective tissue diseases, infections, lymphoproliferative diseases and other granulomatous diseases. Zonation of the granuloma can also be seen in infectious diseases. It is essential that infection is ruled out by appropriate cultures and tests. In most reported patients there have been no serious associated diseases, but there are exceptions and so each patient must be evaluated carefully for the presence of such diseases, especially ‘silent’ inflammatory bowel disease and paraproteinaemias. SGP most often responds to conservative treatments thus sparing the patient exposure to immunosuppressive medications in many cases. Oral antibiotics, sulphones and intralesional injections of corticosteroids have all been successful. There may be a part for topical immunosuppressants such as tacrolimus used either alone or in combination with other agents. There is insufficient experience to say there is a treatment of choice for SGP. And now the name. SGP is both descriptive and also not entirely specific. It is best not to delete ‘granulomatous’ from the name as this would raise confusion with the superficial forms of pyoderma associated with myeloproliferative disease or with ‘atypical’ Sweet’s syndrome. It appears clinicians are able to recognize this disorder based on its clinical and histopathological characteristics. Once the mechanism of disease has been worked out then the terminology should go the way of other non-specific names such as ‘lethal midline granuloma’ or ‘malignant pyoderma’. Until that time, we can live with the shortcomings of this name.