TO THE EDITOR

A 28 years old man came to our dermatology clinic with chief complain of painful leg ulcers from 1 year ago. During the last year, he received different kinds of antibiotics and topical creams but he did not get any improvement. In physical examination we found nodulocystic acne with hypertrophic scars on his face, chest and proximal upper extremity (Figure 1& Figure 2).

In addition to this, we found inflammatory nodules with chronic drainage in the axilla and groin compatible with hidradenitis suppurativa (Figure 3).There were three ulcers on the pretibial area of both legs. The ulcers had purulent base with irregular, undermined borders (Figure 4 & Figure 5).

We made a biopsy from edge of one of the ulcers and the pathology was compatible with pyoderma gangrenosum (Figure 6 & Figure 7).

The family history of pyoderma gangrenosum, hidradenitis suppurativa, crohn’s disease and ulcerative colitis was not relevant in the first-degree relatives of the patient. Routine and immunological investigations were normal or negative, except for an increased C-reactive protein and anemia. In this setting we made a diagnosis of PASH syndrome.

PASH syndrome is a rare autoinflammatory skin disease, which has been recently described by Braun-Falco M. et al. [1]. This syndrome is clinically characterized by Pyoderma gangrenosum, acne, and suppurative hidradenitis, which is similar to PAPA syndrome but patients who suffer from PASH does not show arthritis and visceral involvement in contrast to patients who suffer from PAPA and aseptic abscesses syndromes. The only genetic explanation of the disease was considered as an increased number of CCTG microsatellite repeats in the PSTPIP1promoter region [1]. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms [2]. One patient was treated with the interleukin (IL)-1 receptor antagonist anakinraand responded well, although without complete remission [1]. In this case, we recommended him to use Infliximab, but we lost him for follow up.

Conclusion

The term autoinflammatory skin disease comprises a range of disorders which all caused by or associated with mutations of genes regulating innate immunity. Adequate management starts with an early diagnosis which requires recognition of rare entities such as PASH syndrome that we summarized here. Appropriate treatment may prevent undue medical expenses and minimize organ damage.

1. Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T (2012) Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)--a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol 66: 409-415.
2. Staub J, Pfannschmidt N, Strohal R, Braun-Falco M, Lohse P, et al. (2014) Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. J Acad Dermatol Venereol.