Treatment of pyoderma gangrenosum with intravenous immunoglobulin

BACKGROUND: Intravenous immunoglobulin (IVIG) is increasingly being used to treat inflammatory and autoimmune disease. OBJECTIVES: To elucidate the efficacy of IVIG as an adjunct treatment for pyoderma gangrenosum (PG). PATIENTS/METHODS: Ten patients with PG were treated with IVIG at Johns Hopkins Department of Dermatology. All patients had severe mutilating and/or refractory disease requiring multi-agent therapy. The charts were reviewed retrospectively. RESULTS: Seven of the ten patients had clearance of PG lesions in the setting of IVIG and six of these patients maintained efficacy with repeated IVIG treatment. Five patients complained of nausea with treatment, and in one case nausea was severe and intractable. One patient developed an immune reaction requiring diphenhydramine and methylprednisolone and another experienced aseptic meningitis. CONCLUSIONS: IVIG may be an effective adjuvant in the treatment of PG and has an acceptable side-effect profile. Randomized, placebo-controlled, double-blinded trials are needed to confirm this hypothesis.     

Facial Sweet's syndrome mimicking rosacea fulminans

A 36-year-old man presented with a non-pruritic, erythematous facial rash with peri-oral and peri-orbital sparing. The initial clinicopathological diagnosis was rosacea fulminans, which was treated with 25 mg oral prednisolone and cephalexin. The patient re-presented 1 week later with exacerbation of his rash in addition to constitutional symptoms of fever and malaise. A further skin biopsy was taken and the marked neutrophilic infiltrate in the absence of vasculitis made the diagnosis of Sweet's syndrome (acute febrile neutrophilic dermatosis). High-dose prednisolone (50 mg daily), topical hydrocortisone cream and ichthammol in zinc ointment were commenced with rapid clinical improvement. This case highlights the importance of considering Sweet's syndrome as a differential diagnosis when presented with a facial eruption.     

Portable negative‐pressure wound therapy for pyoderma gangrenosum: Report of two cases

Pyoderma gangrenosum is a chronic non‐infectious neutrophilic dermatosis that causes undermining ulcers. Topical therapies for the deep ulcers of pyoderma gangrenosum have not been established. To investigate whether negative‐pressure wound therapy is effective for a pyoderma gangrenosum ulcer, we used the PICO single use negative‐pressure wound therapy system (Smith & Nephew, London, UK) for two pyoderma gangrenosum patients. In these cases, the ulcers decreased in size and necrolytic tissue was removed notably. Moreover, there were no secondary infections nor was there Koebner phenomena. Our cases suggest that portable negative‐pressure wound therapy can be a treatment option for deep, intractable ulcers caused by pyoderma gangrenosum. Because portable negative‐pressure wound therapy devices afford increased mobility to patients, they can give the patient a better quality of life than standard negative‐pressure wound therapy systems do.     

Pyoderma gangrenosum associated with sclerosing cholangitis, type 1 diabetes mellitus and ulcerative colitis.

We describe the case of a 22-year-old black female with type 1 diabetes mellitus diagnosed when she was 12 years old. She first presented (March 1994) with pustules and ulcerations on the upper and lower limbs, trunk and scalp at the age 17. The diagnosis of pyoderma gangrenosum was made. Since presentation, changes in liver function were detected and subsequent study led to the diagnosis of sclerosing cholangitis. The diagnosis of ulcerative colitis was made after colonoscopy. Partial response was obtained with minocycline and clofazimine, but treatment with 5-aminosalicylic acid achieved no improvement of the ulcerations. Liver transplantation, followed by immunosuppressive therapy led to complete regression of the cutaneous lesions.     

Dapsone in rosacea fulminans

Rosacea fulminans is a rare disease with female predominance characterized by abrupt onset of pustules, papules, and confluent nodules on the face. The conventional treatment consists of systemic glucocorticoids and isotretinoin. We present the case of a 56-year-old woman with a marked facial papulopustular eruption that had followed an initial period of severe seborrhoea. Conventional treatment produced no clear improvement. Dapsone treatment achieved complete healing in 5 weeks.     

Sporotrichosis masquerading as pyoderma gangrenosum: case report and review of 19 cases of sporotrichosis

We present the case of a 59-year-old woman who had large ulcerations on her right leg that were diagnosed initially as pyoderma gangrenosum and treated with three immunosuppressive agents (cyclosporin, prednisone and azathioprine) for 6 months. Results of a biopsy at 6 months showed numerous cigar-shaped bodies consistent with Sporothrix schenckii; identification was confirmed by tissue culture. A retrospective review was performed for all cases diagnosed as sporotrichosis from tissue culture or biopsy specimens at the Mayo Clinic. Nineteen cases were identified. The present case was the only one in which fungal organisms were visible on histological examination. The present case emphasizes the importance of making a definitive histological diagnosis in unusual ulcer cases or in suspected cases of pyoderma gangrenosum before the initiation of immunosuppressive therapy. The large number of cigar-shaped bodies in the tissue is a rare finding in sporothrix infection and has been reported in only two cases previously.     

Pyoderma gangrenosum and underlying diseases in Japanese patients: A regional long‐term study

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.