Search

Pyoderma gangrenosum is a very strange disease usually diagnosed on clinical grounds only and without any characteristic biological disturbance. It must be neither missed nor diagnosed too easily at the expense of vascular or infective ulcerations which are much more frequent. Its physiopathology remains shrouded in mystery since the abnormalities that have been found were extremely varied and sometimes conflicting, and were observed in short series. Thus, pyoderma gangrenosum appears as a syndrome which if often causeless or due to multiple diseases and might well be split into different entities in the forthcoming years. Despite its obscure pathogenesis, most patients can be cured by systemic corticosteroid therapy, sulfones or clofazimine. The main point of interest of that disease is that in almost 50 percent of the cases it is associated with a severe underlying pathology.

Pyoderma gangrenosum is a rare skin disease of unknown pathogenesis associated, in almost 8 out of 10 cases, with a systemic disease, notably enterocolitis or hemopathy. We report the case of a 57-year old man who had been presenting with pyoderma gangrenosum for 5 years when he developed a rheumatoid-like seronegative chronic polyarthritis. The occurrence, some time later, of a supraclavicular adenopathy led to the diagnosis of Hodgkin's disease. To our knowledge, the pyoderma-chronic polyarthritis-Hodgkin's lymphoma association has never been reported. Treatment of the lymphoma resulted in complete disappearance of cutaneous and articular symptoms. The fact that neither the skin disease nor the polyarthritis recurred during a 3-year follow-up after treatment was discontinued, incites us to discuss the possibility that the pyoderma and the polyarthritis observed in this patient were neoplastic diseases.

Pyoderma gangrenosum (PG) is an uncommon ulcerative disease of the skin. The cause is unknown but the condition is often associated with other diseases such as rheumatoid arthritis, ulcerative colitis, Crohn's disease or monoclonal gammopathy. The association between PG and haematological malignancies (acute leukaemia, Myeloproliferative disorders) is infrequent. Two cases of PG associated with haemopathy are described; one had primary thrombocythaemia and the other, acute myeloblastic leukaemia following for myeloma. The significance of this association is discussed in the light of other observations previously reported in the literature.

The authors report a case of pyoderma gangrenosum associated with a positive B27 pelvispondylitis. The search for a hemorrhagic rectocolitis, possible link between the two syndromes, revealed to be negative. Pelvispondylitis is an unusual complication of pyoderma gangrenosum. Review of the literature shows only one case of sacroiliitis, beside seronegative polyarthritis unrelated to HLA B27.

The pyodermatitis proliferation around or on chronic leg ulcers derived from bone infection or of varicose origin is rare. The author, who has only seen 7 cases over the last ten years, stresses the importance of making an aetiological diagnosis which often has to be based on repeated histological examinations. Two clinically similar features have, in fact, very different natures and prognoses: one, pseudoepitheliomatous pyodermatitis (5 cases), is only a benign inflammatory reaction which is easily cured by simple curetage; the other, spiny cell epithlioma (2 cases), requires wide excision, or even amputation of the limb.

Three cases and a pathogenic discussion. We report three cases of clinically typical pyoderma gangrenosum (PG) remarkable for the presence, at histology, of inflammatory vascular lesions of varying intensity. Case No. 1. A 15-year old boy without previous pathology was admitted for necrotic cutaneous lesions typical of PG on both legs. Biopsy in the peri-ulcerous rim showed cellular infiltration of the dermis, principally by granulocytes, associated with granulocytic vasculitis with fibrinoid necrosis (fig. 1 and 2). Laboratory examinations only showed a decrease of CH 50, and attempts at making an aetiological diagnosis of this case of PG met with failure. PG regressed under treatment with disulone combined with topical corticosteroid therapy. Case No. 2. A 62-year old woman with no previous severe disease was admitted for PG of her right leg at the ulcero-necrotic stage (fig. 3). Biopsy in the PG rim showed infiltration of the dermis, predominantly by granulocytes, associated with granulocytic vasculitis and fibrinoid necrosis of the walls of small vessels (fig. 4). Investigations in search of a cause revealed benign thyroid gland hyperplasia. PG was cured after a 5-week oral treatment with minocycline. Case No. 3. This 63-year old woman with arterial hypertension was admitted for PG of both legs. Some lesions at a very early stage were bullous (fig. 5). Biopsy in the peripheral swelling showed polymorphous cellular infiltration of the dermis with a predominance of granulocytes. These cells infiltrated the vessels the walls of which were turgid but without fibrinoid necrosis (fig. 6).(ABSTRACT TRUNCATED AT 250 WORDS)

Two new cases of pyoderma gangrenosum with chronic myeloid leukemia are described and added to three others of the literature. Pyoderma gangrenosum is often bullous, painful and progresses quickly. It seems to have a bad prognosis in myeloid leukemia as in polycythemia vera, in which it appears lately as an evolution to myelofibrosis or acute leukemia transformation. Chemotactic disorders of polynuclear may be the main etiological factor in these cases.

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)

Fourteen cases of pyoderma gangrenosum are reported. Twelve were associated with various diseases: ulcerative colitis, Crohn disease, colic tumor, rheumatoid arthritis, chronic myeloid leukemia, agammaglobulinemia, respiratory infections (2 cases) and post surgical intervention. In 2 cases pyoderma gangrenosum appeared to be primary. The clinical characteristics of the affection make it a very particular type of ulcer: pustular onset, frequent localization to other than leg, rapid eccentric spread, frequent associated morbidity (mainly digestive and hematologic). The pathogenesis is still unknown and the treatment is not well codified.

Pyoderma gangrenosum (PG) is a rare condition remarkable for its association with particular diseases, notably haemopathies and gastrointestinal diseases. As regards haemopathies, the associations most frequently encountered are with myeloid malignancies and monoclonal dysglobulinaemia. The association of PG with mainly inflammatory digestive tract diseases is also classical. The lack of publications concerning gastric atrophy and the dual haematological and gastric pathology which characterizes our case have prompted us to report it. A 60-year-old woman without significant history was admitted for PG on both knees, following vesiculo-bullous lesions. Laboratory examinations detected a normochromic anaemia tending to be macrocytic, a marked inflammatory syndrome and a monoclonal lambda light chain IgA peak at protein immunoelectrophoresis. Bone marrow biopsy, skeletal radiography and a search for Bence-Jones proteinuria were normal or negative. Colonoscopy showed no abnormality, but fibroscopy of the upper digestive tract revealed a severe gastric atrophy en plaques. Serum vitamin B12 level was moderately low, but there was no other sign of pernicious anaemia. After one month treatment with systemic corticosteroids, healing was obtained under replacement vitamin therapy. PG recurred a few months later; serum vitamin B12 level was normal, and the lesions healed after systemic corticosteroid treatment. In non-myelomatous dysglobulinaemia IgA is frequently found and there is no light chain predominance. PG often precedes dysglobulinaemia. Evolution towards a true myeloma seems to be exceptional. In a recent publication, 17 cases of association between PG and myeloma were mentioned, the IgA type being most common. Protein electrophoresis is indispensable in patients with PG. Five cases of congenital hypogammaglobulinaemia have been recorded, including three with IgA deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

We report one case of amicrobic pustulosis cured with Dedrogyl (25-hydroxycholecalciferol), active metabolite of the vitamin D. The importance of our case stems from the physiopathologic study of this cure. The patient is a 16-year-old boy suffering from a hypoparathyroidism since his younger days. Consequently, he shows a hypocalcemia and convulsive seizures due to the hypoparathyroidism. Those seizures had been wrongly attributed to a primary epilepsy of a neurologic origin and a treatment with Phenobarbital had been instituted. In fact, they were side-effects of the hypoparathyroidism and the Phenobarbital had only aggravated the hypoparathyroidal hypocalcemia as a result of its effect on the metabolism of the vitamin D (deviation of this metabolism by enzyme induction at the level of the liver). The Dedrogyl (25-hydroxycholecalciferol) has restored a normal phospho-calcium balance and the hypocalcemia has disappeared as well as the convulsive seizures which, one year later, had not reappeared while they had previously been continual. And above all, standing back one year, we have recorded a complete cure of the amicrobic pustulosis on account of the Dedrogyl.

Pyoderma gangrenosum developed in a 45-year-old caucasian woman who had an 18 year past history of seronegative polyarthritis without any visceral or haematologic associated abnormalities. A literature review revealed only 12 reported cases. Extensive laboratory evaluation failed to detect alterations of the immune functions. The presence of a vasculitis with C3 deposits suggested that levamisole may have played a role in eliciting the lesion. Clofazimine, administered for a period of one month, was a relative failure.

The authors report three personal cases of phagedenic pyoderma associated with hemopathy. Studying twenty-five other cases, described in the reviews, the question can be debated on three levels: 1) The clinical characteristics of hemopathic pyoderma remain non-specific. However blisters, pustules, and even vegetating lesions occur very often; 2) the etiology of hemopathy is subject to change; acute leukaemia or myeloproliferation syndrome. During the polyglobulars associated with pyoderma, anemia and myelofibrosis appear quite constantly; 3) finally on the pathogenic level, the recent works tend towards the hypothesis of a damage in the functioning of the polymorphonuclears hence displaying the increase in the inflammation during phagedenic pyoderma.

Three cases of pyoderma gangraenosum were associated with Takayasu arteritis (pulseless disease), characterized by disappearance of pulse, hypertension and/or aortic insufficiency. Another 11-year-old boy, suffering from a serious congenital heart malformation, presented skin lesions of pyoderma gangraenosum. In spite or rather low incidence of association of ulcerative colitis (10.4 p. 100), Japanese cases show frequent association of cardiovascular diseases, especially Takayasu arteritis (27.1 p. 100).

A 67-year-old woman suffered from an ovarian carcinoma with lymph nodes metastasis. During 3 years, she was treated with alkylating agents (Melphalan). At the end of therapy, no recurrence was observed. Two years later, she developed concomitantly pyoderma gangrenosum and acute myelomonocytic leukaemia. Death occurred rapidly. The association between pyoderma gangrenosum and acute leukaemia is discussed in the light of 16 cases previously reported in the literature. In this case, an induction of leukaemia by cytostatic drugs seems likely. The authors conclude that pyoderma gangrenosum may be considered as a cutaneous signs of acute leukaemia.