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Cancer complicating chronic ulcerative and scarifying mucocutaneous disorders.

Skin affected by a burn cancer is scarred, ulcerated, and often appears as erythema ab igne clinically in adjacent skin. The latent period in burn scar malignancy is much longer for SCC than BCC. Malignant melanoma and various sarcomas are reported to arise in burn scars, too. The other extreme on the temperature scale can less often result in enough permanent acral damage that poor wound healing may eventually result in cancer, usually SCC. About 1% of patients with chronic osteomyelitis develop cancer, usually SCC in sinus tracts. As with tumors arising in burn scars and chronic leg ulcers of varied etiology, black patients are disproportionately overrepresented in osteomyelitic malignancy. In nearly all of the patients with radiation-induced skin cancer, concomitant radiodermatitis is present. As with burn scar and osteomyelitic cancer, x-ray related cancer has a long latent period. Similar to burn scar cancer, SCC predominates in osteomyelitis and occurs on the extremities. BCC, when it arises, is more common on the face and neck in burn- and radiation-induced tumors. Multiple tumors are frequent as is recurrence in x-ray malignancy. Mortality is high: one out of three to four patients with burn scar, osteomyelitic, and radiation cancer die of dermatosis-related malignancy. Recently, radioactivity-contaminated gold rings have been implicated in causing SCC. Carcinoma tends to occur in irradiated benign dermatoses whereas sarcomas tend to complicate irradiated malignancies. Stasis ulceration and anogenital fistulae may rarely lead to cancer, SCC in the former and adenocarcinoma in the latter. SCC can rarely develop in four related conditions (acne conglobata, dissecting perifolliculitis of the scalp, hidradenitis suppurativa, and pilonidal sinus) after a lengthy latent period; prognosis is poor with a high metastatic rate. A whole host of chronic cutaneous infections can lead to malignancy occasionally; these include lupus vulgaris, lymphogranuloma veverum, granuloma inguinale, leprosy, actinomycosis, and candidiasis. BCC more than SCC is known to complicate smallpox vaccination sites. Certain erosive and/or scarring dermatoses other than those mentioned above can be unusually affected by secondary malignancy. Discoid lupus erythematosus lesions often subjected to the carcinogenic effects of sunlight can degenerate into SCC in patients with either light or dark skin. In acrodermatis chronica atrophicans, a condition not often seen in the United States, the involved skin, particularly of the lower extremities, is susceptible to SCC, lymphoma, and BCC. Epidermolysis bullosa, especially the recessive dystrophic variant, can be complicated by SCC on affected mucous membrane and acral skin.(ABSTRACT TRUNCATED AT 400 WORDS)

Burns↗

[Steatocystoma multiplex conglobatum].

Two patients are described in whom steatocystoma multiplex conglobatum presented as recurrent axillary abscesses. This disease entity is rare and can easily be confused with hidradenitis suppurativa or acne conglobata.

Abscess↗

[Clinical and bacteriological evaluation of TMS-19-Q in superficial suppurative skin and soft tissue infection].

Clinical effectiveness of TMS-19-Q, a new macrolide antibiotic, was evaluated in superficial infectious diseases classified into 6 groups at 13 departments of dermatology. The results obtained were as follows: Final global improvement rating in 311 cases were excellent in 91, good in 158, fair in 45 and poor in 17 and the effective rate was 80.1%. Effective rates in each group were 71.1% in 1st group (folliculitis and acne pustulosa), 78.6% in 2nd group (furuncle, furunculosis and carbuncle), 100% in 3rd group (impetigo), 76.9% in 4th group (phlegmone, superficial lymphangitis, erysipelas and infectious paronychia), 88.7% in 5th group (inflammatory atheroma, subcutaneous abscess, hidradenitis suppurative and acne conglobata) and 77.3% in 6th group (secondary infection). Dominant strains isolated were S. aureus (40.7%), S. epidermidis (26.9%) and anaerobic bacteria (20.8%). S. aureus was frequently isolated from most of all disease. On the other hand, S. epidermidis and anaerobic bacteria were isolated mainly from 1st and 5th group. Optimum daily doses would be over 600 mg. Slight adverse reactions such as gastrointestinal disorders, eruption and malaise were observed in 12 cases.

Adolescent↗

[Anterior thoracic and intervertebral erosive joint diseases associated with palmoplantar pustulosis].

The authors report three cases of palmo-plantar pustulosis associated with articular signs: erosive arthritis of the right first sternocostal joint in 2 cases (without hypertrophy of the clavicle or the sternum) and atlanto-occipital arthropathy with marked neck stiffness in another case. The HLA phenotype of one case was: A2 - A9 - B14 - X - DR3 - DR4. A surgical sterno-costal biopsy revealed non-specific inflammatory lesions in 2 cases. In one of these cases, a Corynebacterium sp. was isolated. The clinical course was favourable in response to local antibiotic therapy in one case (follow-up of 8 years) and after treatment with non-steroidal anti-inflammatory agents in 2 cases (follow-up of one to two years). The skin biopsy revealed non-spongiform (and therefore non-psoriatic) unilocular pustulosis, distinguishing this non-bacterial pustulosis from pustular palmo-plantar psoriasis with which it is frequently confused. These cases are similar to the cases of "pustulotic arthro-osteitis" reported by Japanese authors (Sonozaki et al.), which appear to be rare in Europe. They seem to be an early form in a vast range of spondylo-arthropathies including rheumatism and acne conglobata. The aetio-pathogenesis of this syndrome is discussed; one of the cases is strongly suggestive of an infectious origin (Corynebacterium). These lesions do not appear to be a form of reactive arthritis, as the presence of HLA B27 is rare in both the European and the Japanese cases.

Adult↗

[Chronic arthritis in palmo-plantar pustulosis. Bacterid of Andrews].

Report of a 30 years old man story pustulosis palmaris et plantaris, spondylodiscitis L5-S1, right sterno-clavicular arthritis, right sacro-iliitis, and pubic symphysitis. Multiple bacteriologic investigations did not discover any infectious articular process. Histologic articular findings were inflammatory pattern, non steroid anti-inflammatory drugs corrected the arthritis. Anterior thoracic arthropathies are quasi-constant. Inflammatory spondylodiscitis are frequent, while sacro-iliac involvement is infrequent, this pattern distinguishing pustular arthro-osteitis from classic pelvispondylitis. Radiologic lesions are peculiar by predominance on one articular side at each site. Nosology is discussed. Slow infection by non virulent germs has been suspected , but seems improbable. This affection seems close seronegative spondylo-arthropathies and reactive arthritis, like acne conglobata arthritis for instance. Usual absence of HLA-B27 antigen must be noted.

Adult↗

[Reconstructive surgery of the anoperineal region].

The embryology and anatomy of the ano-perineal region explain the complex pathology which is difficult to treat. Six types of lesions are discussed. Congenital skin lesions: naevus or angiomas, requiring the same procedures of local autoplasty and graft as those of other regions, whenever they are extensive. Infectious lesions are dominated by Verneuil's disease, chronic hidradenitis or acne conglobata. Treatment involves wide excision which is sufficiently deep, followed by skin graft. Cancerous or pre-cancerous lesions. A distinction must be made between Bowen's disease and Paget's disease: the first is a precancerous lesion and the second is a sign of underlying carcinoma. The treatment is very different for the two conditions. Radiation dermatitis is not exceptional. Surgical treatment is required, but it is difficult. Radio-necroses can be repaired with epiplooplasty and a musculo-cutaneous flap from gluteus maximus. Post-traumatic and surgical sequelae sometimes need to be repaired by a plastic operation. Anal continence can be treated by surgical repair.

Anal Canal↗

Blood glutathione-peroxidase levels in skin diseases: effect of selenium and vitamin E treatment.

Blood glutathione-peroxidase (GSH-Px) was determined in 61 healthy subjects and 506 patients with various skin disorders. Depressed levels were observed in patients with psoriasis, eczema, atopic dermatitis, vasculitis, mycosis fungoides and dermatitis herpetiformis. Low values of GSH-Px were also found in some patients with pemphigoid, acne conglobata, polymyositis, rheumatoid arthritis, scleroderma and systemic lupus erythematodes. Vegetarian diet, malnutrition and alcohol abuse could possibly account for the low values in some patients. Fifty patients with low GSH-Px levels were treated with tablets containing 0.2 mg selenium as Na2SeO3 and 10 mg tocopheryl succinate. The GSH-Px levels increased slowly within 6-8 weeks of treatment. The clinical effect was encouraging and calls for controlled studies.

Adolescent↗

[Multiple hyperostosis with unilateral sacroiliitis. A new spondyloarthropathy].

Six patients (4 male and 2 female) presenting with an original, predominantly osseous disease are reported. The disease is characterized by a chronic inflammatory process affecting the thorax, the spine, the femur in 1 case and in all patients only one of the sacro-iliac joints. The condition had begun 11 to 28 years previously and had been active for 6 to 24 years. One case started with regressive polyarthritis. There was no visceral involvement. Radiograms showed bone condensation with hyperostosis, sometimes hypertrophic, unrelated to any articular space. Depending on each patient, the lesions involved the clavicles, the sternum, the posterior arch of the ribs, the vertebrae, the inferior femoral metaphysis. The only joints involved were the sacro-iliac and anubrio-sternal joints. The areas affected showed high radioisotope uptake. There was discrete biochemical inflammatory syndrome. Three of the 6 patients belonged to the HLA group B 27. The multiple biopsies performed showed no specific lesions. The disease proceeds by successive attacks lasting several months and responding poorly to anti-inflammatory drugs. One patient developed Crohn's disease, another, retroperitoneal fibrosis. In some respects, this condition is remindful of sterno-clavicular hyperostosis and of the osteo-arthropathy in palmoplantar pustulosis or acne conglobata. It seems to constitute a new variety of spondylo-arthropathy intermediate between the above-mentioned diseases and genuine ankylosing spondylitis.

Adolescent↗

[Perianal pyoderma].

Perianal pyoderma (hidradenitis suppurativa-acne conglobata) is a chronic disease which in its progressive form cannot be treated by conservative methods. Extensive suppurative foci must be radically excised incl. all systems of fistulas right down to the fascia and the defects which develop are allowed to heal per secundam or after several weeks they can at a different period of time be covered by a skin transplant. The authors present their 30-year experience with surgical treatment of 39 patients. Treatment was in all instances successful after radical surgical excision. All these patients have fully recovered as regards work capacity as well as social aspects.

Adolescent↗

[Differentiation of various Propionibacterium species from acne-vulgaris efflorescences].

Samples of pustules taken from 37 patients with acne (a. vulgaris, a. papulopustulosa, a. conglobata) were examined using different culture mediums for the growth of anaerobic to microaerophilic coryneforms. We identified 46 isolates of Propionibacteria which were tested according to their morphological, biochemical, serological, and phagelysotopic reactions. Three types of Propionibacteria were found: P. acnes (33), P. granulosum (11) and P. avidum (2). Nine samples showed P. acnes combined with P. avidum, four samples from patients with acne conglobata showed P. granulosum or P. avidum only. Tests from 37 patients are too limited to reach definite conclusions. We are not able to suggest any correlation between type and degree of clinical acne, and the isolated Propionibacteria. These data indicate that pustules from patients with acne of all clinical degrees can contain P. acnes as well as other anaerobic coryneforms. Therefore, other species of anaerobic bacteria, particularly those of the "propionibacteria type", should be included in any discussion about the aetiological factors of acne.

Acne Vulgaris↗

A study of musculoskeletal manifestations in 12 patients with SAPHO syndrome.

Synovium and synovial fluid findings in SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome have not been well characterized, and only a few patients have been described in the Americas. We describe clinical, pathologic, and synovial fluid findings in 12 patients with the SAPHO syndrome: hidradenitis suppurativa (7), acne fulminans or conglobata (3), acneiform folliculitis (1) and palmoplantar pustulosis (1). Routine synovial fluid studies were performed in 6 patients, and light and transmission electron microscopic studies were performed in synovium in 2 patients and in bone in 1. The most common musculoskeletal manifestations included erosive or non-erosive oligoarthritis involving metacarpal phalangeal (MCP) and metatarsal phalangeal (MTP) joints as seen in 9 patients, sclerosis of the sacroiliac joints as seen in 5 patients, and osteitis pubis as seen in 1. Three patients had signs of skeletal hyperostosis. The patients with acne fulminans and acneiform folliculitis had chronic aseptic multifocal osteomyelitis. Synovial fluid was sterile in 7, mildly inflammatory in 5, and highly inflammatory in 2. Electron microscopic studies of synovium in 2 patients and of bone in 1 were not useful to detect microorganisms. Three African-American patients with hidradenitis suppurativa presented with pyoderma gangrenosum, and 2 of them had leukocytoclastic vasculitis, and a life threatening course unresponsive to antibiotics, corticosteroids and immunosuppressive therapy. SAPHO in the Americas is most severe in African-Americans with hidradenitis suppurativa, and it presents with heterogeneous musculoskeletal and cutaneous manifestations including erosive polyarthritis or oligoarthritis with nonspecific mild inflammatory fluid. Leukocytoclastic vasculitis and recalcitrant pyoderma gangrenosum were seen in 2 of our patients with the most severe hidradenitis suppurativa. SAPHO syndrome may present with clinical manifestations similar to those seen with seronegative spondyloarthropathies, but it has distinctive cutaneous, radiographic articular, and bone manifestations. Sites of chronic infection need aggressive antibiotic therapy and may need surgical resection.

Journal Article↗

The acquired hyperostosis syndrome: a little known skeletal disorder with distinctive radiological and clinical features.

The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The predominant location of lesions is the sternocostoclavicular region (approximately 80% of patients), less frequent are involvement of the spine, pelvis, and appendicular skeleton. In 20%-60% of cases AHS is associated with palmoplantar pustulosis, psoriasis, or severe acne (acne fulminans or conglobata). The X-ray appearance of AHS is a more or less homogeneous increase in density with blurred margins, which on scintiscan with labeled phosphate compounds is associated with intense accretion of tracer. These features are associated with a variable increase in the acute phase reactants and a conspicuously low increase, if any, in serum alkaline phosphatase. The therapeutic modalities which have been used so far are entirely symptomatic. Long-lasting improvement has been reported following percutaneous anti-inflammatory radiation therapy.

Acne Vulgaris↗

Coincidence of Behçet's disease and SAPHO syndrome.

A young woman presented with deteriorated visual acuity due to acute retinal vasculitis. The diagnosis of Behçet's disease was made on the basis of the ophthalmologic findings and recurrent oral and genital aphthous ulcerations. In the past, this young woman had been operated on several times because of progressive aseptic osteitis of the right clavicle. The actual examination revealed chronic osteitis, osteosclerosis and hyperostosis of the anterior chest wall, as well as severe acne papulopustulosa et conglobata. These findings resulted in an additional diagnosis of SAPHO syndrome. This report is the first description of Behçet's disease occurring in association with SAPHO syndrome, both fulfilling the diagnostic criteria.

Acquired Hyperostosis Syndrome↗

[Therapy of severe acne and acne rosacea with oral 13-cis-retinoic acid (Isotretinoin)].

Forty patients suffering of different forms of acne (papulo-pustular, nodulo-cystic, conglobata, rosacea), all in severe conditions and non-responding to other treatments, have been administered 13-cis-retinoic acid p.o. The treatment resulted in a complete and ultimate healing in 31 pts (77.5%) and a marked amelioration in the remaining 9 cases. The initial drug dosage was 40 mg/die (an average of 0.66 mg/kg/die) but it was reduced along the treatment to 2.5 mg/die, a still effective dose. The average treatment duration was 24 weeks (range: 12 to 40). The tolerance was generally excellent, but some adverse effect have been recorded, mainly localized in the skin and mucosa. Increases of total serum cholesterol (66% of the cases) and of triglyceride (72%) level have been observed. This effect was reversible at the end of the treatment. As a conclusion we can confirm that the 13-cis-retinoic acid is the most effective drug for the pharmacotherapy of severe acne.

Acne Vulgaris↗

[Inhibition of the function of the neutrophil granulocyte caused by autologous serum from patients with acne vulgaris].

Sixteen patients with acne vulgaris were studied. All patients had severe inflammatory lesions, nodulocystic and conglobata. Of them, 12 was male and 4 female, their age was between 16 and 24 years old. In all the cases laboratory test to evaluate chemotactic, nitroblue-tetrazolium and lysosomal activity of granulocyte were performed. The protocol included in vitro incubations with and without autologous serum for considered their action on the phagocytic cell. The results shows an inhibitory action of the autologous serum in chemotactic (d-mean, 34.25%; SEM, 2.39, p less than 0.01), nitroblue-tetrazolium test (d-mean, 21.06%; SEM, 2.85, p less than 0.05), and lysosomal activity (d-mean, 21.43%; SEM, 2.80, p less than 0.05). The authors suggest that granulocyte alterations that occur in inflammatory forms of acne vulgaris is a secondary phenomenon may be caused by seric IgE or vasoactive substances released during immune responses.

Acne Vulgaris↗