Acute ulcerative acne conglobata (acne fulminans) with erythema nodosum.
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Acne conglobata, a severe form of acne vulgaris, often produces pronounced disfigurement. We describe a 23-year-old man with acne conglobata. Although this type of acne is often more marked on the back rather than on the face, our patient was unusual in that it was localized mainly to the cheeks. Conventional medical therapy was ineffective, or contraindicated. Radical surgical excision with subtotal removal of cheek skin and subcutaneous tissue was performed with an excellent result.
Acne conglobata - hidradenitis suppurativa - as a particular form of acne vulgaris is, in its pronounced, chronic progressive form, incontrollable by conservative means. Smaller foci of infection need only a local excision of skin and subcutis followed by primary wound closure or secondary healing. When large areas are involved, a generous excision including removal of the fistula system is followed by a 1-3 week intermediary phase with hydrotherapy and antiseptic local treatment. Clean granulation is waited upon and covered in a second session with a skin grafting. In the area of joints, a third session with flap-plastic under sterile conditions is necessary, if limitations to motility are present or are threatened by scar contracture. From 1975-1982, 14 patients with pronounced acne conglobata were successfully treated by a surgical procedure in multiple sittings. All patients could, in this manner, be rehabilitated both professionally and socially.
Familial acne conglobata is more widespread and severe than "ordinary" acne conglobata and involves unusual areas such as the antecubital fossae and ankles. Of six members in one family with familial acne conglobata, two developed large and aggressive, well-differentiated squamous cell carcinomas within their disease. One had destruction of a large part of the right cranial vault; the other had most of his second cervical vertebra destroyed by metastatic squamous cell carcinoma. Isotretinoin controlled the granulomatous acneiform lesions but had no effect on the cancers.
Acne conglobata is an uncommon nodulocystic condition that is often resistant to therapy. This disorder typically begins in adulthood and presents as numerous comedones, papules, pustules, nodules, abscesses, and draining sinus tracts involving the chest, back, and buttocks. These lesions frequently become secondarily infected with gram-positive bacteria and often heal with scarring. Pathology usually reveals inflammatory infiltrate around follicles, which can often disrupt the normal dermal architecture. Acne conglobata is particularly disfiguring and socially detrimental to patients because of its chronicity, severity, and treatment challenge.
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Acne conglobata has been reported to be more common and more severe in XYY chromosomal aberration than in normal subjects. Whereas only exceptional cases have been observed in the more common XXY Klinefelter karyotype. We report a 17-year-old male with both Klinefelter's syndrome and acne conglobata, spreading from face to upper arms, trunk, buttocks and upper legs. The serum level of testosterone was normal, whereas luteinizing hormone and follicle-stimulating hormone were elevated. Skin lesions responded well to a combined therapeutic regimen including oral isotretinoin and prednisolone. An overview of severe acne in relation to chromosomal abnormalities is given and diagnostic and therapeutic options discussed.
Hidradenitis suppurativa and acne conglobata are well-described chronic dermatologic diseases. Although the exact incidence of these disorders is unknown, both are relatively uncommon conditions. The incidence of spondyloarthropathy is less than 1% in the general population. Therefore, a triad of hidradenitis suppurativa, acne conglobata and spondyloarthropathy is a rare syndrome described only in a few case reports in the literature. We report a case of hidradenitis suppurativa and acne conglobata associated with spondyloarthropathy.
A 24-year old man developed a bilateral sacroiliitis in association with acne conglobata. The acne conglobata was followed after the use of prednisone during 9 months. The prednisone was taken for a severe form of sarcoidosis. This may be a new instance of a relatively uncommon and poorly understood relationship between a particular form of acne and associated arthritis.
46 patients suffering from severe forms of acne conglobata were treated with 13-cis-retinoic acid. The initial dosages of 40, 60 or 80 mg of 13-cis-retinoic acid per day were adapted according to the success of therapy or to severe side effects. Pustules and papules responded promptly followed by the reduction of nodes and cysts. The lesions of the face responded quicker than those of chest and back. After 6 months of therapy, the overall reduction of all acne lesions was 94.4% for the face and 85.8% for chest and back. Thus, the out-standing efficacy of 13-cis-retinoic acid in severe forms of acne conglobata has been documented once more.
We evaluated 10 patients with hidradenitis suppurative or acne conglobata who developed arthritis. In contrast to patients with acne fulminans and arthritis, all our subjects were adults over 22 years of age; nine were black; and four were women. Nine patients had episodic inflammatory oligoarthritis affecting mainly larger joints of the upper and lower extremities. Eight patients had roentgenographic evidence of peripheral arthritis, four with erosions. Nine had clinical axial arthropathy, but roentgenograms showed abnormalities of the axial skeleton in all 10 patients. Pyoderma gangrenosum, erythema nodosum, conjunctivitis, urethritis, and oral and penile ulcers occurred in some patients. Rheumatoid factor was negative in all patients; the erythrocyte sedimentation rate was elevated in nine; five had chronic anemia; four had circulating immune complexes; and complement components were elevated in four. There was no increased incidence of HLA-B27 or other HLA-B7 cross-reacting antigens. A temporal relation of skin and joint disease activity was suggested. We report a spondyloarthropathy associated with hidradenitis suppurativa and acne conglobata. Clinical and laboratory manifestations suggest the arthropathy may be reactive to chronic cutaneous infection.
The pathophysiology and treatment of hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp, which constitute the follicular occlusion triad, are reviewed. The unusual occurrence of all three components in a single patient resistant to medical management is presented with a review of the literature. This patient's course was complicated by multiple synchronous squamous cell carcinomas developing in a localized area where his acne conglobata was most pronounced. Although one lesion was clinically obvious, the majority were less suspicious for cancer. Occult cancer should be considered in recalcitrant cases of acne conglobata where isolated areas fail to respond to medical management, particularly when 13-cis-retinoic acid has been used.
We report a 16-year-old male in whom pyoderma gangrenosum appeared in conjunction with acne conglobata. The patient also developed a seronegative spondyloarthropathy that was the main presenting complaint. There was no evidence of inflammatory bowel disease. Treatment with isotretinoin was successful. Both acne and pyoderma lesions healed and the articular symptoms improved. The present case, together with other reports in the literature show that acne conglobata must be included in the list of possible associations of pyoderma gangrenosum. We also comment on acne arthritis, a relatively frequent phenomenon, although still not generally known, in acne conglobata.
Leukocytes derived from the peripheral blood of 10 patients with acne conglobata demonstrated enhanced chemotactic and random migratory activities in comparison with leukocytes from 10 healthy subjects. This enhancement is most probably secondary to the state of chronic infection and inflammation. These findings probably rule out a defect in leukocyte motility as a cause of the severity of the lesions in acne conglobata.
16 patients suffering from acne conglobata were prospectively examined by means of analytical interviews and 5 psychometric procedures before and 6 months after oral treatment with 13-cis retinoic acid (isotretinoin). In comparison with a control group of psychosomatic patients, acne conglobata patients are more frequently affected by childhood influences leading to a neurotic personality structure already before the outbreak of acne; the patients more often complain of disturbed social contact, depressive moods, or general disorders. After successful treatment with isotretinoin, we observed augmented self-confidence and positive aggressiveness, on one hand, and increase of anxiety depressive moods, and general complaints, on the other. These effects are not drug related in a pharmacological way. These observations suggest the influence of psychic factors in the pathogenesis of acne conglobata. Regarding the medical management of these patients, it should be considered that psychic and psychosomatic disorders might be intensified after successful drug therapy.
Twenty patients (21.4 +/- 4.3 years) with extensive acne conglobata affecting the face, chest and back, were treated for a period of six months with isotretinoin (13-cis retinoic acid, 13-cis RA) at a dosage of 1 mg/kg body weight/day (mean daily dose 72.7 +/- 9.8 mg 13-cis RA). In all cases, the acne conglobata cleared up completely. With the exception of symptoms produced by mucocutaneous exsiccation, no side effects were observed; the laboratory parameters were all within normal limits during the anti-acne treatment phase. There was no recurrence of the disease within a period of one year after cessation of treatment (n = 13). Screening spermatograms performed at two-month intervals during the treatment phase revealed, from the fourth month of treatment onward, a statistically significant increase in the mean sperm density with sperm morphology and motility unchanged as compared with the situation prior to treatment. One year after conclusion of treatment, all measured spermatogram parameters had returned to the initial values. The investigations revealed no evidence of any negative influence of the 6-month treatment with isotretinoin on spermatogenesis.
The authors report a case of painful joint, para-articular and muscular involvement accompanying acne conglobata. Fifteen other cases of rheumatism associated with this very special type of acne have been reported. The patients affected are young men suffering from acne conglobata (severe form of acne characterised by its ulcerating course and the possibility of its being accompanied by systemic manifestations), who suddenly develop fever, an altered general state and assymetrical arthralgia involving mainly the large joints, accompanied by signs of muscular involvement. The course is one of spontaneous recovery, relapses being possible. This falls within the context of the group of rheumatic disorders associated with the signs of an inflammatory skin disease.