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Excerpt from Acne ConglobataSynonyms, Key Words, and Related Terms: AC, pyoderma gangrenosum, aseptic arthritis, PAPA syndrome, acne fulminans, AF Please click here to view the full topic text: Acne ConglobataBackgroundAcne conglobata (AC) is an uncommon and unusually severe form of acne characterized by burrowing and interconnecting abscesses and irregular scars (both keloidal and atrophic), often producing pronounced disfigurement. The comedones often occur in a group of 2 or 3, and cysts contain foul-smelling seropurulent material that returns after drainage. The nodules are usually found on the chest, the shoulders, the back, the buttocks, the upper arms, the thighs, and the face.1 AC may develop as a result of a sudden deterioration of existing active papular or pustular acne, or it may occur as the recrudescence of acne that has been quiescent for many years. Pyoderma gangrenosum, AC, and aseptic arthritis are clinically distinct inflammatory disorders. Although this triad of symptoms rarely occurs in an individual patient, it was reported in a 3-generation kindred with autosomal dominant transmission of the 3 disorders; this condition is called familial pyoderma gangrenosum, AC, and aseptic arthritis (PAPA) syndrome. PathophysiologyThe primary causes of AC remain unknown. Chromosomal defects in the XXY karyotype may be responsible for severe forms of AC. In contrast, the XXY karyotype of Klinefelter syndrome is believed to exclude severe acne; however, 1 patient with the unusual combination of Klinefelter syndrome and AC has been reported.2 The association of this disease with specific human leukocyte antigen (HLA) phenotypes has not been proven. The HLA-A and HLA-B phenotypes were evaluated in 65 patients with AC, in whom antigen frequencies were found to be normal. Other patients with AC and hidradenitis suppurativa were studied; 4 of 6 patients had HLA-B7 cross-reacting antigens (ie, HLA-B7, HLA-Bw22, HLA-B27, HLA-Bw40, HLA-Bw42), and all had HLA-DRw4.3 PAPA syndrome has been mapped to a locus on the long arm of chromosome 15 (maximum 2-point logarithm of odds score 5.83; recombination fraction [straight theta] 0 at locus D15S206).4 Assuming complete penetrance, haplotype analysis of recombination events defined an interval of 10 centimorgans between loci D15S1023 and D15S979. This finding suggests that these clinically distinct disorders may share a genetic etiology. FrequencyInternationalAC is an uncommon disease. Mortality/MorbidityAC can produce pronounced disfigurement. Severe scarring produces psychological impairment; individuals with AC are often ostracized, or they may feel excluded. AC has also been responsible for anxiety and depression in many patients. SexThe disease affects males more frequently than females. AgeThe onset of AC usually occurs in young adults aged 18-30 years, but infants may develop this condition as well. Please click here to view the full topic text: Acne Conglobata |
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