Acne 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. 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.
Pathophysiology
The 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.
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.
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).1 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.
Frequency
International
AC is an uncommon disease.
Mortality/Morbidity
AC 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.
Sex
The disease affects males more frequently than females.
Age
The onset of AC usually occurs in young adults aged 18-30 years, but infants may develop this condition as well.
Treatment
Medical Care
The therapy of choice for AC is isotretinoin 0.5-1 mg/kg for 4-6 months.
- Simultaneous use of systemic steroids, such as prednisone 1 mg/kg/d for 2-4 weeks, may also prove beneficial, particularly if systemic symptoms are evident.
- Alternatives include oral tetracycline 2 g/d or erythromycin 2 g/d, either alone or with isotretinoin or prednisone.
- For treatment-resistant cases, dapsone 50-150 mg/d is recommended; this treatment should be carefully monitored.
- Along with vigorous medical therapy, emotional support is essential.
- Treatment of AC with infliximab has been tried; the authors do not recommend this therapy.
Surgical Care
- Large hemorrhagic nodules may be aspirated.
- Intralesional triamcinolone or cryotherapy may also be valuable.
- Occasionally, surgical excision of interconnecting large nodules may be beneficial.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Prednisolone (Delta-Cortef, Econopred, Articulose-50) |
|---|---|
| Description | Synthetic adrenocortical steroid with predominantly glucocorticoid properties. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production. |
| Adult Dose | 0.5-1 mg/kg/d PO for 6 wk; taper as condition improves Single morning dose is safer for long-term use, but divided doses have more anti-inflammatory effect |
| Pediatric Dose | Initial: 0.14-2 mg/kg/d PO divided tid/qid (4-60 mg/m2/d) |
| Contraindications | Documented hypersensitivity; viral, fungal, connective tissue, or tubercular infection; peptic ulcer disease; hepatic dysfunction; GI tract disease |
| Interactions | Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur |
Drug Category: Retinoids
Vitamin A derivatives have many roles. They encourage cellular differentiation, they are antiproliferative, and they serve as immunomodulators.
| Drug Name | Isotretinoin (Accutane) |
|---|---|
| Description | Oral agent that treats serious dermatologic conditions. Isotretinoin is the synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to beta-carotene. Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization. Effective March 1, 2006, FDA requires that prescribers of isotretinoin, patients who take isotretinoin, and pharmacists who dispense isotretinoin all must register with the iPLEDGE system. |
| Adult Dose | Initial: 0.5 mg/kg/d PO, increase gradually (usually 1 mg/kg/d) for 20 wk |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | Toxicity may occur with beta carotene coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine |
| Pregnancy | X - Contraindicated in pregnancy |
| Precautions | May decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; exaggerated healing response of acne lesions (ie, excessive granulation with crusting) may occur; patients with diabetes may experience problems in controlling blood glucose levels while on isotretinoin; avoid exposure to UV light or sunlight until tolerance is achieved; discontinue if rectal bleeding, abdominal pain, or severe diarrhea occurs; mood swings or depression may occur; caution in history of depression |
| Drug Name | Tretinoin (Avita, Retin-A, Retin-A Micro) |
|---|---|
| Description | Structurally related to vitamin A. May be helpful for recalcitrant disease, but recurrence is common. Long-term, low-dose therapy may be suitable for selected patients. May cause skin irritation in some patients. Also, has been linked to promotion of angiogenesis; however, has not demonstrated increased telangiectasias. Also inhibits microcomedo formation and eliminates lesions. Makes keratinocytes in sebaceous follicles less adherent and easier to remove. Available as 0.025%, 0.05%, and 0.1% creams. Available also as 0.01% and 0.025% gels. |
| Adult Dose | Begin with lowest concentration of tretinoin formulation and increase as tolerated; apply hs or qod; lower frequency of application if irritation develops |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Toxicity may occur with vitamin A coadministration; toxicity increased when coadministered with sulfur, benzoyl peroxide, resorcinol, or any product with strong drying effects; phototoxicity increased when coadministered with tetracyclines, fluoroquinolones, or thiazides |
| Pregnancy | C - Safety for use during pregnancy has not been established |
| Precautions | Photosensitivity may occur with excessive sunlight exposure; burning, stinging, peeling, pruritus, or erythema has been reported at site of application; caution with eczema (may cause severe irritation); avoid contact with mucous membranes, mouth, and angles of nose |
Drug Category: Antibiotics
Therapy must be comprehensive and cover all likely pathogens in the context of the clinical setting. Antibiotic selection should be guided by blood culture sensitivity whenever feasible.
| Drug Name | Roxithromycin (Rulid, Oxoid) |
|---|---|
| Description | Not available in the United States. Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, thereby arresting RNA-dependent protein synthesis. |
| Adult Dose | 150-300 mg PO bid for 4-6 wk, continue if response is favorable |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; coadministration with pimozide |
| Interactions | Toxicity increases with coadministration of fluconazole and pimozide; plasma levels of certain benzodiazepines may increase, prolonging CNS depression; arrhythmias and increase in QTc intervals occur with disopyramide; coadministration with omeprazole may increase plasma levels of both agents |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Caution in liver disease; GI tract adverse effects are common (administer doses pc); discontinue if nausea, vomiting, malaise, abdominal colic, or fever occur |
| Drug Name | Clarithromycin (Biaxin) |
|---|---|
| Description | 6-methoxy erythromycin. Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, thereby arresting RNA-dependent protein synthesis. |
| Adult Dose | 250-500 mg PO bid for 4-6 wk, continue if response is favorable |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; coadministration with pimozide |
| Interactions | Toxicity increases with coadministration of fluconazole and pimozide; effects decrease and GI tract adverse effects may increase with coadministration of rifabutin or rifampin; may increase toxicity of anticoagulants, cyclosporine, tacrolimus, digoxin, omeprazole, carbamazepine, ergot alkaloids, triazolam, and HMG-CoA reductase inhibitors; plasma levels of certain benzodiazepines may increase, prolonging CNS depression; arrhythmias and increase in QTc intervals occur with disopyramide; coadministration with omeprazole may increase plasma levels of both agents |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Coadministration with ranitidine or bismuth citrate is not recommended with CrCl <25> |
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