You are in: eMedicine Specialties > Dermatology > DISEASES OF THE DERMIS PachydermoperiostosisArticle Last Updated: Apr 14, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: Supriya Goyal, MD, Consulting Dermatologist Supriya Goyal is a member of the following medical societies: Alpha Omega Alpha Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Gregory M Richards, MD, Staff Physician, Department of Human Oncology, University of Wisconsin School of Medicine and Public Health; Instructor of Radiotherapy Technology (RT 412), University of Wisconsin; Rajiv Goyal, MD, Consulting Staff, Department of Radiology, Kaiser Permanente Medical Center Editors: Michelle Pelle, MD, Clinical Assistant Professor, Division of Dermatology, Department of Medicine, University of California at San Diego; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System Author and Editor Disclosure Synonyms and related keywords: primary hypertrophic osteoarthropathy, idiopathic hypertrophic osteoarthropathy, hereditary hypertrophic osteoarthropathy, Touraine-Solente-Gole syndrome, osteoarthropathy, PDP, pachydermia INTRODUCTIONSection 2 of 9
  BackgroundHypertrophic osteoarthropathy is divided into primary and secondary forms. Pachydermoperiostosis (PDP), the primary form, accounts for 5% of all cases of hypertrophic osteoarthropathy. Secondary hypertrophic osteoarthropathy, also called pulmonary hypertrophic osteoarthropathy, is associated with underlying cardiopulmonary diseases and malignancies. This latter condition is not discussed here but may be found in the article Dermatologic Manifestations of Pulmonary Disease. PDP is a rare hereditary disorder that was first described in 1868. It is characterized by digital clubbing, pachydermia (thickening of the facial skin and/or scalp), and periostosis (swelling of periarticular tissue and subperiosteal new bone formation). PDP is associated with pain, polyarthritis, cutis verticis gyrata, seborrhea, eyelid ptosis, and hyperhidrosis. Touraine et al described 3 forms of PDP: (1) a complete form with pachydermia and periostitis, (2) an incomplete form with evidence of bone abnormalities but lacking pachydermia, and (3) a forme fruste with prominent pachydermia and minimal-to-absent skeletal changes. FrequencyUnited StatesPDP is a rare disorder, and the precise incidence is unknown. Mortality/Morbidity
RacePDP is more common in African Americans than in whites. SexThe male-to-female case ratio is approximately 7:1.
AgePDP typically begins during childhood or adolescence and progresses gradually over the next 5-20 years before stabilizing. CLINICALSection 3 of 9
HistoryPatients may complain of the following signs or symptoms:
PhysicalSkin, hair, and nail examination may reveal the following:
CausesPDP is often familial. It is believed to be inherited in an autosomal dominant pattern with variable penetrance; however, autosomal recessive forms have been reported. PDP has been associated in case reports with a variety of other disorders, including the following:
DIFFERENTIALSSection 4 of 9
Acromegaly
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| Drug Name | Ibuprofen (Ibuprin, Motrin, Advil, Excedrin IB) |
|---|---|
| Description | DOC for patients with mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis. |
| Adult Dose | 400 mg PO q4-6h, 600 mg PO q6h, or 800 mg PO q8h while symptoms persist; not to exceed 3.2 g/d |
| Pediatric Dose | 20-70 mg/kg/d PO divided tid/qid; start at low end of dosing range and titrate; not to exceed 2.4 g/d |
| Contraindications | Documented hypersensitivity; peptic ulcer disease; recent GI tract bleeding or perforation; renal insufficiency; high risk of bleeding |
| Interactions | Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy; may increase postoperative bleeding |
| Drug Name | Indomethacin (Indocin, Indochron E-R) |
|---|---|
| Description | Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis. |
| Adult Dose | 25-50 mg PO bid/tid 75 mg SR PO bid; not to exceed 200 mg/d |
| Pediatric Dose | 1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d |
| Contraindications | Documented hypersensitivity; GI tract bleeding; renal insufficiency |
| Interactions | Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists) |
| Drug Name | Meloxicam (Mobic) |
|---|---|
| Description | Decreases activity of cyclo-oxygenase, which, in turn, inhibits prostaglandin synthesis. These effects decrease formation of inflammatory mediators. |
| Adult Dose | 7.5 mg/d PO; may increase to 15 mg/d PO |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; active GI tract bleeding |
| Interactions | Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur (discontinue if leukopenia, granulocytopenia, or thrombocytopenia persists) |
| Drug Name | Celecoxib (Celebrex) |
|---|---|
| Description | Primarily inhibits COX-2. COX-2 is an isoenzyme induced during pain and inflammatory stimuli. Inhibition of COX-1 may contribute to NSAID GI tract toxicity. At therapeutic concentrations, the COX-1 isoenzyme is not inhibited, thus GI tract toxicity may be decreased. Seek lowest dose of celecoxib for each patient. |
| Adult Dose | 200 mg/d PO; alternatively, 100 mg PO bid |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Coadministration with fluconazole may increase plasma concentrations because of inhibition of celecoxib metabolism; coadministration with rifampin may decrease plasma concentrations |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Category D in third trimester of pregnancy; may cause fluid retention and peripheral edema; caution in compromised cardiac function, hypertension, and conditions predisposing patients to fluid retention; caution in severe heart failure and hyponatremia because may deteriorate circulatory hemodynamics; NSAIDs may mask usual signs of infection; caution in presence of existing controlled infections; evaluate therapy when symptoms or laboratory results suggest liver dysfunction |
| Drug Name | Naproxen (Aleve, Naprosyn, Naprelan, Anaprox) |
|---|---|
| Description | For relief of mild to moderate pain; inhibits inflammatory reactions and pain by decreasing the activity of cyclo-oxygenase and prostaglandin synthesis. |
| Adult Dose | 250-500 mg PO bid; may increase to 1.5 g/d for limited periods |
| Pediatric Dose | <2 years: Not established >2 years: 5-7 mg/kg/dose PO bid/tid; not to exceed 20 mg/kg/d or 1000 mg/d |
| Contraindications | Documented hypersensitivity; peptic ulcer disease; recent GI tract bleeding or perforation; renal insufficiency |
| Interactions | Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Category D in third trimester of pregnancy; acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia rarely occurs, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug |
Article Last Updated: Apr 14, 2006
