Background: Acrokeratosis neoplastica (AN) is a rare psoriasiform dermatosis that is associated consistently with internal malignancy, usually squamous cell carcinoma (SCC) of the upper aerodigestive tract. Skin manifestations include acute onset of onychodystrophy and violaceous scaly patches on the helices, nose, and malar and acral surfaces. Usually, skin manifestations precede diagnosis of the malignancy.
The term Bazex syndrome describes 2 different entities: AN and the genetic syndrome of basal cell carcinomas, follicular atrophoderma, hypotrichosis, and hypohidrosis or hyperhidrosis.
Pathophysiology: The skin manifestations of AN parallel the disease course, suggesting the presence of circulating antibodies to tumor antigens. Cytokines may play a role.
Frequency:
- In the US: Approximately 125 cases have been reported in the literature.
Mortality/Morbidity:
- Mortality and morbidity are related directly to the available treatment options for the underlying neoplasm.
- In most reported cases, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis.
Sex: In one review, 105 of 113 cases were in males.
Age: Mean age of onset is 61 years.
Treatment
Medical Care:
Treatment
Medical Care:
- Treatment depends on the type and stage of the underlying neoplasm. Often, treating the neoplasm improves the cutaneous disease. If the neoplasm is cured, skin signs abate.
- While specific treatment of the skin improves the eruption, this usually is not curative.
- Topical corticosteroids may improve scaly lesions.
- Therapy with cholecalciferol also has been reported to be of benefit.
Consultations:
- Consultation with an internal medicine specialist for malignancy workup is appropriate.
- Consultation with a hematologist, oncologist, otolaryngologist and/or gastroenterologist also can assist with focused evaluations.
Medication
The goal of pharmacotherapy is to improve scaly lesions, reduce morbidity, and prevent complications.
Drug Category: Corticosteroids -- These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
The goal of pharmacotherapy is to improve scaly lesions, reduce morbidity, and prevent complications.
Drug Category: Corticosteroids -- These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
| Drug Name | Betamethasone (Diprolene, Betatrex) -- For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. |
|---|---|
| Adult Dose | Apply thin film bid/qid until response |
| Pediatric Dose | Administer as in adults with caution |
| Contraindications | Documented hypersensitivity; paronychia; cellulitis; impetigo; angular cheilitis; erythrasma; erysipelas; rosacea; perioral dermatitis; and acne |
| Interactions | None reported |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Do not use in skin with decreased circulation; can cause atrophy of groin, face, and axillae; if infection develops and is not responsive to antibiotic treatment, discontinue until infection is under control; do not use monotherapy to treat widespread plaque psoriasis |
Drug Category: Dietary Supplements -- For treatment of vitamin D deficiency or prophylaxis of vitamin D deficiency. Vitamin D and its analogs have profound effects on cellular proliferation and differentiation in addition to immunomodulatory effects. Calcipotriene, a vitamin D analog, is a well-accepted treatment for psoriasis, which is a condition that shares some similarities with AN. However, the reports of efficacy in the treatment of AN with vitamin D are anecdotal.
| Drug Name | Cholecalciferol (Delta-D, Vitamin D-3) -- Stimulates absorption of calcium and phosphate from small intestine and promotes release of calcium from bone into blood. Antiproliferative and anti-inflammatory effects on skin. |
|---|---|
| Adult Dose | Delta-D: 400 IU PO qd Vitamin D-3: 1000 IU PO qd Calcitriol: 0.25-1 mcg PO qd |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; hypercalcemia; malabsorption syndrome |
| Interactions | Colestipol, mineral oil, and cholestyramine may decrease absorption of ergocalciferol from small intestine; thiazide diuretics may increase effects of vitamin D; may interfere with calcium channel blockers |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Caution in impaired renal function, renal stones, heart disease, or arteriosclerosis |
RSS Feed (xml)
