eMedicine Specialties > Dermatology > Papulosquamous Diseases
Psoriasis, Pustular: Treatment & Medication
Updated: Dec 29, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Patients with the generalized form of pustular psoriasis eruption often are admitted to the hospital to ensure adequate hydration, bed rest, and avoidance of excessive heat loss. Treatment with bland topical compresses and saline or oatmeal baths assists in soothing and debriding affected areas. This topical strategy is effective in many pediatric patients as the sole therapy.
- Start systemic medications together with the proper supportive measures. Oral retinoids, methotrexate, cyclosporine, 6-thioguanine, and hydroxyurea have been used with success.8,9,10 One case report describes successful therapy with cyclosporine for pustular psoriasis during pregnancy.11
- Novel systemic therapies such as biologics (eg, alefacept, etanercept, infliximab) have been used successfully in some cases of pustular psoriasis. Guidelines regarding their use in this type of psoriasis are needed because some anecdotal reports describe paradoxical induction of pustular psoriasis by some of these biologics.12 One case report describes successful infliximab treatment (5 mg/kg) of pustular psoriasis in a pregnant woman. The woman delivered a healthy female baby via cesarean. The neonate breastfed for 1 month and developed normally. No detectable adverse effects have been noted in the neonate, despite potential exposure to infliximab throughout gestation and breastfeeding.13
- Other therapies, such as topical calcineurin inhibitors (eg, tacrolimus, pimecrolimus) have also been shown effective in some cases of pustular psoriasis localized to the palms and soles. A 2009 case report describes effective treatment of palmoplantar pustular psoriasis using adalimumab.14
- Phototherapy15
- Oral psoralen plus UV-A (PUVA): Patients usually are too toxic and too erythrodermic during a flare to tolerate PUVA; however, some studies have shown that PUVA may be a safe and effective treatment in controlling flares of pustular psoriasis in pediatric patients as well as adults. Typically, PUVA is started once the patient has been stabilized on acitretin.
- UV-B and narrow-band UV-B: While the literature is scant regarding the use of phototherapy for pustular psoriasis, narrow-band UV-B may be a reasonable choice, since it has achieved therapeutic results similar to those of PUVA in other forms of psoriasis.
- Retinoid plus PUVA: Acitretin is administered first at 0.2-0.5 mg/kg for 7 days, then PUVA is added 3 times per week. Upon clearance, acitretin can be withdrawn, and maintenance phototherapy with PUVA or, preferably, narrowband UVB, can be continued as needed.
Consultations
Request consultations with medical subspecialists according to the degree of systemic involvement.
Medication
The goals of pharmacotherapy for pustular psoriasis are to reduce morbidity and prevent complications.
Retinoids
Decrease cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. Modulate keratinocyte differentiation. Have been shown to reduce risk of skin cancer formation in renal transplant patients.
Acitretin (Soriatane)
Retinoic acid analog, similar to etretinate and isotretinoin. Etretinate is primary metabolite and acitretin has demonstrated clinical effects similar to those seen with etretinate. Mechanism of action is unknown. More effective when used in conjunction with PUVA.
Adult
0.2-0.5 mg/kg PO qd over several wk; alternatively, 25 or 50 mg/d PO initially given as single dose with main meal; 25-50 mg/d PO after initial response to treatment; terminate therapy when lesions have resolved sufficiently
Pediatric
Not established
Coadministration with methotrexate increases risk of hepatotoxicity; concomitant use with tetracycline or minocycline increases risk of pseudotumor cerebri; ethanol induces formation of etretinate, which has much longer half-life; acitretin interferes with contraceptive effect of microdosed progestin "minipill" preparations; concomitant use of vitamin A increases risk of additive toxicity
Documented hypersensitivity; pregnancy, concomitant vitamin A, significant liver disease
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; recommended that contraception be continued for at least 3 y after treatment with acitretin ends; etretinate may form from acitretin, which takes approximately 2-3 y to clear; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated
Isotretinoin (Accutane)
Oral agent that treats serious dermatologic conditions. Synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to vitamin A.
Has been used to treat pustular psoriasis.
A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.
Adult
1 mg/kg PO qd or bid
Pediatric
Not established
Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; acitretin may reduce plasma levels of carbamazepine
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
May decrease night vision; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; patients with diabetes may experience problems in controlling blood sugar; avoid excessive exposure to UV light or sunlight
Antimetabolites
Regulate cell growth and differentiation.
Methotrexate (Folex, Rheumatrex)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction. Satisfactory response seen in 3-6 wk following administration.
Adjust dose gradually to attain satisfactory response.
Fever, toxicity, and pustulation may decrease within 24-48 h, but erythroderma usually persists; may take several weeks to work well.
Adult
0.2-0.4 mg/kg PO/IM/wk divided into 3 parts q12h over 36 h qwk or as single weekly dose; alternatively, 10-25 mg/wk PO/IM or 2.5-7.5 mg PO q12h for 3 doses/wk
Pediatric
Not established
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX; coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase MTX plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Overdose may result in widespread skin erosions, ulceration, and toxic epidermal necrolysislike manifestations, as well as bone marrow suppression; with long-term use, hepatic fibrosis can occur, therefore, periodic liver biopsies are recommended; monitor CBC counts qmo and liver and renal function q1-3 mo during therapy (monitor more frequently during initial dosing, dose adjustments, or if risk of elevated levels [eg, dehydration]); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs
Cyclosporine (Sandimmune, Neoral)
Demonstrated to be helpful in a variety of skin disorders, especially psoriasis. Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease for a variety of organs.
In children and adults, base dosing on ideal body weight.
Adult
2.5-5 mg/kg/d PO divided bid
Pediatric
1-3 mg/kg/d PO bid; not to exceed 5-7 mg/kg/d
Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease cyclosporine concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase cyclosporine toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin
Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UV-B radiation in psoriasis since it may increase risk of cancer
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO; increased carcinogenicity in patients on methotrexate, PUVA, coal tar, or anthralin therapy; renal dysfunction; hypertension
More on Psoriasis, Pustular |
| Overview: Psoriasis, Pustular |
| Differential Diagnoses & Workup: Psoriasis, Pustular |
 Treatment & Medication: Psoriasis, Pustular |
| Follow-up: Psoriasis, Pustular |
| Multimedia: Psoriasis, Pustular |
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References
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Further Reading
Keywords
psoriasis, pustular psoriasis, von Zumbusch psoriasis
