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Pruritic Urticarial Papules and Plaques of Pregnancy

Overview

This common and very pruritic disorder of pregnancy of unknown cause usually begins in the third trimester and resolves with delivery. Unlike herpes gestationis, postpartum onset or exacerbation is rare. Therapy is empiric and aims to control symptoms until the eruption abates following delivery.

First steps

1. A topical high-potency to superpotent steroid cream applied two to four times daily. The strength should be guided by the severity of the pruritus.
2. Oral diphenhydramine 25-50 mg 3 times daily.

Subsequent steps

Only rarely are systemic steroids required in this condition. Prescribe the minimum effective dose (initially usually 0.5-1 mg/kg in a single dose each morning) and rapidly taper to the lowest controlling dose.

Pitfalls

1. Herpes gestationis initially may resemble the pruritic urticarial papules and plaques of pregnancy. A biopsy for direct immunofluorescence may be indicated.
2. Scabies and other insect bites are morphologically similar to the pruritic papular rashes of pregnancy. Look carefully for burrows and take a history of animal exposure.

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Necrobiosis Lipoidica Diabeticorum

Overview

Lesions of necrobiosis lipoidica diabeticorum (NLD) are usually, but not always, associated with juvenile-onset diabetes. Tending to occur singly or in crops on the anterior lower legs, they can appear anywhere on the body, however. Lesions on the leg exhibit more of a tendency to ulcerate than lesions elsewhere.

First Steps

1. For nonulcerated, symptomatic lesions (asymptomatic lesions need not be treated), intralesional triamcinolone acetonide 3-5 mg/ml. Small amounts of this solution are injected intradermally into plaques via a 30-guage needle. Injections are repeated every 2-3 weeks.
2. Application of superpotent topical steroids with occlusion for several weeks can be effective.

Ancillary Steps

1. Administration of pentoxiphylline 400-800 mg three times plus ASA 81 mg daily.
2. For persistent erythema and telangiectasia, not resolving with the above therapy, pulse dye laser can be utilized for cosmetic improvement.

Subsequent Steps

1. Topical tacrolimus ointment 0.1% may be used for refractory lesions.
2. Topical or systemic PUVA may improve NLD, and can even be used in cases with ulceration, with reduction in pain after several treatments.
3. In severe refractory cases requiring systemic treatment, consider the following options:
   a. Cyclosporine 2.5-5 mg/kg per day for several months
   b. Mycophenolate mofetil 2-3 g/day
   c. Infliximab 5 mg/kg per treatment for a total of 3 treatments at 0, 2, and 6 weeks.
4. NLD has a tendency to ulcerate. These ulcerations may be infected by bacteria, so nonhealing lesions should be cultured, and appropriate topical and oral antibiotics should be considered if the methods outlined below are ineffective. At times the ulcerated lesions will be very painful. The techniques used to heal chronic ulcers can be applied to the ulcerated lesions of NLD, including these treatments:
   a. Application of semipermeable dressings
   b. Becaplermin applied once daily to the ulceration for 12 hours, or once weekly under occlusive, semipermeable dressing, as for venous insufficiency ulcers.
   c. Topical or intralesional granulocyte-macrophage colony-stimulating factor
   d. Dermagraft or other skin substitute equivalent applied at regular intervals. Multiple applications may be required. This is frequently very effective in reducing/eliminating the pain as well as stimulating ulcer healing.

Pitfalls

1. Lesions of NLD can be very resistant to the above management, and may not respond. Moreover, new lesions may continue to form.
2. There is no relationship between severity of NLD and diabetic status.

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Porphyria Cutanea Tarda

Overview

Porphyria cutanea tarda (PCT) is the most common porphyria. It is inherited as an autosomal dominant disorder in 20% of cases and is acquired in association with other disorders in the remaining 80% of cases. PCT is related to a deficiency of the enzyme uroporphyrinogen decarboxylase.

First steps

1. Stop exacerbating drugs (estrogens, OCPs, iron).
2. Counsel the patient to stop all alcohol ingestion.
3. Inform the patient that sunlight is a cofactor, and have the patient protect the skin from the sun until therapy has lowered porphyrin levels. Sunscreens are inadequate. Opaque physical blocks are required.
4. Obtain a baseline 24-hour urine quantitative porphyrin level to guide therapy.
5. Evaluate for hepatocellular disease with a physical examination and LFTs, a hepatitis C serology, and an assay for the hemochromatosis gene. Hepatitis C infection and hepatoma may precipitate porphyria cutanea tarda.
6. In the nonanemic patient, repeated venesection of 400-500 ml of blood every 2-4 weeks may be performed. Hospitalized patients may have more frequent phlebotomies.

Alternative steps

1. Low-dose chlorquine 125 mg 2 times/week is quite effective. Check the LFTs, then give a test dose of chlorquine 125 mg, and recheck the LFTs before instituting therapy. Hydroxychlorquine 250 mg twice weekly may also be used.
2. PCT patients on dialysis may be treated with erythropoietin.

Subsequent steps

1. Phlebotomy is continued until clinical improvement occurs, keeping the hemoglobin in the 10- to 11-g range. Urine porphyrins fall with phlebotomy and may continue to fall for months after phlebotomy is discontinued.
2. Antimalarial therapy is continued until urinary porphyrins are less than 300 mg/24 hours. Monitor LFTs monthly.
3. Antimalarial therapy may be combined with repeated large or small phlebotomies in patients not responding to a single modality treatment alone.

Pitfalls

1. Do not give iron to correct the anemia induced by phlebotomy.
2. Higher doses of antimalarials may induce a severe hepatotoxic crisis, with fever, chills, vomiting, and elevated LFTs.
3. A clinically and histologically identical syndrome with normal porphyrins (pseudoporphyria) may be induced by certain phototoxic agents, notably tetracycline, NSAIDs, and estrogen. Urinary porphyrins are required to establish the correct diagnosis. Patients on dialysis may develop true porphyria or pseudoporphyria. Plasma and stool porphyrin levels are needed for diagnosis in this setting.
4. Nonopaque sunscreens are not protective, as the active spectrum is at 400-410 nm, the borderline of long UV and visible violet radiation (Soret band).

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U mềm lây

Molluscum Contagiosum

Overview

The therapy of molluscum contagiosum is divided into three treatment groups: children, adults, and the immunosuppressed. Molluscum contagiosum limited to the genital area in children should raise the possibility of child abuse.

Children

Children may have a few lesions or many lesions (50 to hundreds).

First Steps

Over 50 lesions

1. For children with many lesions, no therapy is an acceptable approach. These lesions eventually will disappear spontaneously without sequelae. Parents are reluctant to accept this option, but heroic measures are unnecessary and potentially scarring both physically and emotionally.
2. In the cooperative child, cantharidin applied for 2-6 hours may cause enough irritation to cause lesions to involute.

Under 50 lesions

If the number of lesions is manageable and the child is at least in part cooperative, multiple topical therapies may work.

1. Administer cryotherapy with liquid nitrogen to the individual lesions.
2. Apply 1 tiny drop of cantharidin to the tip of each lesion and wash off in 2-6 hours. Do not occlude. Before allowing the patient to move around, be sure the medication is dry to the touch so it does not spread to normal skin. Lesions will crust and fall off in less than 1 week. This medication must be applied by the doctor in the office. It cannot be used in occluded areas (axillae, groin, inner thighs) or around the eyes.
3. Pricking the surface of a lesion with a #11 blade will often lead to inflammation and resolution of that lesion. Pressing out the central core of the lesion with a comedone extractor will guarantee resolution.
4. Cooperative children occasionally may be able to tolerate the pain of curetting individual lesions.

Adults

Molluscum contagiosum in normal adults is usually an STD and is found in the genital area. Adults with extensive lesions outside the genital area must be evaluated for immunosuppression, especially HIV infection.

First Steps

1. Cryotherapy with liquid nitrogen is quick and effective.
2. Evaluate for other STDs.
3. Examine and treat the patient's sexual partner(s).
4. Advise the patient that the lesions are sexually transmissible.

Alternative Steps

1. Destruction of each lesion by pricking with a large (18-guage) needle or a #11 blade. Removal of the core with a comedone extractor will enhance resolution.
2. Adults will usually tolerate curettage of individual lesions.
3. Electrocautery may be used to destroy smaller lesions. Anesthesia with topical EMLA may allow this form of treatment without injected anesthesia.

Subsequent Steps

One treatment is usually inadequate to eradicate all lesions. See the patient at biweekly intervals until no lesions are present, then 4-6 weeks after the last visit for a final check.

Pitfalls

1. The most common error is diagnosing molluscum as genital warts. If there is any question, refer the patient.
2. Avoid cantharidin in the genital area.

Immunosuppressed

In severe immunosuppression, especially in advanced AIDS, extensive facial or genital molluscum are very common. Total cure is almost impossible. (Individual lesions in general do not spontaneously resolve as in healthy adults and children.) Lesions are treated for cosmesis at the patient's request.

First Steps

Molluscum contagiosum in the setting of HIV infection is a direct consequence of significant immunosuppression. The treatment of choice is institution of Highly Active Antiretroviral Therapy (HAART). Once the helper T cell count exceeds 100, the molluscum begin to resolve, leaving no scars. This takes several months. Aggressive therapy that potentially would scar should not be undertaken until the full beneficial effects of immune reconstitution have been realized.

1. Liquid nitrogen cryotherapy (preferably by spray rather than a swab) is effective and well tolerated for smaller lesions.
2. For facial or truncal molluscum, cantharidin applied for 2-6 hours and then washed off will also resolve lesions. This has the advantage of usually being painless.
3. Instruct the patient to use only an electric razor (preferably with floating heads) to shave. This type of shaving is less likely to spread the lesions.

Alternative Steps

1. Pricking and curetting lesions, although also effective, have disadvantages. First, this approach is potentially hazardous to the care provider because he or she may be exposed to blood. Second, this may provide a portal of entry for infection. If these methods are used, appropriate precautions are necessary.
2. 5-Fluorouracil 5% cream may cause inflammation of lesions and their gradual resolution. Treatment should be used at the frequency tolerated by the patient in order to maintain mild inflammation of the lesions. This is usually once or twice weekly in fair-skinned persons, but up to once daily in persons of color. Several months of treatment may be required.
3. In severe cases, trichloroacetic acid peels, up to 50% concentration, may be considered.

Pitfalls

In the setting of immunosuppression, other infectious agents (e.g., herpes simplex, cryptococcus neoformans) may produce lesions mimicking molluscum contagiosum. If there is any question as to the correct diagnosis, a biopsy is in

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