Plaques with hyperpigmented borders - Clinical Advisor

Plaques with hyperpigmented borders

Slideshow

  • Case #1

    2015 January Dermatology Look-Alikes

    Case #1

  • Case #2

    2015 January Dermatology Look-Alikes

    Case #2

Case #1

A 35-year-old black woman presents as an inpatient consult for lesions on the chest and thigh. The patient said the lesions developed several years ago, after she fell on a gravel surface outdoors. The lesions were asymptomatic. She reported no fever, chills, and weight loss. Her medical history is significant for a suspected connective tissue disease for which she had been maintained on chronic low-dose oral prednisone. The patient’s family and social histories were noncontributory. Her physical examination is notable for pink lichenified, crusty plaques with hyperpigmented borders on the right anterior thigh and right chest.

Case #2

A 49-year-old black woman presented for continued management of skin lesions that had been present for more than 20 years with a relapsing, remitting course. The lesions were asymptomatic. She denied any history of preceding trauma before onset of these lesions. A review of systems was negative for arthritis, arthralgias, chest pain, mouth sores, and neurologic complaints. Her medical history was noncontributory, and she was not on any medications, but she smoked. Her physical examination was notable for numerous atrophic, scarred plaques with hyperpigmented borders on the scalp, face, ears, and upper arms.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Nodule on scaphoid fossa of the ear and Vesiculobullous rash with atopic dermatitis. Then take the post-test here.


Case #1Chromomycosis is a subcutaneous fungal infection that is acquired by direct inoculation of the organism into the skin.1 A thorn injury may be recalled in about 16% of patients.1 Chromomycosis occurs most commonly in subtropical climates.2 However, it can...

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Case #1

Chromomycosis is a subcutaneous fungal infection that is acquired by direct inoculation of the organism into the skin.1 A thorn injury may be recalled in about 16% of patients.1 Chromomycosis occurs most commonly in subtropical climates.2 However, it can occasionally be seen in temperate areas such as the United States, Europe, and Canada.2 Males are affected 4 times as frequently as females.1

Chromomycosis is often related to occupational exposure; men aged 20 to 60 years who work in farming, mining, or rural areas are noted to be at higher risk than the general population.2 Immunosuppression, such as that caused by chronic steroid use, can increase the risk of disease. 


The majority of cases are caused by the following organisms: Fonsecaea pedrosoi, Fonsecaea compacta, Fonsecaea monophora, Rhinocladiella aquaspera, Cladophialophora carrionii (formerly Cladosporium carrionii), and Phialophora verrucosa.2 The genus of these causative organisms can be recalled by the mnemonic “Found Round Copper Penny.” Overall, Fonsecaea pedrosoi is the most common cause of chromomycosis.1 These fungi are found in soil, decaying plants, and wood, hence the increased risk in occupational exposures.2 Uncommonly, patients may have co-infection with paracoccidiomycosis, mycetoma, or invasive phaeohyphomycosis, which is typically discovered incidentally on histologic examination.1

The classic description of chromomycosis is that of a papule or nodule that subsequently develops into a verrucous or granulomatous plaque.2 The original papule may grow through direct extension and/or through satellite lesions.1 The most common location of the lesion is the leg, a result of chromomycosis often being acquired from occupational exposures, such as farming or mining.2 Typically, only one extremity is affected.2 However, any site may be affected, including the upper extremity or face.1 The plaque may appear annular as the central portion resolves with scarring.2 The border may appear verrucous or nodular.1 If left untreated, several lesions may coalesce to form a multinodular mass or multiple lesions may be scattered on affected areas.2 There are usually no constitutional symptoms.2 Secondary bacterial infections may result in regional lymphadenitis.1 Rarely, chromomycosis may present as a subcutaneous nodule or mass.2 Other rare manifestations include a lymphangitic pattern or longitudinal melanonychia.1 Involvement of the central nervous system has been reported rarely, both with and without cutaneous involvement.1 In long-standing lesions, squamous cell carcinoma may develop.1

The immune response of the patient may determine the clinical and histologic presentations. A T-helper 2 (Th2) immunologic response may result in verrucous plaques, whereas a Th1 immunologic response may present with an erythematous atrophic plaque.1

Diagnosis requires a skin biopsy. The diagnostic finding on hematoxylin and eosin (H&E) stain is the detection of the fungal organisms known as sclerotic or Medlar bodies.2 These round pigmented organisms are highly characteristic and resemble copper pennies.2 Other typical but nonspecific histologic findings include pseudoepitheliomatous hyperplasia, intraepidermal abscesses, and suppurative and granulomatous inflammation.2 When needed, tissue samples can be analyzed by polymerase chain reaction to determine the specific causative organism.2

The differential diagnoses include other infectious diseases and discoid lupus erythematosus (DLE). Other infectious granulomatous diseases with scarring include cutaneous tuberculosis, tertiary syphilis, blastomycosis, leishmaniasis, and Majocchi granuloma.2 These infectious processes are often clinically nonspecific, and a biopsy for H&E and culture is necessary for diagnosis. Mycetoma is another subcutaneous fungal infection that occurs from implantation, and therefore, it affects a similar demographic. However, mycetoma is often accompanied by edema and draining sinuses and grains.2 DLE is a cutaneous form of lupus that may also have annular borders with central scarring; however, DLE most commonly affects the head and neck. If the extremities are involved, there are almost always accompanying lesions on the head, neck, or ears. In all cases, a biopsy is diagnostic of chromomycosis because of its unique histologic findings. 


Fungi responsible for the majority ofcases of chromomycosis2

Fonsecaea pedrosoi
Fonsecaea compacta
Fonsecaea monophora
Rhinocladiella aquaspera
Cladophialophora carrionii
Phialophora verrucosa

No standard treatment regimen exists. Treatment with 200 to 400 mg/day of itraconazole for at least 6 months may have cure rates of up to 80% to 90%.2 Similarly, voriconazole and posaconazole have also been reported as efficacious, but these agents are less appealing due to the number of side effects, which include visual disturbances, photosensitivity, and risk for hepatotoxicity.2 With prolonged use, voriconazole has been associated with an increased risk for squamous cell carcinoma and melanoma. Terbinafine (500 mg/day for at least 7 months) or 5-flucytosine with oral thiabendazole, intravenous amphotericin B, or an oral triazole has also been reported to be efficacious.2 If the lesion is small, surgical excision with systemic antifungal treatment should be considered.2 Other reported treatments include heat therapy or cryotherapy; however, these modalities are not commonly pursued.2 In severe treatment-refractory cases, amputation may be necessary.1

Our patient began treatment with itraconazole during her hospital stay. However, she was lost to follow-up as her care was transferred to a health-care facility in her home state.


Case #2

Discoid lupus erythematosus (DLE) is one form of chronic cutaneous lupus erythematosus. DLE tends to affect adults, with women receiving the diagnosis more frequently than men at a ratio of 2 to 1.1

Only 5% to 15% of patients with DLE go on to meet diagnostic criteria for systemic lupus erythematosus (SLE).2 However, among patients with SLE, 80% will have cutaneous findings including DLE, malar rash, or photosensitivity.1

Clinically, DLE can vary in appearance depending on the length of time the lesion has been present and the ethnicity of the patient. Regardless of race, lesions often begin as erythematous or pink macules or indurated plaques.1 However, in patients with darker skin, erythema may be difficult to appreciate. With time, lesions develop adherent scale and often evolve to develop atrophy, scarring, and changes in pigment.1 In black patients, this pigmentation change is most characteristically noted to have a rim of hyperpigmentation and central depigmentation.1 In patients with lighter skin, the plaques tend to appear gray or have little alteration in pigment.1 Patients are often noted to have patulous follicles that are reminiscent of ice-pick scars seen in persons with a history of severe acne. When the hyperkeratosis extends into these patulous follicles, small spines may be seen on the undersurface of the scale, sometimes referred to as resembling carpet tacks or langue de chat (cat’s tongue).1

The lesions of DLE may be localized or generalized. In localized DLE, lesions are most commonly located above the neck, favoring the scalp, bridge of nose, malar cheeks, lower lip, and ears.1 On the scalp, lesions often begin as erythematous patches or plaques. Active scalp involvement is usually characterized by perifollicular erythema and easily extractable anagen hairs.1 With time, these areas often become depigmented with scarring alopecia that may be either completely smooth or show dilated follicular openings.1 Active lesions may be tender or pruritic.1 On the lips, DLE may be hyperkeratotic or eroded.1 Hyperkeratotic lesions on lips may be gray or red.1 Eroded lesions are often surrounded by a narrow, red inflammatory zone.1 Localized DLE may also affect the mucosa including the mouth, nose, eye, or vulva.1

In generalized DLE, the chest, upper extremities, head, and neck are most commonly affected. Abnormalities in laboratory test results, including elevated erythrocyte sedimentation rate, antinuclear antibodies, and single-stranded-DNA antibodies, are more commonly seen in generalized DLE than in localized DLE.1

In chronic lesions, squamous cell carcinoma may develop.


The diagnosis of DLE often can be made on clinical grounds alone. However, when the diagnosis is in question, a skin biopsy of a well-established, active lesion is often diagnostic. Histopathology varies based on the stage of the lesion, and early lesions or scarred, inactive lesions may show nonspecific findings. Characteristic histopathologic findings of active DLE are superficial and deep, dense nodular infiltrates comprised mostly of lymphocytes. Vessels, follicles, and the eccrine glands are typically involved.1 A thin epidermis with effacement of the rete ridges is often seen.1 Interface change is almost always present as well. As in most forms of cutaneous lupus, increased mucin and thickening of the basement membrane zone is often seen. 


Key points about discoid lupus erythematosus (DLE)

Of patients with DLE, only 5% to 15% go on to meet criteria for systemic lupus erythematosus (SLE).
Of patients with SLE, not all patients will have features of DLE.
Scarring is almost always seen with DLE.
It is exceedingly rare to have DLE lesions located below the neck in the absence of lesions above the neck. That is, if you have a single lesion that looks like DLE located on the trunk or extremities without any similar lesions on the neck or above, reconsider the diagnosis.
Any non-healing papule or ulcer in chronic lesions of DLE should be biopsied to rule out squamous cell carcinoma.
Sun protection and smoking cessation are necessary components of DLE treatment. Topical, intralesional, and systemic therapies are likely to fail if the patient has continued sun exposure and/or continues to smoke.

In addition to performing a skin biopsy to be examined by routine hematoxylin and eosin (H&E) stain, a biopsy for analysis by direct immunofluorescence may sometimes be useful. Direct immunofluorescence is positive in more than 75% of well-established lesional skin. Unless patients have complaints suggestive of SLE, serologies are usually not ordered as only a minority of patients with DLE go on to develop SLE. However, if patients do have systemic complaints, they should be tested for antinuclear antibodies, anti-double-stranded DNA, C1q, complete blood count (to assess for leukopenia), and a urinalysis (to assess for hematuria and proteinuria) before referral to a rheumatologist. 


The differential diagnosis varies depending on the ethnicity of the patient and whether the lesion is active or chronic. In scarred lesions that have been inactive for extended periods of time, the differential can include vitiligo. Vitiligo will not have the hyperpigmented borders that are often seen in burnt-out DLE. DLE can also mimic deep fungal infections such as chromomycosis. Chromomycosis tends to affect the lower extremities whereas DLE favors the head and neck, trunk, and upper extremities. In white patients, active DLE lesions may appear so erythematous and scaly that they may be mistaken for actinic keratoses. In most cases, the distinction between these entities may be made on clinical grounds and when necessary, a skin biopsy is often diagnostic. 


All patients with cutaneous lupus, including DLE, should be advised to avoid sunlight and wear sunscreen with a high sun-protection factor (SPF) daily.1

For patients with localized DLE, 2.5 to 10 mg/mL of intralesional triamcinolone and/or potent or superpotent topical steroids are effective.1 Second-line treatment for localized disease includes topical calcineurin inhibitors. 


For patients with generalized DLE, systemic therapy should be initiated. First-line treatment is an antimalarial agent. Hydroxychloroquine is often used as it is believed to have minimal side effects compared with chloroquine or quinacrine. Although retinal toxicity with certain antimalarials is a concern, the risk is minimal if the dose of hydroxychloroquine is 6.5 mg/kg/day or less. The typical dose of hydroxychloroquine for patients is 100 mg by mouth twice daily. All patients should be advised on smoking cessation; smoking is thought to decrease the efficacy of hydroxychloroquine by inducing cytochrome P450 1A2. 


For acute disease flares, a short prednisone taper of less than 3 weeks may be beneficial.1 The most common side effect of short courses of prednisone is difficulty sleeping. For refractory cutaneous disease, there have been some reports of improvement with thalidomide, azathioprine, methotrexate, mycophenolate mofetil, and tocilizumab. 


The patient in this case is currently managed with 100 mg of hydroxychloroquine by mouth twice daily and intralesional kenalog. However, the efficacy of her treatments are limited by noncompliance with recommendations for use of sun protection and smoking cessation.

Audrey Chan, MD, is a pediatric dermatology fellow at Texas Children’s Hospital in Houston.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Nodule on scaphoid fossa of the ear and Vesiculobullous rash with atopic dermatitis. Then take the post-test here.


References


  1. James WD, Berger T, Elston D. Andrews’ Diseases of the Skin. 11th ed. Philadelphia, Pa.: Elsevier; 2011:313-314.

  2. Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. 3rd ed. St. Louis, Mo.: Mosby Elsevier; 2011:chap. 77. 


All electronic documents accessed on January 7, 2015.


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