Shawna Langley, MD

Many dermatologic procedures are performed on the face, such as skin biopsies, surgical excisions, and cosmetic procedures, which can increase the risk for accidental ocular injuries.^1,2 Ocular chemical burns have been reported to account for approximately 3% to 20% of ocular injuries^3,4 and are one of the few ocular emergencies dermatologists may encounter in practice. Given the potentially severe consequences of permanent vision changes or loss, it is important to take precautionary steps in preventing chemical exposures and know how to appropriately manage ophthalmic emergencies when they occur.^1,5-8 In this article, we describe a patient with a transient ocular chemical injury from exposure to aluminum chloride hexahydrate that completely resolved with immediate care. We also offer practical guidance for the general dermatologist in the acute management of acidic chemical burns to the eye, highlighting immediate copious irrigation as the most important step in preventing severe permanent damage. Given that aluminum chloride hexahydrate is an acidic solution, we focus predominantly on the approach to acidic chemical exposures to the eye.

Case Report

A 61-year-old woman was seen in the dermatology outpatient clinic for a shave biopsy on the left cheek followed by aluminum chloride application for hemostasis. Following the biopsy, the patient stated she felt the sensation that something had dripped into the left eye and she felt a burning pain. There was a 30- to 60-second delay in irrigation of the eye, as it was at first unclear what had occurred. The patient reported an increased burning sensation, and at that point she was instructed to begin flushing the eye with tap water from the examination room sink for 15 to 20 minutes; she wanted to stop irrigation after a few minutes, and convincing her to continue thorough irrigation was somewhat challenging. It was determined that aluminum chloride hexahydrate had dripped from an oversaturated cotton swab in transit from the tray to the biopsy site.

The patient was urgently directed to the ophthalmology clinic and evaluated by an ophthalmologist within 1 to 2 hours of chemical exposure. Visual acuity of the affected left eye was noted to be 20/30 -2 with correctional glasses, and slit lamp examination revealed moderate injection of the conjunctiva and sclera, and at least 3 punctate epithelial erosions and punctate staining of the inferior aspects of the cornea, consistent with a chemical injury. The remaining ocular examination was normal for both eyes. She was diagnosed with keratitis of the left eye from chemical exposure to aluminum chloride and was prescribed loteprednol etabonate ophthalmic suspension 0.5% and tobramycin ophthalmic solution 0.3% to be applied to the left eye 4 times daily, with follow-up 4 days later.

At follow-up, the patient denied any pain, though she was not using the prescribed eye drops consistently. On examination, the patient showed improvement in visual acuity to 20/20 -2 and complete resolution of the keratitis, with slit lamp examination showing clear conjunctiva, sclera, and cornea. Given complete resolution, the eye drops were discontinued.

Comment

Factors Contributing to Ocular Chemical Injuries
Chemical burns to the eyes during cosmetic or surgical procedures are one of the few acute ocular emergencies dermatologists may encounter in practice. If not managed properly, the eye may be permanently damaged. Therefore, dermatologists must be confident in the initial management of ocular chemical burns (Table 1; Figure).

Mechanism of Ocular Chemical Burns
The extent of injury is predominantly determined by 2 factors: (1) the chemical properties of the substance, and (2) the length of exposure.^5,9,10 Potential chemical exposures and their reported ocular effects are listed in Table 2.^11-21 Alkaline chemical burns often have the gravest outcome, as they can rapidly penetrate into the internal ocular structures, potentially leading to cataracts and glaucoma.⁹ Hydroxyl ions, often found in alkaline chemicals, are capable of rapidly denaturing the corneal matrix and triggering release of proteolytic enzymes through a series of inflammatory responses. Conversely, ocular damage from most acidic chemicals often is limited to the more superficial structures, such as the cornea and conjunctiva, given that acids may cause corneal proteins to coagulate, thus forming a barrier that slows further penetration into deeper structures.⁹ Nonetheless, corneal damage can still have a devastating impact on visual acuity, as the cornea provides 65% to 75% of the eye’s total focusing power.²² For both alkaline and acidic chemicals, immediate profuse irrigation is most critical in determining the clinical course.^23-26 To provide perspective, potent alkaline chemicals may penetrate into the anterior chamber of the eye within 15 seconds,⁹ and delayed initiation of irrigation by even 5 to 15 minutes may lead to irreversible intraocular damage.²⁷

Immediate Management
If potential chemical exposure to the eye is suspected either by the health care provider or patient, immediately irrigate the affected eye(s) for at least 15 to 30 minutes (longer for alkaline burns) with at least 1 to 2 L of irrigation fluid until the pH is between 7 and 7.2.^{3-5,9,27,34,35} Irrigation fluids reported to be used include normal saline, Ringer lactate solution, normal saline with sodium bicarbonate, and balanced salt solution.⁵ If no solutions are readily available, immediate irrigation with tap water is sufficient for diluting and washing away the chemical and has been reported to have better clinical outcomes than delaying irrigation.^5,24-26 Studies have shown that prolonged irrigation corresponded with reduced severity, shortened healing time, shorter in-hospital treatment duration, and quicker return to work.^5,26

If an eye wash station is not available, the patient can gently flush the eye under a sink faucet set to a gentle stream of lukewarm water.^6,7 The health care provider also may manually irrigate the eye. Necessary equipment includes a large syringe or clean eyecup, irrigating fluid, local anesthetic drops for comfort, a towel to soak up excessive fluid, and a bowl or kidney dish to collect the irrigated fluid.³⁴ Providers should first wash their hands. If necessary, anesthetic eye drops may be added for comfort. Lay a towel over the patient’s neck and shoulders and position the patient at a comfortable angle. Place a bowl adjacent to the patient’s cheek to collect the irrigating fluid and have the patient tilt his/her head such that the irrigated fluid would flow into the bowl. Pour a steady stream of the irrigating fluid over the eye from a height of no more than 5 cm.^6,7,34

During irrigation, ensure that the patient’s eye(s) is wide open and that all ocular surfaces, including the area underneath the eyelids, are thoroughly washed; everting the eyelids may be beneficial. Ask the patient to move his/her eye(s) in all directions while irrigating. If available, place a litmus strip in the conjunctival fornix to ensure that the goal pH of 7 to 7.2 is reached.⁹ The pH should be rechecked every 15 to 30 minutes to ensure there has been no change, as hidden crystalized chemical particles may continue to elute chemicals, causing further injury.³ Contact lenses, if present, should be removed as soon as practical, as lenses can trap chemicals; however, immediate initiation of irrigation should not be delayed⁸ (Table 1).

Identify and verify the chemical suspected to have been exposed to the patient’s eye. The material safety data sheet, which may often be found online if a hard copy is not available, may provide valuable information for the ophthalmologist.³⁶ After thorough irrigation, refer the patient urgently to ophthalmology or the emergency department for prompt evaluation. The emergency department is frequently equipped with polymethylmethacrylate scleral lenses, also called Morgan Lens, which consist of a plastic lens connected via tubing to a bag of irrigation fluid (eg, Ringer lactate solution), allowing for prolonged continuous irrigation of the conjunctiva and cornea. The ophthalmologist will conduct a visual acuity test and complete a thorough eye examination to assess the extent of ischemic injury to the conjunctiva or sclera and damage to the corneal epithelium and internal ocular structures.⁹

Generally, topical antibiotics, artificial tears, and topical steroids may be provided to patients with mild injury with close follow-up.^9,37 For higher-grade injuries, broad-spectrum topical antibiotics, oral antibiotics, topical corticosteroids, vitamin C, and surgical treatments may be additionally recommended (Table 3). Long-term follow-up may be recommended by the ophthalmologist to monitor for potential late complications, such as glaucoma from damage to the trabecular meshwork, corneal abnormalities and limbal stem cell deficiency, symblepharon formation, or eyelid abnormalities.⁹

Conclusion

We report a case of a transient chemical burn to the eye secondary to exposure to aluminum chloride hexahydrate. Complete resolution of the injury was achieved with prompt irrigation and urgent medical management by ophthalmology. This case emphasizes the potential for ocular emergencies in the dermatology setting and highlights the steps for appropriate management should a chemical burn to the eye occur. We emphasize the importance of immediate profuse irrigation for 15 to 30 minutes and urgent evaluation by an ophthalmologist. Dermatologists should be cognizant of potential hazards to the eye during facial procedures and always take proper precautions to decrease the risk for ocular injuries.

Many dermatologic procedures are performed on the face, such as skin biopsies, surgical excisions, and cosmetic procedures, which can increase the risk for accidental ocular injuries.^1,2 Ocular chemical burns have been reported to account for approximately 3% to 20% of ocular injuries^3,4 and are one of the few ocular emergencies dermatologists may encounter in practice. Given the potentially severe consequences of permanent vision changes or loss, it is important to take precautionary steps in preventing chemical exposures and know how to appropriately manage ophthalmic emergencies when they occur.^1,5-8 In this article, we describe a patient with a transient ocular chemical injury from exposure to aluminum chloride hexahydrate that completely resolved with immediate care. We also offer practical guidance for the general dermatologist in the acute management of acidic chemical burns to the eye, highlighting immediate copious irrigation as the most important step in preventing severe permanent damage. Given that aluminum chloride hexahydrate is an acidic solution, we focus predominantly on the approach to acidic chemical exposures to the eye.

Case Report

A 61-year-old woman was seen in the dermatology outpatient clinic for a shave biopsy on the left cheek followed by aluminum chloride application for hemostasis. Following the biopsy, the patient stated she felt the sensation that something had dripped into the left eye and she felt a burning pain. There was a 30- to 60-second delay in irrigation of the eye, as it was at first unclear what had occurred. The patient reported an increased burning sensation, and at that point she was instructed to begin flushing the eye with tap water from the examination room sink for 15 to 20 minutes; she wanted to stop irrigation after a few minutes, and convincing her to continue thorough irrigation was somewhat challenging. It was determined that aluminum chloride hexahydrate had dripped from an oversaturated cotton swab in transit from the tray to the biopsy site.

The patient was urgently directed to the ophthalmology clinic and evaluated by an ophthalmologist within 1 to 2 hours of chemical exposure. Visual acuity of the affected left eye was noted to be 20/30 -2 with correctional glasses, and slit lamp examination revealed moderate injection of the conjunctiva and sclera, and at least 3 punctate epithelial erosions and punctate staining of the inferior aspects of the cornea, consistent with a chemical injury. The remaining ocular examination was normal for both eyes. She was diagnosed with keratitis of the left eye from chemical exposure to aluminum chloride and was prescribed loteprednol etabonate ophthalmic suspension 0.5% and tobramycin ophthalmic solution 0.3% to be applied to the left eye 4 times daily, with follow-up 4 days later.

At follow-up, the patient denied any pain, though she was not using the prescribed eye drops consistently. On examination, the patient showed improvement in visual acuity to 20/20 -2 and complete resolution of the keratitis, with slit lamp examination showing clear conjunctiva, sclera, and cornea. Given complete resolution, the eye drops were discontinued.

Comment

Factors Contributing to Ocular Chemical Injuries
Chemical burns to the eyes during cosmetic or surgical procedures are one of the few acute ocular emergencies dermatologists may encounter in practice. If not managed properly, the eye may be permanently damaged. Therefore, dermatologists must be confident in the initial management of ocular chemical burns (Table 1; Figure).

Mechanism of Ocular Chemical Burns
The extent of injury is predominantly determined by 2 factors: (1) the chemical properties of the substance, and (2) the length of exposure.^5,9,10 Potential chemical exposures and their reported ocular effects are listed in Table 2.^11-21 Alkaline chemical burns often have the gravest outcome, as they can rapidly penetrate into the internal ocular structures, potentially leading to cataracts and glaucoma.⁹ Hydroxyl ions, often found in alkaline chemicals, are capable of rapidly denaturing the corneal matrix and triggering release of proteolytic enzymes through a series of inflammatory responses. Conversely, ocular damage from most acidic chemicals often is limited to the more superficial structures, such as the cornea and conjunctiva, given that acids may cause corneal proteins to coagulate, thus forming a barrier that slows further penetration into deeper structures.⁹ Nonetheless, corneal damage can still have a devastating impact on visual acuity, as the cornea provides 65% to 75% of the eye’s total focusing power.²² For both alkaline and acidic chemicals, immediate profuse irrigation is most critical in determining the clinical course.^23-26 To provide perspective, potent alkaline chemicals may penetrate into the anterior chamber of the eye within 15 seconds,⁹ and delayed initiation of irrigation by even 5 to 15 minutes may lead to irreversible intraocular damage.²⁷

Immediate Management
If potential chemical exposure to the eye is suspected either by the health care provider or patient, immediately irrigate the affected eye(s) for at least 15 to 30 minutes (longer for alkaline burns) with at least 1 to 2 L of irrigation fluid until the pH is between 7 and 7.2.^{3-5,9,27,34,35} Irrigation fluids reported to be used include normal saline, Ringer lactate solution, normal saline with sodium bicarbonate, and balanced salt solution.⁵ If no solutions are readily available, immediate irrigation with tap water is sufficient for diluting and washing away the chemical and has been reported to have better clinical outcomes than delaying irrigation.^5,24-26 Studies have shown that prolonged irrigation corresponded with reduced severity, shortened healing time, shorter in-hospital treatment duration, and quicker return to work.^5,26

If an eye wash station is not available, the patient can gently flush the eye under a sink faucet set to a gentle stream of lukewarm water.^6,7 The health care provider also may manually irrigate the eye. Necessary equipment includes a large syringe or clean eyecup, irrigating fluid, local anesthetic drops for comfort, a towel to soak up excessive fluid, and a bowl or kidney dish to collect the irrigated fluid.³⁴ Providers should first wash their hands. If necessary, anesthetic eye drops may be added for comfort. Lay a towel over the patient’s neck and shoulders and position the patient at a comfortable angle. Place a bowl adjacent to the patient’s cheek to collect the irrigating fluid and have the patient tilt his/her head such that the irrigated fluid would flow into the bowl. Pour a steady stream of the irrigating fluid over the eye from a height of no more than 5 cm.^6,7,34

During irrigation, ensure that the patient’s eye(s) is wide open and that all ocular surfaces, including the area underneath the eyelids, are thoroughly washed; everting the eyelids may be beneficial. Ask the patient to move his/her eye(s) in all directions while irrigating. If available, place a litmus strip in the conjunctival fornix to ensure that the goal pH of 7 to 7.2 is reached.⁹ The pH should be rechecked every 15 to 30 minutes to ensure there has been no change, as hidden crystalized chemical particles may continue to elute chemicals, causing further injury.³ Contact lenses, if present, should be removed as soon as practical, as lenses can trap chemicals; however, immediate initiation of irrigation should not be delayed⁸ (Table 1).

Identify and verify the chemical suspected to have been exposed to the patient’s eye. The material safety data sheet, which may often be found online if a hard copy is not available, may provide valuable information for the ophthalmologist.³⁶ After thorough irrigation, refer the patient urgently to ophthalmology or the emergency department for prompt evaluation. The emergency department is frequently equipped with polymethylmethacrylate scleral lenses, also called Morgan Lens, which consist of a plastic lens connected via tubing to a bag of irrigation fluid (eg, Ringer lactate solution), allowing for prolonged continuous irrigation of the conjunctiva and cornea. The ophthalmologist will conduct a visual acuity test and complete a thorough eye examination to assess the extent of ischemic injury to the conjunctiva or sclera and damage to the corneal epithelium and internal ocular structures.⁹

Generally, topical antibiotics, artificial tears, and topical steroids may be provided to patients with mild injury with close follow-up.^9,37 For higher-grade injuries, broad-spectrum topical antibiotics, oral antibiotics, topical corticosteroids, vitamin C, and surgical treatments may be additionally recommended (Table 3). Long-term follow-up may be recommended by the ophthalmologist to monitor for potential late complications, such as glaucoma from damage to the trabecular meshwork, corneal abnormalities and limbal stem cell deficiency, symblepharon formation, or eyelid abnormalities.⁹

Conclusion

We report a case of a transient chemical burn to the eye secondary to exposure to aluminum chloride hexahydrate. Complete resolution of the injury was achieved with prompt irrigation and urgent medical management by ophthalmology. This case emphasizes the potential for ocular emergencies in the dermatology setting and highlights the steps for appropriate management should a chemical burn to the eye occur. We emphasize the importance of immediate profuse irrigation for 15 to 30 minutes and urgent evaluation by an ophthalmologist. Dermatologists should be cognizant of potential hazards to the eye during facial procedures and always take proper precautions to decrease the risk for ocular injuries.

Many dermatologic procedures are performed on the face, such as skin biopsies, surgical excisions, and cosmetic procedures, which can increase the risk for accidental ocular injuries.^1,2 Ocular chemical burns have been reported to account for approximately 3% to 20% of ocular injuries^3,4 and are one of the few ocular emergencies dermatologists may encounter in practice. Given the potentially severe consequences of permanent vision changes or loss, it is important to take precautionary steps in preventing chemical exposures and know how to appropriately manage ophthalmic emergencies when they occur.^1,5-8 In this article, we describe a patient with a transient ocular chemical injury from exposure to aluminum chloride hexahydrate that completely resolved with immediate care. We also offer practical guidance for the general dermatologist in the acute management of acidic chemical burns to the eye, highlighting immediate copious irrigation as the most important step in preventing severe permanent damage. Given that aluminum chloride hexahydrate is an acidic solution, we focus predominantly on the approach to acidic chemical exposures to the eye.

Case Report

A 61-year-old woman was seen in the dermatology outpatient clinic for a shave biopsy on the left cheek followed by aluminum chloride application for hemostasis. Following the biopsy, the patient stated she felt the sensation that something had dripped into the left eye and she felt a burning pain. There was a 30- to 60-second delay in irrigation of the eye, as it was at first unclear what had occurred. The patient reported an increased burning sensation, and at that point she was instructed to begin flushing the eye with tap water from the examination room sink for 15 to 20 minutes; she wanted to stop irrigation after a few minutes, and convincing her to continue thorough irrigation was somewhat challenging. It was determined that aluminum chloride hexahydrate had dripped from an oversaturated cotton swab in transit from the tray to the biopsy site.

The patient was urgently directed to the ophthalmology clinic and evaluated by an ophthalmologist within 1 to 2 hours of chemical exposure. Visual acuity of the affected left eye was noted to be 20/30 -2 with correctional glasses, and slit lamp examination revealed moderate injection of the conjunctiva and sclera, and at least 3 punctate epithelial erosions and punctate staining of the inferior aspects of the cornea, consistent with a chemical injury. The remaining ocular examination was normal for both eyes. She was diagnosed with keratitis of the left eye from chemical exposure to aluminum chloride and was prescribed loteprednol etabonate ophthalmic suspension 0.5% and tobramycin ophthalmic solution 0.3% to be applied to the left eye 4 times daily, with follow-up 4 days later.

At follow-up, the patient denied any pain, though she was not using the prescribed eye drops consistently. On examination, the patient showed improvement in visual acuity to 20/20 -2 and complete resolution of the keratitis, with slit lamp examination showing clear conjunctiva, sclera, and cornea. Given complete resolution, the eye drops were discontinued.

Comment

Factors Contributing to Ocular Chemical Injuries
Chemical burns to the eyes during cosmetic or surgical procedures are one of the few acute ocular emergencies dermatologists may encounter in practice. If not managed properly, the eye may be permanently damaged. Therefore, dermatologists must be confident in the initial management of ocular chemical burns (Table 1; Figure).

Mechanism of Ocular Chemical Burns
The extent of injury is predominantly determined by 2 factors: (1) the chemical properties of the substance, and (2) the length of exposure.^5,9,10 Potential chemical exposures and their reported ocular effects are listed in Table 2.^11-21 Alkaline chemical burns often have the gravest outcome, as they can rapidly penetrate into the internal ocular structures, potentially leading to cataracts and glaucoma.⁹ Hydroxyl ions, often found in alkaline chemicals, are capable of rapidly denaturing the corneal matrix and triggering release of proteolytic enzymes through a series of inflammatory responses. Conversely, ocular damage from most acidic chemicals often is limited to the more superficial structures, such as the cornea and conjunctiva, given that acids may cause corneal proteins to coagulate, thus forming a barrier that slows further penetration into deeper structures.⁹ Nonetheless, corneal damage can still have a devastating impact on visual acuity, as the cornea provides 65% to 75% of the eye’s total focusing power.²² For both alkaline and acidic chemicals, immediate profuse irrigation is most critical in determining the clinical course.^23-26 To provide perspective, potent alkaline chemicals may penetrate into the anterior chamber of the eye within 15 seconds,⁹ and delayed initiation of irrigation by even 5 to 15 minutes may lead to irreversible intraocular damage.²⁷

Immediate Management
If potential chemical exposure to the eye is suspected either by the health care provider or patient, immediately irrigate the affected eye(s) for at least 15 to 30 minutes (longer for alkaline burns) with at least 1 to 2 L of irrigation fluid until the pH is between 7 and 7.2.^{3-5,9,27,34,35} Irrigation fluids reported to be used include normal saline, Ringer lactate solution, normal saline with sodium bicarbonate, and balanced salt solution.⁵ If no solutions are readily available, immediate irrigation with tap water is sufficient for diluting and washing away the chemical and has been reported to have better clinical outcomes than delaying irrigation.^5,24-26 Studies have shown that prolonged irrigation corresponded with reduced severity, shortened healing time, shorter in-hospital treatment duration, and quicker return to work.^5,26

If an eye wash station is not available, the patient can gently flush the eye under a sink faucet set to a gentle stream of lukewarm water.^6,7 The health care provider also may manually irrigate the eye. Necessary equipment includes a large syringe or clean eyecup, irrigating fluid, local anesthetic drops for comfort, a towel to soak up excessive fluid, and a bowl or kidney dish to collect the irrigated fluid.³⁴ Providers should first wash their hands. If necessary, anesthetic eye drops may be added for comfort. Lay a towel over the patient’s neck and shoulders and position the patient at a comfortable angle. Place a bowl adjacent to the patient’s cheek to collect the irrigating fluid and have the patient tilt his/her head such that the irrigated fluid would flow into the bowl. Pour a steady stream of the irrigating fluid over the eye from a height of no more than 5 cm.^6,7,34

During irrigation, ensure that the patient’s eye(s) is wide open and that all ocular surfaces, including the area underneath the eyelids, are thoroughly washed; everting the eyelids may be beneficial. Ask the patient to move his/her eye(s) in all directions while irrigating. If available, place a litmus strip in the conjunctival fornix to ensure that the goal pH of 7 to 7.2 is reached.⁹ The pH should be rechecked every 15 to 30 minutes to ensure there has been no change, as hidden crystalized chemical particles may continue to elute chemicals, causing further injury.³ Contact lenses, if present, should be removed as soon as practical, as lenses can trap chemicals; however, immediate initiation of irrigation should not be delayed⁸ (Table 1).

Identify and verify the chemical suspected to have been exposed to the patient’s eye. The material safety data sheet, which may often be found online if a hard copy is not available, may provide valuable information for the ophthalmologist.³⁶ After thorough irrigation, refer the patient urgently to ophthalmology or the emergency department for prompt evaluation. The emergency department is frequently equipped with polymethylmethacrylate scleral lenses, also called Morgan Lens, which consist of a plastic lens connected via tubing to a bag of irrigation fluid (eg, Ringer lactate solution), allowing for prolonged continuous irrigation of the conjunctiva and cornea. The ophthalmologist will conduct a visual acuity test and complete a thorough eye examination to assess the extent of ischemic injury to the conjunctiva or sclera and damage to the corneal epithelium and internal ocular structures.⁹

Generally, topical antibiotics, artificial tears, and topical steroids may be provided to patients with mild injury with close follow-up.^9,37 For higher-grade injuries, broad-spectrum topical antibiotics, oral antibiotics, topical corticosteroids, vitamin C, and surgical treatments may be additionally recommended (Table 3). Long-term follow-up may be recommended by the ophthalmologist to monitor for potential late complications, such as glaucoma from damage to the trabecular meshwork, corneal abnormalities and limbal stem cell deficiency, symblepharon formation, or eyelid abnormalities.⁹

Conclusion

We report a case of a transient chemical burn to the eye secondary to exposure to aluminum chloride hexahydrate. Complete resolution of the injury was achieved with prompt irrigation and urgent medical management by ophthalmology. This case emphasizes the potential for ocular emergencies in the dermatology setting and highlights the steps for appropriate management should a chemical burn to the eye occur. We emphasize the importance of immediate profuse irrigation for 15 to 30 minutes and urgent evaluation by an ophthalmologist. Dermatologists should be cognizant of potential hazards to the eye during facial procedures and always take proper precautions to decrease the risk for ocular injuries.

Case Report

A 54-year-old woman presented with a painful pruritic rash on the hands and feet of 7 years’ duration. She reported intermittent joint pain but denied muscle weakness. Physical examination revealed fissured fingertips and heavy scaling of the palms and lateral fingers (Figure 1). Violaceous scaly papules were seen on the distal and proximal interphalangeal joints (Figure 2). A severe plantar keratoderma also was noted (Figure 3). Pink scaly plaques were present on the bilateral elbows and postauricular skin. Diffuse mat telangiectases covered the malar skin. Extensive poikilodermatous skin changes covered approximately 20% of the total body surface area. Salt-and-pepper patches and papules were noted over the bilateral thighs. She reported an uncertain history of recent radiographs of one or both hands, which showed no joint degeneration characteristic of psoriatic arthritis. She previously had been given a diagnosis of psoriasis by an outside dermatologist but was not responding to topical therapy.

Several skin biopsies showed histologic evidence of dermatomyositis (DM)(Figure 4). Prominent basement thickening also was seen on periodic acid–Schiff staining (not shown). Laboratory workup showed negative antinuclear antibodies and anti–Jo-1, anti-Ku, and anti-Mi2 antibodies. Muscle enzymes including creatinine kinase and aldolase were within reference range. Pelvic ultrasonography and mammography were negative. Pulmonary function tests were unremarkable. High-resolution chest computed tomography (CT) was ordered because of a history of chronic cough; however, no evidence of malignancy or interstitial lung disease was seen. The patient was diagnosed with amyopathic dermatomyositis (ADM). Rheumatology was consulted and initiated oral hydroxychloroquine therapy. After 3 months, the patient’s cutaneous disease did not respond and she reported having headaches associated with this medication; therefore, methotrexate was started. Within 2 months of treatment, full resolution of the plantar keratoderma (Figure 5) and clearance of the scaling/fissuring of the hands as well as the psoriatic-appearing plaques on the elbows was noted.

Comment

Amyopathic DM is a subset of DM that accounts for 10% to 20% of DM cases.^1,2 Sontheimer’s³ diagnostic criteria for ADM require histopathologic confirmation of the hallmark skin findings of classic DM and lack of muscle weakness or muscle enzyme (creatine kinase/aldolase) elevation for at least 2 years.

Similar to classic DM, ADM typically presents in the fifth decade of life and has a female predilection.^1,4 The term hypomyopathic DM is used to describe patients who exhibit classic skin findings and evidence of muscle involvement on magnetic resonance imaging, electromyography, biopsy, or serum enzymes but have no clinical evidence of muscle weakness for at least 6 months. Together, hypomyopathic DM and ADM are referred to as clinically ADM (CADM). Patients who have met the criteria for hypomyopathic DM or ADM may later develop frank myopathy, progressing to a diagnosis of CADM, which may occur in as many as 10% to 13% of cases of CADM.^1,2 Clinical evidence of muscle weakness typically is heralded by elevation of creatine kinase and aldolase; therefore, patients with ADM should have muscle enzymes periodically checked.

Cutaneous findings of ADM are the same as the hallmark skin findings in CADM.³ Poikiloderma appears as thin telangiectatic skin in a background of mottled hyperpigmentation and hypopigmentation. It represents chronic inflammation and often occurs in sun-exposed areas. Poikiloderma located on the posterior neck and shoulders is known as the shawl sign and on the lateral thighs as the holster sign.⁵ The term mechanic’s hands is used to describe the clinical finding of palmar erythema with scaling and fissuring of the fingertips.⁶ Scalp findings include erythematous, atrophic, scaly plaques resembling psoriasis and nonscarring alopecia.⁷ Gottron papules are nearly pathognomonic for DM. These violaceous papules often are pruritic and found over the finger joints, in contrast to the hand rash of lupus erythematosus that involves the skin between finger joints.⁸ Psoriatic-appearing plaques overlying the elbows and knees are known as Gottron sign and can contribute to misdiagnosis as psoriasis.⁸ The classic heliotrope rash presents as a violaceous hue in the periorbital area and may be associated with periorbital edema.⁹ Calcinosis cutis is common in CADM but rarely is reported in ADM.¹⁰ Nail findings include periungual hyperemia, cuticular overgrowth, and nail bed changes due to avascular areas and dilated capillaries. The cutaneous histopathologic findings in ADM are the same as with CADM: a smudged dermoepidermal interface, vacuolar alterations of the basal layer, and dermal mucin deposits.

Palmoplantar keratoderma rarely is reported as a cutaneous finding in DM. The finding of keratoderma has mainly been reported in association with Wong-type DM, a rare subtype of DM with features of pityriasis rubra pilaris.^11-13 Palmoplantar keratoderma also has been reported in a case of an ADM-like hydroxyurea-induced eruption¹⁴ and as an early presenting feature in one patient with CADM and one with juvenile DM.^15,16

The autoantibody profile in patients with ADM varies from that of CADM and can be helpful in both diagnosis and prognosis. Similar to CADM, the majority of patients with ADM have positive antinuclear antibodies.^2,17 Anti–Jo-1 (an anti–aminoacyl-transfer RNA synthetase) antibody frequently is found in CADM but rarely in ADM.² Anti–Jo-1 is predictive of interstitial lung disease (ILD) in CADM. Positive anti–Jo-1 in combination with Raynaud phenomenon and mechanic’s hands is referred to as antisynthetase syndrome in patients with CADM.^18,19 An antibody uniquely linked with CADM is the anti–CADM-140/MDA5 antibody and can be a marker of rapidly progressing ILD in these patients.²⁰ Anti–Mi-2 is another myositis-specific antibody not commonly found in ADM but is present in 15% to 30% of DM cases.^2,21 In CADM, the anti–Mi-2 antibody is associated with the shawl sign, ragged cuticles, and carpal tunnel syndrome and has a favorable prognosis.^17,21 Myositis-associated autoantibodies (eg, anti-Ku) are found in patients with symptoms overlapping both DM and scleroderma or other connective tissue diseases.²² More recently described, the anti-p155/140 antibody is highly specific (up to 89%) for occult malignancy in DM.²³

Lung disease is an important association in ADM. When it develops, it may be more aggressive compared to lung disease associated with CADM.^24-26 In a systematic review of 197 cases of ADM by Gerami et al,² 10% of patients had ILD, and it was fatal in 42% of cases. Most cases of ILD associated with CADM were diagnosed as interstitial pneumonitis or diffuse alveolar disease; bronchiolitis obliterans organizing pneumonia and basilar fibrosis also were recorded.² Anti–Jo-1 antibodies often accompany lung disease in CADM but are not typically found in lung disease associated with ADM. The anti–CADM-140/MDA5 antibody is associated with an increased risk for rapidly progressing ILD in patients with CADM.²⁰ Recommended baseline screening for lung disease in DM includes chest radiography, pulmonary function tests with diffusion capacity,⁸ and in some instances high-resolution chest CT.²⁷ Follow-up visits should include screening for symptoms of ILD such as cough, shortness of breath, or dyspnea. Treatment of myopathy-associated ILD is systemic steroids combined with various immunosuppressants including cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, and intravenous immunoglobulin.^28,29

The risk of malignancy in ADM is thought to be similar to the rate of 20% to 25% found in CADM.^1,30-32 The most commonly reported malignancies associated with ADM are nasopharyngeal, breast, lung, ovarian, colorectal, pancreatic, and stomach cancers and lymphoma/leukemia.^2,33 Patients with ADM should be screened for malignancy at diagnosis, then yearly for 3 years.^8,31,33 In addition to history, physical examination, and age/sex-appropriate screening, a complete blood cell count, chemistry panel, urinalysis, stool guaiac, CA 125, CA 19-9, chest radiograph, and abdominal ultrasound should be performed. For women, mammography and pelvic ultrasonography should be completed.³¹ Some experts also recommend a full-body CT scan. Because Asian patients have a higher risk for nasopharyngeal carcinoma, referral to an ear, nose, and throat surgeon for direct visualization also can be considered.³³ The risk of cancer in patients with DM compared to the general population is increased for at least the first 5 years after diagnosis, but most associated cancers are found within the first 3 years.³⁴

Several therapies have been found useful in ADM. Because lesions often are photoexacerbated, sun protection is essential. Antimalarials such as hydroxychloroquine are considered first-line therapy. Clinicians must be aware of 2 possible hydroxychloroquine side effects that can uniquely confuse the clinical picture in ADM. The first is a rash, most often morbilliform and pruritic, that occurs in DM more frequently than in other diseases.³⁵ The second is a myopathy found in as many as 6.7% of patients using antimalarials for rheumatic disease,³⁶ which can clinically mimic the progression of ADM to CADM.³⁷ Two small retrospective case series found that methotrexate was beneficial in ADM.^38,39 Methotrexate also has been reported as an efficacious treatment of ILD in patients with connective tissue diseases.^40,41 Intravenous immunoglobulin and other immunosuppressants are additional agents to be considered.⁴²

In summary, ADM is an important subset of DM and is more likely to present to dermatology practices than to other specialists. Amyopathic DM shares cutaneous findings with DM, and both overlap and differ with respect to other key disease characteristics including autoantibody profile, associated lung disease, and malignancy risk. Palmoplantar keratoderma is a rarely reported skin finding in DM. We report a case of ADM with the unique finding of severe plantar keratoderma. The fact that our patient’s keratoderma and other skin findings resolved concomitantly during methotrexate therapy leads us to believe that the keratoderma was a unique skin manifestation of the ADM itself.

Case Report

A 54-year-old woman presented with a painful pruritic rash on the hands and feet of 7 years’ duration. She reported intermittent joint pain but denied muscle weakness. Physical examination revealed fissured fingertips and heavy scaling of the palms and lateral fingers (Figure 1). Violaceous scaly papules were seen on the distal and proximal interphalangeal joints (Figure 2). A severe plantar keratoderma also was noted (Figure 3). Pink scaly plaques were present on the bilateral elbows and postauricular skin. Diffuse mat telangiectases covered the malar skin. Extensive poikilodermatous skin changes covered approximately 20% of the total body surface area. Salt-and-pepper patches and papules were noted over the bilateral thighs. She reported an uncertain history of recent radiographs of one or both hands, which showed no joint degeneration characteristic of psoriatic arthritis. She previously had been given a diagnosis of psoriasis by an outside dermatologist but was not responding to topical therapy.

Several skin biopsies showed histologic evidence of dermatomyositis (DM)(Figure 4). Prominent basement thickening also was seen on periodic acid–Schiff staining (not shown). Laboratory workup showed negative antinuclear antibodies and anti–Jo-1, anti-Ku, and anti-Mi2 antibodies. Muscle enzymes including creatinine kinase and aldolase were within reference range. Pelvic ultrasonography and mammography were negative. Pulmonary function tests were unremarkable. High-resolution chest computed tomography (CT) was ordered because of a history of chronic cough; however, no evidence of malignancy or interstitial lung disease was seen. The patient was diagnosed with amyopathic dermatomyositis (ADM). Rheumatology was consulted and initiated oral hydroxychloroquine therapy. After 3 months, the patient’s cutaneous disease did not respond and she reported having headaches associated with this medication; therefore, methotrexate was started. Within 2 months of treatment, full resolution of the plantar keratoderma (Figure 5) and clearance of the scaling/fissuring of the hands as well as the psoriatic-appearing plaques on the elbows was noted.

Comment

Amyopathic DM is a subset of DM that accounts for 10% to 20% of DM cases.^1,2 Sontheimer’s³ diagnostic criteria for ADM require histopathologic confirmation of the hallmark skin findings of classic DM and lack of muscle weakness or muscle enzyme (creatine kinase/aldolase) elevation for at least 2 years.

Similar to classic DM, ADM typically presents in the fifth decade of life and has a female predilection.^1,4 The term hypomyopathic DM is used to describe patients who exhibit classic skin findings and evidence of muscle involvement on magnetic resonance imaging, electromyography, biopsy, or serum enzymes but have no clinical evidence of muscle weakness for at least 6 months. Together, hypomyopathic DM and ADM are referred to as clinically ADM (CADM). Patients who have met the criteria for hypomyopathic DM or ADM may later develop frank myopathy, progressing to a diagnosis of CADM, which may occur in as many as 10% to 13% of cases of CADM.^1,2 Clinical evidence of muscle weakness typically is heralded by elevation of creatine kinase and aldolase; therefore, patients with ADM should have muscle enzymes periodically checked.

Cutaneous findings of ADM are the same as the hallmark skin findings in CADM.³ Poikiloderma appears as thin telangiectatic skin in a background of mottled hyperpigmentation and hypopigmentation. It represents chronic inflammation and often occurs in sun-exposed areas. Poikiloderma located on the posterior neck and shoulders is known as the shawl sign and on the lateral thighs as the holster sign.⁵ The term mechanic’s hands is used to describe the clinical finding of palmar erythema with scaling and fissuring of the fingertips.⁶ Scalp findings include erythematous, atrophic, scaly plaques resembling psoriasis and nonscarring alopecia.⁷ Gottron papules are nearly pathognomonic for DM. These violaceous papules often are pruritic and found over the finger joints, in contrast to the hand rash of lupus erythematosus that involves the skin between finger joints.⁸ Psoriatic-appearing plaques overlying the elbows and knees are known as Gottron sign and can contribute to misdiagnosis as psoriasis.⁸ The classic heliotrope rash presents as a violaceous hue in the periorbital area and may be associated with periorbital edema.⁹ Calcinosis cutis is common in CADM but rarely is reported in ADM.¹⁰ Nail findings include periungual hyperemia, cuticular overgrowth, and nail bed changes due to avascular areas and dilated capillaries. The cutaneous histopathologic findings in ADM are the same as with CADM: a smudged dermoepidermal interface, vacuolar alterations of the basal layer, and dermal mucin deposits.

Palmoplantar keratoderma rarely is reported as a cutaneous finding in DM. The finding of keratoderma has mainly been reported in association with Wong-type DM, a rare subtype of DM with features of pityriasis rubra pilaris.^11-13 Palmoplantar keratoderma also has been reported in a case of an ADM-like hydroxyurea-induced eruption¹⁴ and as an early presenting feature in one patient with CADM and one with juvenile DM.^15,16

The autoantibody profile in patients with ADM varies from that of CADM and can be helpful in both diagnosis and prognosis. Similar to CADM, the majority of patients with ADM have positive antinuclear antibodies.^2,17 Anti–Jo-1 (an anti–aminoacyl-transfer RNA synthetase) antibody frequently is found in CADM but rarely in ADM.² Anti–Jo-1 is predictive of interstitial lung disease (ILD) in CADM. Positive anti–Jo-1 in combination with Raynaud phenomenon and mechanic’s hands is referred to as antisynthetase syndrome in patients with CADM.^18,19 An antibody uniquely linked with CADM is the anti–CADM-140/MDA5 antibody and can be a marker of rapidly progressing ILD in these patients.²⁰ Anti–Mi-2 is another myositis-specific antibody not commonly found in ADM but is present in 15% to 30% of DM cases.^2,21 In CADM, the anti–Mi-2 antibody is associated with the shawl sign, ragged cuticles, and carpal tunnel syndrome and has a favorable prognosis.^17,21 Myositis-associated autoantibodies (eg, anti-Ku) are found in patients with symptoms overlapping both DM and scleroderma or other connective tissue diseases.²² More recently described, the anti-p155/140 antibody is highly specific (up to 89%) for occult malignancy in DM.²³

Lung disease is an important association in ADM. When it develops, it may be more aggressive compared to lung disease associated with CADM.^24-26 In a systematic review of 197 cases of ADM by Gerami et al,² 10% of patients had ILD, and it was fatal in 42% of cases. Most cases of ILD associated with CADM were diagnosed as interstitial pneumonitis or diffuse alveolar disease; bronchiolitis obliterans organizing pneumonia and basilar fibrosis also were recorded.² Anti–Jo-1 antibodies often accompany lung disease in CADM but are not typically found in lung disease associated with ADM. The anti–CADM-140/MDA5 antibody is associated with an increased risk for rapidly progressing ILD in patients with CADM.²⁰ Recommended baseline screening for lung disease in DM includes chest radiography, pulmonary function tests with diffusion capacity,⁸ and in some instances high-resolution chest CT.²⁷ Follow-up visits should include screening for symptoms of ILD such as cough, shortness of breath, or dyspnea. Treatment of myopathy-associated ILD is systemic steroids combined with various immunosuppressants including cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, and intravenous immunoglobulin.^28,29

The risk of malignancy in ADM is thought to be similar to the rate of 20% to 25% found in CADM.^1,30-32 The most commonly reported malignancies associated with ADM are nasopharyngeal, breast, lung, ovarian, colorectal, pancreatic, and stomach cancers and lymphoma/leukemia.^2,33 Patients with ADM should be screened for malignancy at diagnosis, then yearly for 3 years.^8,31,33 In addition to history, physical examination, and age/sex-appropriate screening, a complete blood cell count, chemistry panel, urinalysis, stool guaiac, CA 125, CA 19-9, chest radiograph, and abdominal ultrasound should be performed. For women, mammography and pelvic ultrasonography should be completed.³¹ Some experts also recommend a full-body CT scan. Because Asian patients have a higher risk for nasopharyngeal carcinoma, referral to an ear, nose, and throat surgeon for direct visualization also can be considered.³³ The risk of cancer in patients with DM compared to the general population is increased for at least the first 5 years after diagnosis, but most associated cancers are found within the first 3 years.³⁴

Several therapies have been found useful in ADM. Because lesions often are photoexacerbated, sun protection is essential. Antimalarials such as hydroxychloroquine are considered first-line therapy. Clinicians must be aware of 2 possible hydroxychloroquine side effects that can uniquely confuse the clinical picture in ADM. The first is a rash, most often morbilliform and pruritic, that occurs in DM more frequently than in other diseases.³⁵ The second is a myopathy found in as many as 6.7% of patients using antimalarials for rheumatic disease,³⁶ which can clinically mimic the progression of ADM to CADM.³⁷ Two small retrospective case series found that methotrexate was beneficial in ADM.^38,39 Methotrexate also has been reported as an efficacious treatment of ILD in patients with connective tissue diseases.^40,41 Intravenous immunoglobulin and other immunosuppressants are additional agents to be considered.⁴²

In summary, ADM is an important subset of DM and is more likely to present to dermatology practices than to other specialists. Amyopathic DM shares cutaneous findings with DM, and both overlap and differ with respect to other key disease characteristics including autoantibody profile, associated lung disease, and malignancy risk. Palmoplantar keratoderma is a rarely reported skin finding in DM. We report a case of ADM with the unique finding of severe plantar keratoderma. The fact that our patient’s keratoderma and other skin findings resolved concomitantly during methotrexate therapy leads us to believe that the keratoderma was a unique skin manifestation of the ADM itself.

Case Report

A 54-year-old woman presented with a painful pruritic rash on the hands and feet of 7 years’ duration. She reported intermittent joint pain but denied muscle weakness. Physical examination revealed fissured fingertips and heavy scaling of the palms and lateral fingers (Figure 1). Violaceous scaly papules were seen on the distal and proximal interphalangeal joints (Figure 2). A severe plantar keratoderma also was noted (Figure 3). Pink scaly plaques were present on the bilateral elbows and postauricular skin. Diffuse mat telangiectases covered the malar skin. Extensive poikilodermatous skin changes covered approximately 20% of the total body surface area. Salt-and-pepper patches and papules were noted over the bilateral thighs. She reported an uncertain history of recent radiographs of one or both hands, which showed no joint degeneration characteristic of psoriatic arthritis. She previously had been given a diagnosis of psoriasis by an outside dermatologist but was not responding to topical therapy.

Several skin biopsies showed histologic evidence of dermatomyositis (DM)(Figure 4). Prominent basement thickening also was seen on periodic acid–Schiff staining (not shown). Laboratory workup showed negative antinuclear antibodies and anti–Jo-1, anti-Ku, and anti-Mi2 antibodies. Muscle enzymes including creatinine kinase and aldolase were within reference range. Pelvic ultrasonography and mammography were negative. Pulmonary function tests were unremarkable. High-resolution chest computed tomography (CT) was ordered because of a history of chronic cough; however, no evidence of malignancy or interstitial lung disease was seen. The patient was diagnosed with amyopathic dermatomyositis (ADM). Rheumatology was consulted and initiated oral hydroxychloroquine therapy. After 3 months, the patient’s cutaneous disease did not respond and she reported having headaches associated with this medication; therefore, methotrexate was started. Within 2 months of treatment, full resolution of the plantar keratoderma (Figure 5) and clearance of the scaling/fissuring of the hands as well as the psoriatic-appearing plaques on the elbows was noted.

Comment

Amyopathic DM is a subset of DM that accounts for 10% to 20% of DM cases.^1,2 Sontheimer’s³ diagnostic criteria for ADM require histopathologic confirmation of the hallmark skin findings of classic DM and lack of muscle weakness or muscle enzyme (creatine kinase/aldolase) elevation for at least 2 years.

Similar to classic DM, ADM typically presents in the fifth decade of life and has a female predilection.^1,4 The term hypomyopathic DM is used to describe patients who exhibit classic skin findings and evidence of muscle involvement on magnetic resonance imaging, electromyography, biopsy, or serum enzymes but have no clinical evidence of muscle weakness for at least 6 months. Together, hypomyopathic DM and ADM are referred to as clinically ADM (CADM). Patients who have met the criteria for hypomyopathic DM or ADM may later develop frank myopathy, progressing to a diagnosis of CADM, which may occur in as many as 10% to 13% of cases of CADM.^1,2 Clinical evidence of muscle weakness typically is heralded by elevation of creatine kinase and aldolase; therefore, patients with ADM should have muscle enzymes periodically checked.

Cutaneous findings of ADM are the same as the hallmark skin findings in CADM.³ Poikiloderma appears as thin telangiectatic skin in a background of mottled hyperpigmentation and hypopigmentation. It represents chronic inflammation and often occurs in sun-exposed areas. Poikiloderma located on the posterior neck and shoulders is known as the shawl sign and on the lateral thighs as the holster sign.⁵ The term mechanic’s hands is used to describe the clinical finding of palmar erythema with scaling and fissuring of the fingertips.⁶ Scalp findings include erythematous, atrophic, scaly plaques resembling psoriasis and nonscarring alopecia.⁷ Gottron papules are nearly pathognomonic for DM. These violaceous papules often are pruritic and found over the finger joints, in contrast to the hand rash of lupus erythematosus that involves the skin between finger joints.⁸ Psoriatic-appearing plaques overlying the elbows and knees are known as Gottron sign and can contribute to misdiagnosis as psoriasis.⁸ The classic heliotrope rash presents as a violaceous hue in the periorbital area and may be associated with periorbital edema.⁹ Calcinosis cutis is common in CADM but rarely is reported in ADM.¹⁰ Nail findings include periungual hyperemia, cuticular overgrowth, and nail bed changes due to avascular areas and dilated capillaries. The cutaneous histopathologic findings in ADM are the same as with CADM: a smudged dermoepidermal interface, vacuolar alterations of the basal layer, and dermal mucin deposits.

Palmoplantar keratoderma rarely is reported as a cutaneous finding in DM. The finding of keratoderma has mainly been reported in association with Wong-type DM, a rare subtype of DM with features of pityriasis rubra pilaris.^11-13 Palmoplantar keratoderma also has been reported in a case of an ADM-like hydroxyurea-induced eruption¹⁴ and as an early presenting feature in one patient with CADM and one with juvenile DM.^15,16

The autoantibody profile in patients with ADM varies from that of CADM and can be helpful in both diagnosis and prognosis. Similar to CADM, the majority of patients with ADM have positive antinuclear antibodies.^2,17 Anti–Jo-1 (an anti–aminoacyl-transfer RNA synthetase) antibody frequently is found in CADM but rarely in ADM.² Anti–Jo-1 is predictive of interstitial lung disease (ILD) in CADM. Positive anti–Jo-1 in combination with Raynaud phenomenon and mechanic’s hands is referred to as antisynthetase syndrome in patients with CADM.^18,19 An antibody uniquely linked with CADM is the anti–CADM-140/MDA5 antibody and can be a marker of rapidly progressing ILD in these patients.²⁰ Anti–Mi-2 is another myositis-specific antibody not commonly found in ADM but is present in 15% to 30% of DM cases.^2,21 In CADM, the anti–Mi-2 antibody is associated with the shawl sign, ragged cuticles, and carpal tunnel syndrome and has a favorable prognosis.^17,21 Myositis-associated autoantibodies (eg, anti-Ku) are found in patients with symptoms overlapping both DM and scleroderma or other connective tissue diseases.²² More recently described, the anti-p155/140 antibody is highly specific (up to 89%) for occult malignancy in DM.²³

Lung disease is an important association in ADM. When it develops, it may be more aggressive compared to lung disease associated with CADM.^24-26 In a systematic review of 197 cases of ADM by Gerami et al,² 10% of patients had ILD, and it was fatal in 42% of cases. Most cases of ILD associated with CADM were diagnosed as interstitial pneumonitis or diffuse alveolar disease; bronchiolitis obliterans organizing pneumonia and basilar fibrosis also were recorded.² Anti–Jo-1 antibodies often accompany lung disease in CADM but are not typically found in lung disease associated with ADM. The anti–CADM-140/MDA5 antibody is associated with an increased risk for rapidly progressing ILD in patients with CADM.²⁰ Recommended baseline screening for lung disease in DM includes chest radiography, pulmonary function tests with diffusion capacity,⁸ and in some instances high-resolution chest CT.²⁷ Follow-up visits should include screening for symptoms of ILD such as cough, shortness of breath, or dyspnea. Treatment of myopathy-associated ILD is systemic steroids combined with various immunosuppressants including cyclophosphamide, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, and intravenous immunoglobulin.^28,29

The risk of malignancy in ADM is thought to be similar to the rate of 20% to 25% found in CADM.^1,30-32 The most commonly reported malignancies associated with ADM are nasopharyngeal, breast, lung, ovarian, colorectal, pancreatic, and stomach cancers and lymphoma/leukemia.^2,33 Patients with ADM should be screened for malignancy at diagnosis, then yearly for 3 years.^8,31,33 In addition to history, physical examination, and age/sex-appropriate screening, a complete blood cell count, chemistry panel, urinalysis, stool guaiac, CA 125, CA 19-9, chest radiograph, and abdominal ultrasound should be performed. For women, mammography and pelvic ultrasonography should be completed.³¹ Some experts also recommend a full-body CT scan. Because Asian patients have a higher risk for nasopharyngeal carcinoma, referral to an ear, nose, and throat surgeon for direct visualization also can be considered.³³ The risk of cancer in patients with DM compared to the general population is increased for at least the first 5 years after diagnosis, but most associated cancers are found within the first 3 years.³⁴

Several therapies have been found useful in ADM. Because lesions often are photoexacerbated, sun protection is essential. Antimalarials such as hydroxychloroquine are considered first-line therapy. Clinicians must be aware of 2 possible hydroxychloroquine side effects that can uniquely confuse the clinical picture in ADM. The first is a rash, most often morbilliform and pruritic, that occurs in DM more frequently than in other diseases.³⁵ The second is a myopathy found in as many as 6.7% of patients using antimalarials for rheumatic disease,³⁶ which can clinically mimic the progression of ADM to CADM.³⁷ Two small retrospective case series found that methotrexate was beneficial in ADM.^38,39 Methotrexate also has been reported as an efficacious treatment of ILD in patients with connective tissue diseases.^40,41 Intravenous immunoglobulin and other immunosuppressants are additional agents to be considered.⁴²

In summary, ADM is an important subset of DM and is more likely to present to dermatology practices than to other specialists. Amyopathic DM shares cutaneous findings with DM, and both overlap and differ with respect to other key disease characteristics including autoantibody profile, associated lung disease, and malignancy risk. Palmoplantar keratoderma is a rarely reported skin finding in DM. We report a case of ADM with the unique finding of severe plantar keratoderma. The fact that our patient’s keratoderma and other skin findings resolved concomitantly during methotrexate therapy leads us to believe that the keratoderma was a unique skin manifestation of the ADM itself.