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Fig 2. A, Histopathology of vulvar and right thigh lesions. Skin biopsy specimens from both lesions show areas of necrosis surrounded by a prominent granulomatous reaction. B, Prominent histiocytes and multinucleated giant cells.
with a serum monoclonal gammopathy. Skin biopsy specimen demonstrates a dermal infiltrate composed of palisading granulomas with areas of necrosis and prominent giant cells. Most patients (80%) with NXG have a serum monoclonal gammopathy and a small percentage (10%) develop multiple myeloma.1 The vast majority of patients have periorbital lesions, particularly on the upper or lower eyelid, as in this case.2 Other sites of involvement include the face, trunk, and extremities.3,4 There are only 2 cases of NXG with mucosal or genital involvement reported in the literature. Fortson and Schroeter5 reported a case of NXG of the lips and tongue, and a review by Wood et al1 cited a 26-year-old man with penile NXG in a table summarizing the clinical findings in 12 patients with NXG. To our knowledge, this is the first case of NXG involving the female genitalia. Given the dramatic presentation and extent of genital involvement in this elderly female patient, the leading gynecologic diagnosis was squamous cell carcinoma. Biopsy specimen of the vulvar lesion was essential to rule out underlying malignancy and establish the diagnosis. The patient refused further workup and died 2 weeks after presentation from failure to thrive, although multiple myeloma cannot be ruled out as her underlying cause of death. This case demonstrates the potential for NXG to rapidly progress, leading to dramatic ulceration with significant morbidity and mortality and underscores the need for early recognition of this rare condition. Treatment options for NXG include surgery, intralesional and systemic corticosteroids, and other systemic therapies such as methotrexate, cyclophosphamide, and azathioprine. Vulvar NXG is now an entity that both dermatologists and gynecologists should be aware of, requiring prompt evaluation with biopsy and treatment to prevent potentially disfiguring and debilitating obliteration of the external female genitalia. Open access under CC BY-NC-ND license.
Ilana J. DeLuca, MD, PhD, and Marc E. Grossman, MD Department of Dermatology, Columbia University, New York, New York Funding sources: None. Conflicts of interest: None declared. Correspondence to: Ilana J. DeLuca, MD, PhD, Department of Dermatology, Columbia University, 161 Fort Washington Ave, 12th Floor, New York, NY 10032 E-mail: [email protected] REFERENCES 1. Wood AJ, Wagner VU, Abbott JJ, Gibson LE. Necrobiotic xanthogranuloma. Arch Dermatol 2009;145:279-84. 2. Spicknall KE, Mehregan DA. Necrobiotic xanthogranuloma. Int J Dermatol 2009;48:1-10. 3. Mehregan DA, Winkelmann RK. Necrobiotic xanthogranuloma. Arch Dermatol 1992;128:94-100. 4. Efebera Y, Blanchard E, Allam C, Han A, Lee A, Munshi N. Complete response to thalidomide and dexamethasone in a patient with necrobiotic xanthogranuloma associated with monoclonal gammopathy: a case report and review of the literature. Clin Lymphoma Myeloma Leuk 2011;11:298-302. 5. Fortson JS, Schroeter AL. Necrobiotic xanthogranuloma with IgA paraproteinemia and extracutaneous involvement. Am J Dermatopathol 1990;12:579-84. http://dx.doi.org/10.1016/j.jaad.2014.04.039
Pedicure-associated Mycobacterium chelonae infection in a hospitalized patient To the Editor: The Vanderbilt University Dermatology Consult Service was asked to evaluate a 60-year-old man with a history of nonischemic cardiomyopathy, congestive heart failure, and a 1-week history of painful swelling and ulcerations on his bilateral lower extremities. The patient had insulin-dependent
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Fig 1. Mycobacterial infection of the lower extremities: Eroded pink macules.
Fig 2. Mycobacterial infection of the lower extremities: Acid-fast bacillus test and staining on histology from right lower extremity.
diabetes, took numerous medications, and was awaiting a heart transplant. He had been administered vancomycin and piperacillin/tazobactam at an outside hospital without effect. At the time of his dermatology consultation, the skin of his lower legs exhibited diffuse and variable erythema with numerous punched out ulcerations and erythematous papules (Fig 1). Punch biopsy performed at the time of consultation demonstrated suppurative dermal inflammation with positive results for mycobacteria on acid-fast bacillus test (Fig 2). Upon further investigation, the patient’s family disclosed that the patient regularly went for pedicures at a local nail salon, with his last visit having occurred 1 month before admission. A second biopsy was performed and sent for culture. Within 5 days this tissue grew Mycobacterium chelonae and he was discharged from the hospital with prescriptions for azathioprine, doxycycline, and ciprofloxacin for his skin infection. Because he would require prolonged antibiotic administration to sufficiently clear the infection, he was removed from the transplantation list. One month later the patient was readmitted for worsening heart failure. In the interim he had taken his antibiotics with slow, steady improvement in his mycobacterial cellulitis. Even so, he died of heart
failure before he could be put back on the heart transplant list. The occurrence of rapidly growing mycobacterial infections after nail salon pedicures has been well documented in the literature. Winthrop et al1 reported the first epidemiologic investigation of a large outbreak of mycobacterial furunculosis due to a single strain of Mycobacterium fortuitum among otherwise healthy people who had received pedicures using whirlpool footbaths at a nail salon in California. There have since been several case reports of lower extremity soft tissue infections in predominantly immunocompetent individuals due to rapidly growing mycobacteria (RGM).2-5 Skin microtrauma due to shaving has been identified as a risk factor for disease.1,4 Our case is unique because the patient may have died because the prolonged treatment course necessary to clear his mycobacterial infection precluded heart transplantation. Treatment regimens for RGM vary depending on the isolate of the Mycobacterium, how localized the infection is, and the immune status of the patient. In immunocompetent individuals, treatment of RGM infections requires an average of 4 months with at least 2 antibiotics.4 More extensive or severe disease requires the initial use of parenteral antibiotics and 6 to 12 months of treatment.5 As was the case with our patient, diagnosis may be delayed until skin biopsies are performed.4 Furthermore, swab cultures may be negative and RGM take 3 to 7 days to grow on blood agar. Our case highlights the challenges associated with treating atypical mycobacterial infections. We hope to emphasize the importance of educating immunocompromised patients to avoid pedicure salons especially when open wounds or skin trauma is involved. Adriana N. Schmidt, MD,a John A. Zic, MD,b and Alan S. Boyd, MDb Santa Monica Dermatology Medical Group,a California, and Division of Dermatology, Vanderbilt University Medical Center,b Nashville, Tennessee Funding sources: None. Conflicts of interest: None declared. Correspondence to: Adriana N. Schmidt, MD, 2001 Santa Monica Boulevard Suite 990W, Santa Monica, CA 90404 E-mail: [email protected] REFERENCES 1. Winthrop KL, Abrams M, Yakrus M, Schwartz I, Ely J, Gillies D, et al. An outbreak of mycobacterial furunculosis associated with footbaths at a nail salon. N Engl J Med 2002;346:1366-71.
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2. Redboard KP, Shearer DA, Gloster H, Younger B, Connelly BL, Kindel SE, et al. Atypical Mycobacterium furunculosis occurring after pedicures. J Am Acad Dermatol 2006;54:520-4. 3. Stout JE, Gadkowski B, Rath S, Alspaugh JA, Miller MB, Cox GM. Pedicure-associated rapidly growing mycobacterial infection: an endemic disease. Clin Infect Dis 2011;53:787-92. 4. Sniezek PJ, Graham BS, Busch HB, Lederman ER, Lim ML, Poggemyer K, et al. Rapidly growing mycobacterial infections after pedicures. Arch Dermatol 2003;139:629-34. 5. Winthrop KL, Albridge K, South D, Albrecht P, Abrams M, Samuel MC, et al. The clinical management and outcome of nail salon-acquired Mycobacterium skin infection. Clin Infect Dis 2004;38:38-44. http://dx.doi.org/10.1016/j.jaad.2014.08.012
Facial erythromelalgia: A rare entity to consider in the differential diagnosis of connective tissue diseases
Fig 1. Facial erythromelalgia mimicking connective tissue disease. Biopsy showed vacuolar interface alteration (arrows), perivascular lymphocytic inflammation, and marked vascular congestion (right). Mucin deposition was also evident in the mid to deep dermis (not shown). (Hematoxylin and eosin; original magnification 3400.)
To the Editor: A 53-year-old man presented to an outside dermatologist with an erythematous facial eruption of 4 years’ duration. The eruption was associated with burning and swelling, which the patient reported worsened with sun exposure. These symptoms were debilitating, preventing the patient from working and maintaining daily activities. Initially, the eruption was thought to be rosacea and actinic damage; however, it was unresponsive to standard therapies. Given the refractory nature of the facial erythema and reported photosensitivity, a diagnosis of connective tissue disease (CTD) was considered. Antinuclear, anti-Ro/SSA, and anti-La/SSB antibodies were negative; however, skin biopsy revealed vacuolar interface alteration with perivascular and periadnexal lymphoid infiltration and mucin deposition (Fig 1). A diagnosis of CTD was made, and treatment with hydroxychloroquine and systemic corticosteroids was initiated. However, the patient had minimal clinical response, resulting in subsequent referral to our CTD clinic for management. On our initial examination, the patient had prominent full facial and auricular erythema and edema, as well as erosions of the bilateral helices and a violaceous appearance of his earlobes (Fig 2, A). Despite the biopsy findings, clinical exam did not support a diagnosis of CTD. Instead, facial erythromelalgia was suspected. Upon further questioning, the patient reported skin burning not only with sun exposure, but also with heat exposure. Furthermore, he reported attempting cooling measures, such as the frequent use of ice packs, to relieve his symptoms. With these additional details, a clinical diagnosis of facial and auricular erythromelalgia was established. Further workup excluded potential causes of secondary erythromelalgia. Given that
erythromelalgia is thought to involve both small fiber neuropathy as well as vasculopathy, we initiated therapy with aspirin, pentoxifylline, and gabapentin.1 Subsequently, nifedipine was added to this treatment regimen. At 5 months’ follow-up, the patient reported 80% improvement (Fig 2, B) and was able to return to work and enjoy normal daily activities. Erythromelalgia is a rare condition characterized by episodic erythema, swelling, warmth, and burning, which can become persistent over time. Although it primarily occurs on the extremities, it may also manifest on the ears and face. Heat exposure triggers episodes, and cooling can alleviate symptoms.2 Erythromelalgia can cause significant morbidity and mortality, with suicide being a notable cause of death, making timely recognition and initiation of therapy essential.3 There is no diagnostic test for erythromelalgia; therefore, a high level of clinical suspicion, along with a detailed history and physical exam, is required to establish the diagnosis. Although biopsy findings are often nonspecific, there has been 1 reported case of histopathologic findings of CTD in a patient with erythromelalgia involving the hands.4 This case highlights a rare condition, which can be mistaken for CTD, particularly when the patient presents with facial erythema, reports photosensitivity, or has skin biopsy findings that support CTD. To our knowledge, we report the first case of facial erythromelalgia with biopsy findings demonstrating vacuolar interface dermatitis and mucin deposition. This case underscores the need for physicians who follow patients with CTD to be aware of the clinical presentation of facial erythromelalgia.
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DECEMBER 2014
Fig 2. A, Histopathology of vulvar and right thigh lesions. Skin biopsy specimens from both lesions show areas of necrosis surrounded by a prominent granulomatous reaction. B, Prominent histiocytes and multinucleated giant cells.
with a serum monoclonal gammopathy. Skin biopsy specimen demonstrates a dermal infiltrate composed of palisading granulomas with areas of necrosis and prominent giant cells. Most patients (80%) with NXG have a serum monoclonal gammopathy and a small percentage (10%) develop multiple myeloma.1 The vast majority of patients have periorbital lesions, particularly on the upper or lower eyelid, as in this case.2 Other sites of involvement include the face, trunk, and extremities.3,4 There are only 2 cases of NXG with mucosal or genital involvement reported in the literature. Fortson and Schroeter5 reported a case of NXG of the lips and tongue, and a review by Wood et al1 cited a 26-year-old man with penile NXG in a table summarizing the clinical findings in 12 patients with NXG. To our knowledge, this is the first case of NXG involving the female genitalia. Given the dramatic presentation and extent of genital involvement in this elderly female patient, the leading gynecologic diagnosis was squamous cell carcinoma. Biopsy specimen of the vulvar lesion was essential to rule out underlying malignancy and establish the diagnosis. The patient refused further workup and died 2 weeks after presentation from failure to thrive, although multiple myeloma cannot be ruled out as her underlying cause of death. This case demonstrates the potential for NXG to rapidly progress, leading to dramatic ulceration with significant morbidity and mortality and underscores the need for early recognition of this rare condition. Treatment options for NXG include surgery, intralesional and systemic corticosteroids, and other systemic therapies such as methotrexate, cyclophosphamide, and azathioprine. Vulvar NXG is now an entity that both dermatologists and gynecologists should be aware of, requiring prompt evaluation with biopsy and treatment to prevent potentially disfiguring and debilitating obliteration of the external female genitalia. Open access under CC BY-NC-ND license.
Ilana J. DeLuca, MD, PhD, and Marc E. Grossman, MD Department of Dermatology, Columbia University, New York, New York Funding sources: None. Conflicts of interest: None declared. Correspondence to: Ilana J. DeLuca, MD, PhD, Department of Dermatology, Columbia University, 161 Fort Washington Ave, 12th Floor, New York, NY 10032 E-mail: [email protected] REFERENCES 1. Wood AJ, Wagner VU, Abbott JJ, Gibson LE. Necrobiotic xanthogranuloma. Arch Dermatol 2009;145:279-84. 2. Spicknall KE, Mehregan DA. Necrobiotic xanthogranuloma. Int J Dermatol 2009;48:1-10. 3. Mehregan DA, Winkelmann RK. Necrobiotic xanthogranuloma. Arch Dermatol 1992;128:94-100. 4. Efebera Y, Blanchard E, Allam C, Han A, Lee A, Munshi N. Complete response to thalidomide and dexamethasone in a patient with necrobiotic xanthogranuloma associated with monoclonal gammopathy: a case report and review of the literature. Clin Lymphoma Myeloma Leuk 2011;11:298-302. 5. Fortson JS, Schroeter AL. Necrobiotic xanthogranuloma with IgA paraproteinemia and extracutaneous involvement. Am J Dermatopathol 1990;12:579-84. http://dx.doi.org/10.1016/j.jaad.2014.04.039
Pedicure-associated Mycobacterium chelonae infection in a hospitalized patient To the Editor: The Vanderbilt University Dermatology Consult Service was asked to evaluate a 60-year-old man with a history of nonischemic cardiomyopathy, congestive heart failure, and a 1-week history of painful swelling and ulcerations on his bilateral lower extremities. The patient had insulin-dependent
J AM ACAD DERMATOL
Letters e249
VOLUME 71, NUMBER 6
Fig 1. Mycobacterial infection of the lower extremities: Eroded pink macules.
Fig 2. Mycobacterial infection of the lower extremities: Acid-fast bacillus test and staining on histology from right lower extremity.
diabetes, took numerous medications, and was awaiting a heart transplant. He had been administered vancomycin and piperacillin/tazobactam at an outside hospital without effect. At the time of his dermatology consultation, the skin of his lower legs exhibited diffuse and variable erythema with numerous punched out ulcerations and erythematous papules (Fig 1). Punch biopsy performed at the time of consultation demonstrated suppurative dermal inflammation with positive results for mycobacteria on acid-fast bacillus test (Fig 2). Upon further investigation, the patient’s family disclosed that the patient regularly went for pedicures at a local nail salon, with his last visit having occurred 1 month before admission. A second biopsy was performed and sent for culture. Within 5 days this tissue grew Mycobacterium chelonae and he was discharged from the hospital with prescriptions for azathioprine, doxycycline, and ciprofloxacin for his skin infection. Because he would require prolonged antibiotic administration to sufficiently clear the infection, he was removed from the transplantation list. One month later the patient was readmitted for worsening heart failure. In the interim he had taken his antibiotics with slow, steady improvement in his mycobacterial cellulitis. Even so, he died of heart
failure before he could be put back on the heart transplant list. The occurrence of rapidly growing mycobacterial infections after nail salon pedicures has been well documented in the literature. Winthrop et al1 reported the first epidemiologic investigation of a large outbreak of mycobacterial furunculosis due to a single strain of Mycobacterium fortuitum among otherwise healthy people who had received pedicures using whirlpool footbaths at a nail salon in California. There have since been several case reports of lower extremity soft tissue infections in predominantly immunocompetent individuals due to rapidly growing mycobacteria (RGM).2-5 Skin microtrauma due to shaving has been identified as a risk factor for disease.1,4 Our case is unique because the patient may have died because the prolonged treatment course necessary to clear his mycobacterial infection precluded heart transplantation. Treatment regimens for RGM vary depending on the isolate of the Mycobacterium, how localized the infection is, and the immune status of the patient. In immunocompetent individuals, treatment of RGM infections requires an average of 4 months with at least 2 antibiotics.4 More extensive or severe disease requires the initial use of parenteral antibiotics and 6 to 12 months of treatment.5 As was the case with our patient, diagnosis may be delayed until skin biopsies are performed.4 Furthermore, swab cultures may be negative and RGM take 3 to 7 days to grow on blood agar. Our case highlights the challenges associated with treating atypical mycobacterial infections. We hope to emphasize the importance of educating immunocompromised patients to avoid pedicure salons especially when open wounds or skin trauma is involved. Adriana N. Schmidt, MD,a John A. Zic, MD,b and Alan S. Boyd, MDb Santa Monica Dermatology Medical Group,a California, and Division of Dermatology, Vanderbilt University Medical Center,b Nashville, Tennessee Funding sources: None. Conflicts of interest: None declared. Correspondence to: Adriana N. Schmidt, MD, 2001 Santa Monica Boulevard Suite 990W, Santa Monica, CA 90404 E-mail: [email protected] REFERENCES 1. Winthrop KL, Abrams M, Yakrus M, Schwartz I, Ely J, Gillies D, et al. An outbreak of mycobacterial furunculosis associated with footbaths at a nail salon. N Engl J Med 2002;346:1366-71.
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2. Redboard KP, Shearer DA, Gloster H, Younger B, Connelly BL, Kindel SE, et al. Atypical Mycobacterium furunculosis occurring after pedicures. J Am Acad Dermatol 2006;54:520-4. 3. Stout JE, Gadkowski B, Rath S, Alspaugh JA, Miller MB, Cox GM. Pedicure-associated rapidly growing mycobacterial infection: an endemic disease. Clin Infect Dis 2011;53:787-92. 4. Sniezek PJ, Graham BS, Busch HB, Lederman ER, Lim ML, Poggemyer K, et al. Rapidly growing mycobacterial infections after pedicures. Arch Dermatol 2003;139:629-34. 5. Winthrop KL, Albridge K, South D, Albrecht P, Abrams M, Samuel MC, et al. The clinical management and outcome of nail salon-acquired Mycobacterium skin infection. Clin Infect Dis 2004;38:38-44. http://dx.doi.org/10.1016/j.jaad.2014.08.012
Facial erythromelalgia: A rare entity to consider in the differential diagnosis of connective tissue diseases
Fig 1. Facial erythromelalgia mimicking connective tissue disease. Biopsy showed vacuolar interface alteration (arrows), perivascular lymphocytic inflammation, and marked vascular congestion (right). Mucin deposition was also evident in the mid to deep dermis (not shown). (Hematoxylin and eosin; original magnification 3400.)
To the Editor: A 53-year-old man presented to an outside dermatologist with an erythematous facial eruption of 4 years’ duration. The eruption was associated with burning and swelling, which the patient reported worsened with sun exposure. These symptoms were debilitating, preventing the patient from working and maintaining daily activities. Initially, the eruption was thought to be rosacea and actinic damage; however, it was unresponsive to standard therapies. Given the refractory nature of the facial erythema and reported photosensitivity, a diagnosis of connective tissue disease (CTD) was considered. Antinuclear, anti-Ro/SSA, and anti-La/SSB antibodies were negative; however, skin biopsy revealed vacuolar interface alteration with perivascular and periadnexal lymphoid infiltration and mucin deposition (Fig 1). A diagnosis of CTD was made, and treatment with hydroxychloroquine and systemic corticosteroids was initiated. However, the patient had minimal clinical response, resulting in subsequent referral to our CTD clinic for management. On our initial examination, the patient had prominent full facial and auricular erythema and edema, as well as erosions of the bilateral helices and a violaceous appearance of his earlobes (Fig 2, A). Despite the biopsy findings, clinical exam did not support a diagnosis of CTD. Instead, facial erythromelalgia was suspected. Upon further questioning, the patient reported skin burning not only with sun exposure, but also with heat exposure. Furthermore, he reported attempting cooling measures, such as the frequent use of ice packs, to relieve his symptoms. With these additional details, a clinical diagnosis of facial and auricular erythromelalgia was established. Further workup excluded potential causes of secondary erythromelalgia. Given that
erythromelalgia is thought to involve both small fiber neuropathy as well as vasculopathy, we initiated therapy with aspirin, pentoxifylline, and gabapentin.1 Subsequently, nifedipine was added to this treatment regimen. At 5 months’ follow-up, the patient reported 80% improvement (Fig 2, B) and was able to return to work and enjoy normal daily activities. Erythromelalgia is a rare condition characterized by episodic erythema, swelling, warmth, and burning, which can become persistent over time. Although it primarily occurs on the extremities, it may also manifest on the ears and face. Heat exposure triggers episodes, and cooling can alleviate symptoms.2 Erythromelalgia can cause significant morbidity and mortality, with suicide being a notable cause of death, making timely recognition and initiation of therapy essential.3 There is no diagnostic test for erythromelalgia; therefore, a high level of clinical suspicion, along with a detailed history and physical exam, is required to establish the diagnosis. Although biopsy findings are often nonspecific, there has been 1 reported case of histopathologic findings of CTD in a patient with erythromelalgia involving the hands.4 This case highlights a rare condition, which can be mistaken for CTD, particularly when the patient presents with facial erythema, reports photosensitivity, or has skin biopsy findings that support CTD. To our knowledge, we report the first case of facial erythromelalgia with biopsy findings demonstrating vacuolar interface dermatitis and mucin deposition. This case underscores the need for physicians who follow patients with CTD to be aware of the clinical presentation of facial erythromelalgia.
