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Abrupt Onset of Ulcerative Papules and Nodules on the Face and Genitals

Abrupt Onset of Ulcerative Papules and Nodules on the Face and Genitals Case A man in his 60s with a medical history of hypertension, cerebrovascular accident, colon cancer, and remote cocaine use presented with numerous pruritic crusted papules and nodules on the face and genitals. The eruption presented abruptly over a 2-week course, and the patient attributed it to contact with plants while working in his yard. He denied having similar lesions in the past. He had used over-the-counter topical antibiotic and antihistamine creams, which alleviated the pruritus. He denied recent sexual intercourse, penile discharge, fevers, chills, nausea, or vomiting. Physical examination of the bilateral cheeks, glabella, dorsal nose, and chin revealed multiple skin-colored papules, nodules, and vegetative plaques ranging in size from 4 to 6 mm in diameter, with central umbilication, erosions, and overlying yellow crust (Figure, A). There were similar lesions on the scrotum and inner thighs, with sparing of the penis (Figure, B). There was also a 1-cm ulceration on the left lower lip. The palms and soles were spared. There was no lymphadenopathy noted. One 4-mm punch biopsy specimen was obtained from a nodule on the left medial cheek, bisectioned, and sent for both hematoxylin–eosin staining and tissue cultures for bacteria, fungi, and atypical mycobacteria (Figure, C and D). Figure. View LargeDownload A, Multiple crusted and ulcerative papules and nodules on the face. B, Multiple ulcerated healing papules on the scrotum and umbilicated papules on the left inner thigh. C, Low-power image (hematoxylin-eosin, original magnification ×40). D, A Warthin-Starry silver impregnation stain (original magnification ×40). Box Section Ref ID What Is Your Diagnosis? Atypical mycobacterium infection Cryptococcal infection Eruptive keratoacanthomas Secondary syphilis Read the Discussion Discussion Diagnosis D. Secondary syphilis Microscopic Findings and Clinical Course The biopsy specimen showed a subacute dermatitis with reactive epithelial hyperplasia, abundant plasma cells, and lymphocytes (Figure, C). Warthin-Starry silver impregnation stain highlighted numerous spirochetes (Figure, D). Tissue culture failed to reveal any organisms. Rapid plasma reagin titer was 1:64, and fluorescent treponemal antibody absorption test results were positive. Results of complete blood count and comprehensive metabolic panel were within normal limits. Laboratory testing for human immunodeficiency virus (HIV) yielded negative results. Ophthalmologic evaluation revealed no gross abnormalities. Cerebrospinal fluid (CSF) obtained from lumbar puncture revealed 7 white blood cells/cm2 with normal protein levels and no evidence of organisms. Although the CSF findings of 7 white blood cells/cm2 were likely secondary to trauma from the lumbar puncture, the patient completed a 14-day course of intravenous (IV) penicillin G, 3 million units every 4 hours, out of concern for tertiary syphilis. Following the IV penicillin, the patient also continued 3 million units of intramuscular penicillin weekly for 3 additional weeks. At his 2 week follow-up after IV penicillin treatment, his lesions had completely resolved, with only areas of residual postinflammatory hyperpigmentation. Syphilis is a sexually transmitted infection caused by the bacteria Treponema pallidum. Despite decreases in the incidence of syphilis in the 1990s, there has been a recent resurgence of cases within the past decade.1 The disease is divided into stages including primary, secondary, early latent, late latent, and tertiary. Primary syphilis presents as a painless chancre usually on a mucous membrane at the site of transmission 10 to 30 days after sexual contact. Four to 8 weeks after spontaneous resolution of the primary lesion, high levels of T pallidum result in a manifestation of the disease known as secondary syphilis, which can have a variety of cutaneous presentations. Psoriasiform or lichenoid papules and plaques can occur almost anywhere on the body, often favoring the acral surfaces. Additional cutaneous presentations include annular plaques on the face and granulomatous nodules and plaques known as condyloma lata lesions usually presenting in intertriginous areas and oral mucosa. The scalp can also be affected by a “moth-eaten” alopecia. Tertiary syphilis is classically characterized by gumma that appear as annular nodules that can ulcerate and are typically spirochete-poor.2 This particular case of a sudden, dramatic eruption of crusted and eroded nodules concentrated on the face shares some features with lues maligna, a rare presentation of secondary syphilis characterized by a sudden onset of papules or pustules that evolve into circumscribed necrotic ulcers.1,3 Neisser4 reported 5 clinical distinctions of lues maligna including short incubation period; pronounced constitutional symptoms; involvement of mucous membranes around the nose and mouth; and polymorphous lesions including papules, pustules, and nodules. Rapid plasma reagin testing results may be falsely negative during this time owing to a prozone effect secondary to an overwhelmingly high antibody titer.3 Differential diagnosis includes tertiary syphilis, cutaneous lymphoma, pyoderma gangrenosum, leishmaniasis, deep fungal and/or atypical mycobacterial infection, and pityriasis lichenoides. Although the clinicopathologic variability of syphilis can make the diagnosis challenging, a histological appearance of a psoriasiform and/or lichenoid dermatitis with numerous plasma cells should raise suspicion for syphilis. While standard treatment for secondary syphilis is a single intramuscular injection of 2.4 million units of benzathine penicillin G,1 lues maligna is often treated similarly to late latent syphilis–with 3 doses of penicillin 1 week apart.5,6 Owing to the high serum levels of T pallidum associated with lues maligna, neurosyphilis should be ruled out by either clinical evaluation and/or lumbar puncture. Additional follow-up should include partner notification, HIV testing, and clinical and serologic follow up at 6 and 12 months.7 This case not only highlights an unusual polymorphous eruption of secondary syphilis, but also describes it in an immunocompetent patient and serves as a reminder to always consider syphilis when faced with a polymorphous cutaneous eruption. Back to top Article Information Corresponding Author: Lauren Chantel Payne, MD, MS, BS, Department of Dermatology, Howard University Hospital, 2041 Georgia Ave, Suite 2107, Washington, DC 20060 (lpayne@gmail.com). Published Online: March 2, 2016. doi:10.1001/jamadermatol.2016.0017. Conflict of Interest Disclosures: None reported. Additional Contributions: We would like to thank the patient for granting permission to publish this information. We are also indebted to Wen Chen, MD, MS, for the histopathologic evaluation of the case. She was not compensated for her contributions. Self-assessment Credit: This article is eligible for journal-based self-assessment (1 credit) for Maintenance of Certification (MOC) from the American Board of Dermatology (ABD). After completion of an activity, please log on to the ABD website at www.abderm.org to register your credits. This may be done after each exercise or after accumulating many credits. References 1. Patton ME, Su JR, Nelson R, Weinstock H; Centers for Disease Control and Prevention (CDC). Primary and secondary syphilis—United States, 2005-2013. MMWR Morb Mortal Wkly Rep. 2014;63(18):402-406.PubMedGoogle Scholar 2. Kazlouskaya V, Wittmann C, Tsikhanouskaya I. Pustular secondary syphilis: report of three cases and review of the literature. Int J Dermatol. 2014;53(10):e428-e431.PubMedGoogle ScholarCrossref 3. Fisher DA, Chang LW, Tuffanelli DL. Lues maligna. Presentation of a case and a review of the literature. Arch Dermatol. 1969;99(1):70-73.PubMedGoogle ScholarCrossref 4. Neisser A. Malignant Syphilis. British Journal of Dermatology. 1897; 9:11-26.Google Scholar 5. Kumar B, Muralidhar S. Malignant syphilis: a review. AIDS Patient Care STDS. 1998;12(12):921-925.PubMedGoogle ScholarCrossref 6. Alves J, António AM, Matos D, Coelho R, Cachão P. Malignant lues in an immunocompetent patient. Int J STD AIDS. 2015;26(7):518-520.PubMedGoogle ScholarCrossref 7. Ho EL, Spudich SS. Neurosyphilis and the impact of HIV infection [published online April 20, 2015]. Sex Health. 2015. doi:10.1071/SH14195.PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Dermatology American Medical Association

Abrupt Onset of Ulcerative Papules and Nodules on the Face and Genitals

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Publisher
American Medical Association
Copyright
Copyright © 2016 American Medical Association. All Rights Reserved.
ISSN
2168-6068
eISSN
2168-6084
DOI
10.1001/jamadermatol.2016.0017
pmid
26933833
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Abstract

Case A man in his 60s with a medical history of hypertension, cerebrovascular accident, colon cancer, and remote cocaine use presented with numerous pruritic crusted papules and nodules on the face and genitals. The eruption presented abruptly over a 2-week course, and the patient attributed it to contact with plants while working in his yard. He denied having similar lesions in the past. He had used over-the-counter topical antibiotic and antihistamine creams, which alleviated the pruritus. He denied recent sexual intercourse, penile discharge, fevers, chills, nausea, or vomiting. Physical examination of the bilateral cheeks, glabella, dorsal nose, and chin revealed multiple skin-colored papules, nodules, and vegetative plaques ranging in size from 4 to 6 mm in diameter, with central umbilication, erosions, and overlying yellow crust (Figure, A). There were similar lesions on the scrotum and inner thighs, with sparing of the penis (Figure, B). There was also a 1-cm ulceration on the left lower lip. The palms and soles were spared. There was no lymphadenopathy noted. One 4-mm punch biopsy specimen was obtained from a nodule on the left medial cheek, bisectioned, and sent for both hematoxylin–eosin staining and tissue cultures for bacteria, fungi, and atypical mycobacteria (Figure, C and D). Figure. View LargeDownload A, Multiple crusted and ulcerative papules and nodules on the face. B, Multiple ulcerated healing papules on the scrotum and umbilicated papules on the left inner thigh. C, Low-power image (hematoxylin-eosin, original magnification ×40). D, A Warthin-Starry silver impregnation stain (original magnification ×40). Box Section Ref ID What Is Your Diagnosis? Atypical mycobacterium infection Cryptococcal infection Eruptive keratoacanthomas Secondary syphilis Read the Discussion Discussion Diagnosis D. Secondary syphilis Microscopic Findings and Clinical Course The biopsy specimen showed a subacute dermatitis with reactive epithelial hyperplasia, abundant plasma cells, and lymphocytes (Figure, C). Warthin-Starry silver impregnation stain highlighted numerous spirochetes (Figure, D). Tissue culture failed to reveal any organisms. Rapid plasma reagin titer was 1:64, and fluorescent treponemal antibody absorption test results were positive. Results of complete blood count and comprehensive metabolic panel were within normal limits. Laboratory testing for human immunodeficiency virus (HIV) yielded negative results. Ophthalmologic evaluation revealed no gross abnormalities. Cerebrospinal fluid (CSF) obtained from lumbar puncture revealed 7 white blood cells/cm2 with normal protein levels and no evidence of organisms. Although the CSF findings of 7 white blood cells/cm2 were likely secondary to trauma from the lumbar puncture, the patient completed a 14-day course of intravenous (IV) penicillin G, 3 million units every 4 hours, out of concern for tertiary syphilis. Following the IV penicillin, the patient also continued 3 million units of intramuscular penicillin weekly for 3 additional weeks. At his 2 week follow-up after IV penicillin treatment, his lesions had completely resolved, with only areas of residual postinflammatory hyperpigmentation. Syphilis is a sexually transmitted infection caused by the bacteria Treponema pallidum. Despite decreases in the incidence of syphilis in the 1990s, there has been a recent resurgence of cases within the past decade.1 The disease is divided into stages including primary, secondary, early latent, late latent, and tertiary. Primary syphilis presents as a painless chancre usually on a mucous membrane at the site of transmission 10 to 30 days after sexual contact. Four to 8 weeks after spontaneous resolution of the primary lesion, high levels of T pallidum result in a manifestation of the disease known as secondary syphilis, which can have a variety of cutaneous presentations. Psoriasiform or lichenoid papules and plaques can occur almost anywhere on the body, often favoring the acral surfaces. Additional cutaneous presentations include annular plaques on the face and granulomatous nodules and plaques known as condyloma lata lesions usually presenting in intertriginous areas and oral mucosa. The scalp can also be affected by a “moth-eaten” alopecia. Tertiary syphilis is classically characterized by gumma that appear as annular nodules that can ulcerate and are typically spirochete-poor.2 This particular case of a sudden, dramatic eruption of crusted and eroded nodules concentrated on the face shares some features with lues maligna, a rare presentation of secondary syphilis characterized by a sudden onset of papules or pustules that evolve into circumscribed necrotic ulcers.1,3 Neisser4 reported 5 clinical distinctions of lues maligna including short incubation period; pronounced constitutional symptoms; involvement of mucous membranes around the nose and mouth; and polymorphous lesions including papules, pustules, and nodules. Rapid plasma reagin testing results may be falsely negative during this time owing to a prozone effect secondary to an overwhelmingly high antibody titer.3 Differential diagnosis includes tertiary syphilis, cutaneous lymphoma, pyoderma gangrenosum, leishmaniasis, deep fungal and/or atypical mycobacterial infection, and pityriasis lichenoides. Although the clinicopathologic variability of syphilis can make the diagnosis challenging, a histological appearance of a psoriasiform and/or lichenoid dermatitis with numerous plasma cells should raise suspicion for syphilis. While standard treatment for secondary syphilis is a single intramuscular injection of 2.4 million units of benzathine penicillin G,1 lues maligna is often treated similarly to late latent syphilis–with 3 doses of penicillin 1 week apart.5,6 Owing to the high serum levels of T pallidum associated with lues maligna, neurosyphilis should be ruled out by either clinical evaluation and/or lumbar puncture. Additional follow-up should include partner notification, HIV testing, and clinical and serologic follow up at 6 and 12 months.7 This case not only highlights an unusual polymorphous eruption of secondary syphilis, but also describes it in an immunocompetent patient and serves as a reminder to always consider syphilis when faced with a polymorphous cutaneous eruption. Back to top Article Information Corresponding Author: Lauren Chantel Payne, MD, MS, BS, Department of Dermatology, Howard University Hospital, 2041 Georgia Ave, Suite 2107, Washington, DC 20060 (lpayne@gmail.com). Published Online: March 2, 2016. doi:10.1001/jamadermatol.2016.0017. Conflict of Interest Disclosures: None reported. Additional Contributions: We would like to thank the patient for granting permission to publish this information. We are also indebted to Wen Chen, MD, MS, for the histopathologic evaluation of the case. She was not compensated for her contributions. Self-assessment Credit: This article is eligible for journal-based self-assessment (1 credit) for Maintenance of Certification (MOC) from the American Board of Dermatology (ABD). After completion of an activity, please log on to the ABD website at www.abderm.org to register your credits. This may be done after each exercise or after accumulating many credits. References 1. Patton ME, Su JR, Nelson R, Weinstock H; Centers for Disease Control and Prevention (CDC). Primary and secondary syphilis—United States, 2005-2013. MMWR Morb Mortal Wkly Rep. 2014;63(18):402-406.PubMedGoogle Scholar 2. Kazlouskaya V, Wittmann C, Tsikhanouskaya I. Pustular secondary syphilis: report of three cases and review of the literature. Int J Dermatol. 2014;53(10):e428-e431.PubMedGoogle ScholarCrossref 3. Fisher DA, Chang LW, Tuffanelli DL. Lues maligna. Presentation of a case and a review of the literature. Arch Dermatol. 1969;99(1):70-73.PubMedGoogle ScholarCrossref 4. Neisser A. Malignant Syphilis. British Journal of Dermatology. 1897; 9:11-26.Google Scholar 5. Kumar B, Muralidhar S. Malignant syphilis: a review. AIDS Patient Care STDS. 1998;12(12):921-925.PubMedGoogle ScholarCrossref 6. Alves J, António AM, Matos D, Coelho R, Cachão P. Malignant lues in an immunocompetent patient. Int J STD AIDS. 2015;26(7):518-520.PubMedGoogle ScholarCrossref 7. Ho EL, Spudich SS. Neurosyphilis and the impact of HIV infection [published online April 20, 2015]. Sex Health. 2015. doi:10.1071/SH14195.PubMedGoogle Scholar

Journal

JAMA DermatologyAmerican Medical Association

Published: Jul 1, 2016

References