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Sandpapery Skin

Sandpapery Skin Case A woman in her 70s with a medical history that was significant for polycythemia vera and acute myelogenous leukemia was seen for evaluation of textural skin changes on her face that had been present for 2½ years. She denied having pruritus or pain, but her skin had a rough sandpapery sensation. She reported that the involvement extended to her neck, the upper part of her chest, and her bilateral forearms. She associated the onset of the textural changes with her diagnosis of polycythemia vera. She stated that it began before her diagnosis of acute myelogenous leukemia and the initiation of treatment with hydroxyurea 4 years later. She had previously received treatment with long courses of triamcinolone acetonide cream, 0.1%, bacitracin ointment, and a combination antibiotic ointment of neomycin sulfate, polymyxin B, and bacitracin, with no improvement of her skin’s condition. Her additional daily medication regimen included donepezil hydrochloride, melatonin, and aspirin, 81 mg. Findings from the patient’s physical examination revealed background erythema with numerous rough horny spicules at the follicular ostia on her face, ears, and neck; the upper part of her chest; and her bilateral forearms. Findings from the rest of her examination revealed a pigmented macule on her right cheek. Two punch biopsy specimens were obtained from her right cheek and right postauricular area and submitted for histopathologic examination (Figure). Figure. View LargeDownload Histopathologic images (hematoxylin-eosin) of the punch biopsy specimen from the right side of the lateral neck. Box Section Ref ID What Is The Diagnosis? Trichodysplasia spinulosa–associated polyomavirus Demodex folliculorum–associated spinulosus Ulerythema ophryogenes Hyperkeratotic spicules of multiple myeloma Read the Discussion. Discussion Diagnosis B. Demodex folliculorum–associated spinulosus Microscopic Findings and Clinical Course Findings from histopathologic examination revealed numerous Demodex folliculorum mites in the follicular infundibula with associated hyperkeratosis, focal lichenoid lymphocytic infiltrates, and scattered Civatte bodies (Figure). The patient was prescribed permethrin cream, 5%, nightly for 7 days and then twice weekly thereafter. She reported improvement in her skin’s roughness but stopped using the cream because of a burning sensation of her skin that also correlated with an increase in her hydroxyurea dosage. Her treatment was then changed to ivermectin, 12 mg, 2 doses weekly, and her hydroxyurea dose was concurrently decreased with almost complete resolution of her clinical findings. She now takes ivermectin intermittently as needed for spinulosus recurrence (approximately once per month). Demodexfolliculorum is a saprophytic mite that lives in the hair follicle infundibulum and subsists on sebum and skin cells. Although this mite is commonly found in adults without associated cutaneous pathologic findings, they may be considered pathogenic in patients with skin eruptions when there is increased density of the mites to more than 5/cm2, they are present in the dermis, or there is a documented clinical response to Demodex folliculorum–directed therapies.1,2 Predisposing factors for increased Demodex mite density include increased age, diabetes mellitus, hemodialysis, and immunosuppression.3 Demodex folliculorum–associatedspinulosus has a clinically different appearance from Demodexfolliculorum–associated rosacea; thus, the clinical differential diagnosis should include the digitate keratosis disorders, such as multiple myeloma–associated hyperkeratotic spicules, trichodysplasia spinulosa–associated polyomavirus, and follicular hyperkeratosis, as well as ulerythema ophyrogenes.4 Biopsies will aid in distinguishing between these entities. The results from biopsies of Demodex folliculorum–associated spinulosus specimens revealed numerous D folliculorum mites protruding from hair follicles with surrounding hyperkeratosis and a lymphocytic infiltrate, whereas the results from biopsies of specimens from patients with the other conditions listed here may show incidental Demodex mites in the infundibulum. There are 3 previous case reports5-7 of Demodex folliculorum–associated spinulosus in the setting of polycythemia vera that was treated with hydroxyurea. Two of the 3 patients did not improve until hydroxyurea treatment was discontinued, while the third patient’s condition improved with prescribed permethrin cream, 1%.5-7 The authors of each case report concluded that the immunosuppressed state led to the development of spinulosus due to the Demodex mites although the pathogenesis is still unclear.5 We present another case of Demodex folliculorum–associated spinulosus in an elderly immunocompromised woman who was receiving treatment with hydroxyurea. We believe that Demodex folliculorum–associated spinulosus is an important entity to consider when evaluating a patient with follicular prominence because it is easily diagnosed by biopsy and may be easily treatable. Back to top Article Information Corresponding Author: Julia A. Curtis, MD, Department of Dermatology, University of Utah School of Medicine, 30 N 1900 E, Ste 4A330, Salt Lake City, UT 84132 (julia.curtis@hsc.utah.edu). Published Online: August 19, 2015. doi:10.1001/jamadermatol.2015.2339. Conflict of Interest Disclosures: None reported. References 1. Forton F, Seys B. Density of Demodex folliculorum in rosacea. Br J Dermatol. 1993;128(6):650-659.PubMedGoogle ScholarCrossref 2. Zhao YE, Wu LP, Peng Y, Cheng H. Retrospective analysis of the association between Demodex infestation and rosacea. Arch Dermatol. 2010;146(8):896-902.PubMedGoogle Scholar 3. Lacey N, Ní Raghallaigh S, Powell FC. Demodex mites—commensals, parasites or mutualistic organisms? Dermatology. 2011;222(2):128-130.PubMedGoogle ScholarCrossref 4. Caccetta TP, Dessauvagie B, McCallum D, Kumarasinghe SP. Multiple minute digitate hyperkeratosis. J Am Acad Dermatol. 2012;67(1):e49-e55.PubMedGoogle ScholarCrossref 5. Boutli F, Delli FS, Mourellou O. Demodicidosis as spinulosus of the face: a therapeutic challenge. J Eur Acad Dermatol Venereol. 2007;21(2):273-274.PubMedGoogle Scholar 6. Ballestero-Díez M, Daudén E, Ruíz-Genao DP, Fraga J, García-Díez A. Presence of Demodex in follicular hyperkeratotic spicules on the face. Acta Derm Venereol. 2004;84(5):407-408.PubMedGoogle Scholar 7. Fariña MC, Requena L, Sarasa JL, et al. Spinulosus of the face as a manifestation of demodicidosis. Br J Dermatol. 1998;138(5):901-903.PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Dermatology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 2015 American Medical Association. All Rights Reserved.
ISSN
2168-6068
eISSN
2168-6084
DOI
10.1001/jamadermatol.2015.2339
pmid
26288332
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Abstract

Case A woman in her 70s with a medical history that was significant for polycythemia vera and acute myelogenous leukemia was seen for evaluation of textural skin changes on her face that had been present for 2½ years. She denied having pruritus or pain, but her skin had a rough sandpapery sensation. She reported that the involvement extended to her neck, the upper part of her chest, and her bilateral forearms. She associated the onset of the textural changes with her diagnosis of polycythemia vera. She stated that it began before her diagnosis of acute myelogenous leukemia and the initiation of treatment with hydroxyurea 4 years later. She had previously received treatment with long courses of triamcinolone acetonide cream, 0.1%, bacitracin ointment, and a combination antibiotic ointment of neomycin sulfate, polymyxin B, and bacitracin, with no improvement of her skin’s condition. Her additional daily medication regimen included donepezil hydrochloride, melatonin, and aspirin, 81 mg. Findings from the patient’s physical examination revealed background erythema with numerous rough horny spicules at the follicular ostia on her face, ears, and neck; the upper part of her chest; and her bilateral forearms. Findings from the rest of her examination revealed a pigmented macule on her right cheek. Two punch biopsy specimens were obtained from her right cheek and right postauricular area and submitted for histopathologic examination (Figure). Figure. View LargeDownload Histopathologic images (hematoxylin-eosin) of the punch biopsy specimen from the right side of the lateral neck. Box Section Ref ID What Is The Diagnosis? Trichodysplasia spinulosa–associated polyomavirus Demodex folliculorum–associated spinulosus Ulerythema ophryogenes Hyperkeratotic spicules of multiple myeloma Read the Discussion. Discussion Diagnosis B. Demodex folliculorum–associated spinulosus Microscopic Findings and Clinical Course Findings from histopathologic examination revealed numerous Demodex folliculorum mites in the follicular infundibula with associated hyperkeratosis, focal lichenoid lymphocytic infiltrates, and scattered Civatte bodies (Figure). The patient was prescribed permethrin cream, 5%, nightly for 7 days and then twice weekly thereafter. She reported improvement in her skin’s roughness but stopped using the cream because of a burning sensation of her skin that also correlated with an increase in her hydroxyurea dosage. Her treatment was then changed to ivermectin, 12 mg, 2 doses weekly, and her hydroxyurea dose was concurrently decreased with almost complete resolution of her clinical findings. She now takes ivermectin intermittently as needed for spinulosus recurrence (approximately once per month). Demodexfolliculorum is a saprophytic mite that lives in the hair follicle infundibulum and subsists on sebum and skin cells. Although this mite is commonly found in adults without associated cutaneous pathologic findings, they may be considered pathogenic in patients with skin eruptions when there is increased density of the mites to more than 5/cm2, they are present in the dermis, or there is a documented clinical response to Demodex folliculorum–directed therapies.1,2 Predisposing factors for increased Demodex mite density include increased age, diabetes mellitus, hemodialysis, and immunosuppression.3 Demodex folliculorum–associatedspinulosus has a clinically different appearance from Demodexfolliculorum–associated rosacea; thus, the clinical differential diagnosis should include the digitate keratosis disorders, such as multiple myeloma–associated hyperkeratotic spicules, trichodysplasia spinulosa–associated polyomavirus, and follicular hyperkeratosis, as well as ulerythema ophyrogenes.4 Biopsies will aid in distinguishing between these entities. The results from biopsies of Demodex folliculorum–associated spinulosus specimens revealed numerous D folliculorum mites protruding from hair follicles with surrounding hyperkeratosis and a lymphocytic infiltrate, whereas the results from biopsies of specimens from patients with the other conditions listed here may show incidental Demodex mites in the infundibulum. There are 3 previous case reports5-7 of Demodex folliculorum–associated spinulosus in the setting of polycythemia vera that was treated with hydroxyurea. Two of the 3 patients did not improve until hydroxyurea treatment was discontinued, while the third patient’s condition improved with prescribed permethrin cream, 1%.5-7 The authors of each case report concluded that the immunosuppressed state led to the development of spinulosus due to the Demodex mites although the pathogenesis is still unclear.5 We present another case of Demodex folliculorum–associated spinulosus in an elderly immunocompromised woman who was receiving treatment with hydroxyurea. We believe that Demodex folliculorum–associated spinulosus is an important entity to consider when evaluating a patient with follicular prominence because it is easily diagnosed by biopsy and may be easily treatable. Back to top Article Information Corresponding Author: Julia A. Curtis, MD, Department of Dermatology, University of Utah School of Medicine, 30 N 1900 E, Ste 4A330, Salt Lake City, UT 84132 (julia.curtis@hsc.utah.edu). Published Online: August 19, 2015. doi:10.1001/jamadermatol.2015.2339. Conflict of Interest Disclosures: None reported. References 1. Forton F, Seys B. Density of Demodex folliculorum in rosacea. Br J Dermatol. 1993;128(6):650-659.PubMedGoogle ScholarCrossref 2. Zhao YE, Wu LP, Peng Y, Cheng H. Retrospective analysis of the association between Demodex infestation and rosacea. Arch Dermatol. 2010;146(8):896-902.PubMedGoogle Scholar 3. Lacey N, Ní Raghallaigh S, Powell FC. Demodex mites—commensals, parasites or mutualistic organisms? Dermatology. 2011;222(2):128-130.PubMedGoogle ScholarCrossref 4. Caccetta TP, Dessauvagie B, McCallum D, Kumarasinghe SP. Multiple minute digitate hyperkeratosis. J Am Acad Dermatol. 2012;67(1):e49-e55.PubMedGoogle ScholarCrossref 5. Boutli F, Delli FS, Mourellou O. Demodicidosis as spinulosus of the face: a therapeutic challenge. J Eur Acad Dermatol Venereol. 2007;21(2):273-274.PubMedGoogle Scholar 6. Ballestero-Díez M, Daudén E, Ruíz-Genao DP, Fraga J, García-Díez A. Presence of Demodex in follicular hyperkeratotic spicules on the face. Acta Derm Venereol. 2004;84(5):407-408.PubMedGoogle Scholar 7. Fariña MC, Requena L, Sarasa JL, et al. Spinulosus of the face as a manifestation of demodicidosis. Br J Dermatol. 1998;138(5):901-903.PubMedGoogle ScholarCrossref

Journal

JAMA DermatologyAmerican Medical Association

Published: Nov 1, 2015

References