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traveled abroad but had a history of intravenous drug use. Therefore, leishmania infection was most likely acquired through needle sharing rather than the usual vector-borne transmission. This case report demonstrates the importance of considering PKDL in the differential diagnosis of non-pruritic papules in HIV-positive patients and the clinical similarity of this disease with secondary syphilis. In addition, it brings to light another issue, which is the emergence of traditionally vector-borne diseases outside of their endemic areas, due to alternative means of transmission such as needle sharing. Joana Antunes1, MD Joa˜o Borges da Costa1, MD, PhD Mafalda Guimara˜es2, MD Ineˆs Vaz-Pinto2, MD Luı´s Soares-Almeida1, MD, PhD Paulo Filipe1, MD, PhD 1 Clínica Dermatológica Universitária- Hospital de Santa Maria, Lisbon, Portugal and 2Unidade de Tratamento de Imunodeficiência- HPP Hospital de Cascais, Cascais, Portugal. Joana Antunes, MD Clínica Dermatológica Universitária - Hospital de Santa Maria Av. Prof. Egas Moniz 1649-035 Lisbon Portugal E-mail:
[email protected] Trichodysplasia spinulosa in an adolescent with cystic fibrosis and lung transplantation
Trichodysplasia spinulosa (TS) is a distinctive, rare, probably virally mediated disorder affecting immunosuppressed patients. In the literature, there are only 20 reported cases of TS,1–6 but descriptions of these cases typically include very limited follow-up duration. We present a case of TS with unusually long follow-up. A 14-year-old Caucasian girl with a history of cystic fibrosis presented three years after a lung transplantation with a progressive, disfiguring eruption on the face, extremities, and trunk. Physical examination revealed numerous 2–3-mm papules with spiny, follicular keratoses, especially involving the central face (Fig. 1). She also demonstrated skin thickening and alopecia of the eyebrows. Medications included cyclosporine, muromonabCD3, mycophenolate mofetil, methotrexate, prednisone, and tobramycin. A shave biopsy from the nose and a punch biopsy from the arm revealed dilated large anagen hairs with infundibular dilation and inner root sheath-type cornification (Fig. 2). The bulbs showed no papillae, and International Journal of Dermatology 2013, 52, 1567–1624
Conflicts of interest: The authors have no conflicts of interest to disclose.
References 1 Alvar J, Aparicio P, Aseffa A, et al. The relationship between leishmaniasis and AIDS: the second 10 years. Clin Microbiol Rev 2008; 21: 334–359. 2 ICDDR, B. Post kala-azar dermal leishmaniasis: new observations challenge previous assumptions. Health Sci Bull 2007; 5: 6–12. 3 Zijlstra E, Musa A, Khalil E, et al. Post-kala-azar dermal leishmaniasis. Lancet Infect Dis 2003; 3: 87–98. 4 Stark D, Pett S, Marriott D, Harkness J. Post-kala-azar dermal leishmaniasis due to Leishmania infantum in a human immunodeficiency vírus type 1-infected patient. J Clin Microbiol 2006; 44: 1178–1180. 5 Catorze G. Leishmaniose e SIDA. Med Cutan Iber Lat Am 2005; 33: 237–250. 6 Antinori S, Longhi E, Bestetti G, et al. Post-kala-azar dermal leishmaniasis as an immune reconstitution inflammatory syndrome in a patient with acquired immune deficiency syndrome. Br J Dermatol 2007; 157: 1032–1036. 7 Boumis E, Chinello P, Della Rocca C, et al. Atypical disseminated leishmaniasis resembling post-kala-azar dermal leishmaniasis in an HIV-infected patient. Int J STD AIDS 2006; 17: 351–353. 8 Catorze G, Alberto J, Afonso A, et al. Leishmania infantum/HIV co-infection: cutaneous lesions following treatment of visceral leishmaniasis. Ann Dermatol Venereol 2006; 133: 39–42.
the lower segments of follicles were comprised of nucleated cells with intranuclear inclusion-like areas and irregular-shaped cytoplasmic inclusions/granules. Cryotherapy and keratolytics were not effective. Fortunately, her cutaneous lesions gradually improved over the next few years
Figure 1 Widespread follicular papules, spiny keratoses and skin thickening, most prominent on the central face. ª 2013 The International Society of Dermatology
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subsequent to changes in her immunosuppression, including changing cyclosporine to tacrolimus. She was clear within 2.5 years. She experienced no recurrences. However, her course was complicated by graft rejection, pseudomonas bronchitis, bronchiolitis obliterans, diabetes, and (a)
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chronic renal failure. She died nine years after her initial presentation. Trichodysplasia spinulosa is a recently described, rare, disfiguring condition, with only 20 cases previously reported in the literature.1–6 TS is characterized by hair-like, spiny, follicular keratoses and skin-colored, infiltrated papules, with a predilection for the central face. Alopecia is common, particularly involving the eyebrows and eyelashes. Different terminologies have been used to describe this entity, including viral-associated trichodysplasia, viral-associated TS, viral-associated trichodysplasia of immunosuppression, cyclosporine-induced folliculodystrophy, and pilomatrix dysplasia. TS only occurs in patients with immunosuppression, especially transplant patients and patients taking chemotherapy. TS has been reported in a wide range of ages (5–69 years). Half of affected patients have been pediatric.3–5 Histologically, TS is characterized by enlarged, bulbous anagen hair follicles dilated infundibulum with inner root sheath-type cornification, nucleated cells with trichohyalin granules and missing hair papillae. Ultrastructurally, intranuclear viral particles may be seen, particularly in inner root sheath cells. Recently, a new human polyomavirus (TS-associated polyomavirus, or TSV) has been identified in one affected patient and proposed to cause TS.5 Treatment for TS is limited. As with our case, adjustments in immunosuppression may lead to dramatic clinical improvement. Topical cidofovir and oral valganciclovir have also been beneficial in some cases.3–5 Ablative methods, antibiotics, and retinoids have generally not been helpful and are probably best avoided. In summary, TS is an increasingly reported, severe follicular eruption in immunosuppressed patients, possibly due to a polyomavirus. Our case demonstrates typical clinical features, adds another pediatric case, and provides unusually long follow-up. David R. Berk, MD Dongsi Lu, MD, PhD, and Susan J. Bayliss, MD Department of Pathology and Immunology, Washington University School of Medicine, St Louis, MO, USA E-mail:
[email protected] References Figure 2 (a and b) Shave biopsy from the nose demonstrat-
ing dilated large anagen hairs, infundibular dilation, inner root sheath-type cornification and absence of hair papillae. (c) Cells with prominent trichohyalin granules were present in the lower segment of follicles (hematoxylin and eosin, magnification ·4, ·10, ·20) ª 2013 The International Society of Dermatology
1 Izakovic J, Buchner SA, Duggelin M, et al. [Hair-like hyperkeratoses in patients with kidney transplants. A new cyclosporin side-effect]. Hautarzt 1995; 46: 841–846. 2 Haycox CL, Kim S, Fleckman P, et al. Trichodysplasia spinulosa – a newly described folliculocentric viral infection in an immunocompromised host. J Investig Dermatol Symp Proc 1999; 4: 268–271. International Journal of Dermatology 2013, 52, 1567–1624
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3 Benoit T, Bacelieri R, Morrell DS, Metcalf J. Viralassociated trichodysplasia of immunosuppression: report of a pediatric patient with response to oral valganciclovir. Arch Dermatol 2010; 146: 871–874. 4 Schwieger-Briel A, Balma-Mena A, Ngan B, et al. Trichodysplasia spinulosa – a rare complication in immunosuppressed patients. Pediatr Dermatol 2010; 27: 509–513.
Woolly hair nevus: neonatal alopecia can be an initial manifestation
Dear Editor, Woolly hair nevus is a rare, nonhereditary condition characterized by tightly curled hair in restricted areas of the scalp. Woolly hair nevus first appears at birth or when the scalp hair begins to grow.1 Here we report a case of woolly hair nevus in which a patch of hair loss at birth was the initial manifestation. A 12-day-old Japanese boy presented with a patch of scalp showing hair loss since birth. Examination found an area measuring about 4 cm in diameter showing hair loss on the right occipital region (Fig. 1a). The alopecic patch did not show complete hair loss but rather demonstrated sparse thin hair. There were no broken hairs and exclamation point hairs, and a hair pull test was negative at the periphery of the patch. The patient was otherwise in good health. There was no family history of hair disease. We could not establish a diagnosis and decided to take a wait-and-see approach. One year later, when the baby was brought back to our hospital, there were many short curly hairs growing in the alopecic patch. These hairs were smaller in diameter and lighter in color than the surrounding scalp hair (Fig. 1b). On the left side of the nape of the neck, there was a light brown, slightly elevated pigmented lesion (Fig. 1c). Based on the clinical appearance, we finally diagnosed the lesion as woolly hair nevus. Woolly hair nevus is a rare condition associated with unruly, curly hair in limited to well-demarcated areas of the scalp. The woolly hair is generally a different color and a smaller diameter hair shaft than the normal hair. About 50% of woolly hair nevi are associated with an ipsilateral pigmented, or epidermal nevus, usually on the neck or arm. Woolly hair nevus is usually apparent at birth or when the hair starts to grow. In our case, woolly hair nevus was not apparent at birth; instead, the patient exhibited a patch of hair loss resembling alopecia areata. This alopecic patch did not show complete hair loss but rather showed some short thin hair, although there was no apparent woolly hair. However, when the patient returned to our hospital one year after birth, woolly hair had become obvious. We International Journal of Dermatology 2013, 52, 1567–1624
5 van der Meijden E, Janssens RW, Lauber C, et al. Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromised patient. PLoS Pathog 2010; 6: e1001024. 6 Matthews MR, Wang RC, Reddick RL, et al. Viralassociated trichodysplasia spinulosa: a case with electron microscopic and molecular detection of the trichodysplasia spinulosa-associated human polyomavirus. J Cutan Pathol 2011; 38: 420–431.
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Figure 1 (a) A patch of hair loss in the right occipital region. (b) Woolly hair nevus was apparent 1 year later. (c) A lightbrown, slightly elevated pigmented lesion on left side of the nape of the neck ª 2013 The International Society of Dermatology