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Letters to the Editor
features of piebaldism and their severity does not parallel
the severity of the depigmentation. The present cases
showed the extreme variation in the manifestation of
freckles though the depigmented patches were typical of
piebaldism. A separate, yet unidentified locus probably
controls for the hyperpigmented macules of piebaldism,
distinct from the locus that controls depigmentation.
This hypothesis of separate genetic control for the
depigmented and hyperpigmented components has
two implications. One, it can explain variations of
the phenotypic pattern of freckles independent of
depigmentation. Secondly, “twin spotting” as the
mechanism for development of pigmented macules
within and around depigmented patches, another very
classical and distinctive form of hyperpigmentation in
piebaldism, appears possible. However further genetic
studies are necessary to confirm this hypothesis.
Nilendu Sarma, Sayantani Chakraborty,
Dulal Chandra Bhanja,
Sneha Ranjan Bhattachraya
Department of Dermatology, NRS Medical College, Kolkata, West
Bengal, India
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9.
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129404
Scrub typhus in a child: Looking
behind the ear and beyond
Address for correspondence: Dr. Nilendu Sarma,
PN Colony, Sapui Para, Bally, Howrah - 711 227,
West Bengal, India.
E-mail: [email protected]
Sir,
Scrub typhus is one of the differential diagnoses
that need to be considered in a child with fever
and hepatosplenomegaly. Early identification and
treatment is crucial in preventing complications. This
letter highlights the importance of recognizing an
eschar, a clue to the diagnosis.
Sánchez-Martín M, Pérez-Losada J, Rodríguez-García A,
González-Sánchez B, Korf BR, Kuster W, et al. Deletion of the
SLUG (SNAI2) gene results in human piebaldism. Am J Med
Genet A 2003;122A: 125-32.
Fleischman RA. Human piebald trait resulting from a dominant
negative mutant allele of the c-kit membrane receptor gene.
J Clin Invest 1992;89:1713-7.
Davis BK, Verdol LD. Expansion and contraction of
hypomelanotic areas in human piebaldism. Hum Genet
1976;34:163-70.
Spritz RA, Itin PH, Gutmann DH. Piebaldism and
neurofibromatosis type 1: Horses of very different colors.
J Invest Dermatol 2004;122:xxxiv-xxxv.
Chiu YE, Dugan S, Basel D, Siegel DH. Association of piebaldism,
multiple café-au-lait macules, and intertriginous freckling:
Clinical evidence of a common pathway between KIT and
sprouty-related, ena/vasodilator-stimulated phosphoprotein
homology-1 domain containing protein 1 (SPRED1). Pediatr
Dermatol 2013;30:379-82.
Duarte AF, Mota A, Baudrier T, Morais P, Santos A, Cerqueira R,
et al. Piebaldism and neurofibromatosis type 1: Family report.
Dermatol Online J 2010;16:11.
Angelo C, Cianchini G, Grosso MG, Zambruno G, Cavalieri R,
Paradisi M. Association of piebaldism and neurofibromatosis
type 1 in a girl. Pediatr Dermatol 2001;18:490-3.
Chang T, McGrae JD Jr, Hashimoto K. Ultrastructural study of
two patients with both piebaldism and neurofibromatosis 1.
Pediatr Dermatol 1993;10:224-34.
Stevens CA, Chiang PW, Messiaen LM. Café-au-lait macules
and intertriginous freckling in piebaldism: Clinical overlap
A 3-year-old male child was brought with history of fever
for a week with no other complaints. Fever was high
grade and intermittent. On examination, he was febrile
with a pulse rate of 140/min, respiratory rate of 34/min,
oxygen saturation of 100% in room air, and a normal
cardiovascular status with blood pressure of 90/50 mmHg.
His respiratory examination was unremarkable except
for mild tachypnea and abdominal examination revealed
soft hepato-splenomegaly. He had no neurological
deficits. A thorough head to foot examination revealed
an eschar behind his left pinna [Figure 1] that clinched
the diagnosis. The lesion was 10 mm in size with a
dark brown to black scab resembling a cigarette burn.
The edge of the lesion was undermined with healthy
granulation under the scab. There was no rash or
significant lymphadenopathy. Investigations revealed a
normal total and differential count. Smear for malarial
parasite was negative, Widal test was negative, and blood
culture was sterile. Weil-Felix test and scrub typhus
immunochromatographic test were both positive and the
child was diagnosed to have scrub typhus. He recovered
following 5 days of oral azithromycin.
REFERENCES
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with neurofibromatosis type 1 and Legius syndrome. Am J Med
Genet A 2012;158A: 1195-9.
10. Neurofibromatosis. Conference statement. National Institutes
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1988;45:575-8.
11. Duarte A, Mota A, Baudrier T, Morais P, Santos A, Cerqueira R,
et al. Piebaldism and neurofibromatosis: State of knowledge.
Dermatol Online J 2013;19:17.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2
165
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Letters to the Editor
failure, and acute respiratory distress syndrome.[3,5]
Differential diagnoses include dengue fever, leptospirosis,
typhoid fever, and malaria. Diagnosis is confirmed
by isolation of organism in culture or serological tests
like immunoglobulin M (IgM) capture enzyme-linked
immunosorbent assay (ELISA) which have a retrospective
value but are confirmatory. Other diagnostic modalities
include indirect fluorescent antibody test, Weil-Felix
test, and immunochromatographic tests. Weil-Felix test
has low sensitivity (43%), but high specificity (98%) for
a titer above 1:80.[3] Immunochromatographic tests have
a higher sensitivity and specificity in comparison to
Weil-Felix test.
Figure 1: Healed eschar behind the left ear
ACKNOWLEDGMENT
Scrub typhus is an emerging rickettsial infection caused
by Orientia tsutsugamushi observed in south and
southeast Asian regions. Rats and rodents are reservoirs
and it is transmitted by the trombiculid mite.[1] The mite
prefers moist and hidden areas of the body where its
bite inoculates the bacteria. Local multiplication of the
bacteria produces a papule which ulcerates and undergoes
necrosis and forms the eschar with an erythematous
rim with or without regional lymphadenopathy.[2] The
eschar heals completely in 3-4 week with minimal
hyperpigmentation or scarring. An eschar is seen in 11%
to 92% of individuals with scrub typhus.[3,4] Eschars are
commonly seen in the axilla, groin, trunk, scrotal folds,
and rarely in the scalp, behind the ear, or within the ear
canal. The latter sites are likely to be overlooked if the
diagnosis is not suspected. The eschar of scrub typhus
differs from that of cutaneous anthrax in that the former
resembles a cigarette burn type of shallow ulcer with
undermined edges, whereas in the latter, the lesion is
nodular with elevated margins and black scab. Similarly,
the eschar of scrub typhus can be differentiated from
impetigo by the absence of honey colored crusts seen
in the latter. Echthyma, another skin lesion which
mimics the eschar of scrub typhus is usually larger in
size. Other cutaneous findings in scrub typhus include a
maculopapular rash seen in one-third of individuals and
rarely, multiple eschars.
After entry of the organism into the body, there is
proliferation in the endothelial lining of small blood
vessels causing small vessel vasculitis especially of heart,
brain, liver, and kidney. Direct invasion of the organism
as well as the vasculitic response contribute to the disease
manifestations.[5] Response to treatment is immediate with
defervescence noted within 24-48 h. Azithromycin and
doxycycline are equally effective. Delayed treatment leads
to complications like pneumonia, meningoencephalitis,
myocarditis, capillary leak leading to shock, renal
166
We acknowledge with gratitude the services of Dr. Selvaraj
Stephen, Professor of Microbiology for helping us with the
serological tests.
Chandrasekaran Venkatesh, Russelian Arulraj,
Palanisamy Soundararajan,
Sadagopan Srinivasan
Department of Pediatrics, Mahatma Gandhi Medical College and
Research Institute, Pillaiyarkuppam, Puducherry, India
Address for correspondence: Dr. Chandrasekaran Venkatesh,
Department of Pediatrics, Mahatma Gandhi Medical College and
Research Institute, Pillaiyarkuppam, Puducherry - 607 402, India.
E-mail: [email protected]
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Varghese GM, Abraham OC, Mathai D, Thomas K, Aaron R,
Kavitha ML, et al. Scrub typhus among hospitalized patients
with febrile illness in South India: Magnitude and clinical
predictors. J Infect 2006;52:56-60.
Kundavaram AP, Jonathan AJ, Nathaniel SD, Varghese GM.
Eschar in scrub typhus: A valuable clue to the diagnosis.
J Postgrad Med 2013;59:177-8.
Kumar M, Krishnamurthy S, Delhikumar CG, Narayanan P,
Biswal N, Srinivasan S. Scrub typhus in children at a tertiary
hospital in Southern India: Clinical profile and complications.
J Infect Public Health 2012;5:82-8.
Kim DM, Won KJ, Park CY, Yu KD, Kim HS, Yang TY, et al.
Distribution of eschars on the body of scrub typhus patients:
A prospective study. Am J Trop Med Hyg 2007;76:806-9.
Park JS, Jee YK, Lee KY, Kim KY, Myong NH, Seo PW. Acute
respiratory distress syndrome with scrub typhus: Diffuse
alveolar damage without pulmonary vasculitis. J Korean Med
Sci 2000;15:343-5.
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