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NASOPHARYNGEAL MYIASIS IN ANHIDROTIC ECTODERMAL DYSPLASIA
Riyaz A* ; MD, DCH, DNB, DM,Najeeba Riyaz MD, DVD, DNB**
Anoop P; MBBS, DCH, MD***
*Associate Professor, Department of Pediatrics
**Professor & Head,Department of Dermatology
***Postgraduate Student, Department of Pediatrics
Calicut Medical College, Calicut-8
KERALA, INDIA.
Address for Correspondence
Dr. Riyaz A,
"Arakkal" house,
Chalappuram,
Calicut-673 002,
Kerala, India.
Phone : 91 (0) 495 - 2305095 (Res)
E-mail: saif_gem@hotmail.com
ABSTRACT
Growth of larval forms of the common housefly inside the nasopharynx of a girl is reported. She had anhidrotic ectodermal dysplasia, which is known to be associated with atrophic rhinitis, hypoplastic turbinates and purulent nasal secretions. Inadequate hygiene in presence of these predisposing factors could have contributed to her nasopharyngeal
myiasis.
KEY WORDS
Anhidrotic ectodermal dysplasia, housefly, myiasis.
INTRODUCTION:
Myiasis is the infestation of body tissues of humans and animals by the larvae of Diptera. Unhygienic practices and contaminated wounds are known predisposing factors. Nasopharyngeal myiasis in a girl with the rarer autosomal recessive form of anhidrotic ectodermal dysplasia is reported. Though abnormalities of the nasal cavity and mucosa are not uncommon in this disorder, larval infestation of the nasopharynx has not been reported so far.
CASE REPORT
A three year old girl born of second degree consanguineous parents presented with epistaxis and discharge of worms through both nostrils. She had sparse blond hair with scanty eyelashes and anodontia. She could not sweat and had a hoarse voice. Her nails were normal. Both the parents were normal, but two of her paternal uncles had a similar appearance (Figure-1).
She was highly irritable and had a swollen nose. There was foul smelling nasal discharge for which she had received several courses of antibiotics prior to admission. A nasopharyngeal endoscopy revealed abundant larvae in the nasal cavity. They were removed and the nares irrigated. Subsequently they were identified to be the larval forms of Musca domestica, the common housefly (Figure-2). She is now under follow up, with periodic nasal irrigation. Presently she is doing well and has not had a recurrence of similar complaints.
DISCUSSION
Anhidrotic ectodermal dysplasia, also known as Christ-Siemens-Touraine syndrome, was first described by Thurman in
1848.(1) It is characterized by virtual absence of eccrine and sebaceous glands, abnormalities of skin, hair and teeth and a characteristic facies. It is inherited as an X-linked recessive disorder and more than 90% of patients are males. Females may occasionally be affected by the much rarer autosomal recessive form, as in this
girl.(2) In her case, consanguinity further supports this pattern of inheritance. However, whether an autosomal recessive form actually exists is controversial and has been challenged by a few
authors.(3)
Skin is typically dry and shiny, but not icthyotic. Patients are unable to sweat due to a marked reduction in the number of sebaceous and eccrine sweat
glands.(4) Intolerance to heat and fever is remarkable. Hair is scanty and depigmented; eyelashes are thin or absent. The facies is similar in affected individuals, with frontal bossing, prominent supraorbital ridges, saddle nose and a pointed chin. Dentition is usually delayed. Central incisors when present, are small and peg-like. Hearing and visual anomalies are occasionally encountered. This girl as well as her uncles had almost all the typical features. However, their hearing and vision were normal.
In addition to these typical features, absence of nasal, buccal and bronchial mucous glands is the hallmark of this
disorder.(4) Atrophic rhinitis and persistent foul smelling nasal discharge are quite common. Hypoplastic mucosa, thin nares and failure to develop nasal turbinates result in a roomy nasal cavity, which is highly susceptible to infections.1 Frequent irrigation of the nares may be required to limit the severity of purulent rhinitis. Persistent nasal crusting is also well
documented.(5) However, frank nasal myiasis in such patients has not been reported in the literature.
The persistent presence of purulent nasal secretions over the philtrum and anterior nares could have attracted houseflies to these sites, with subsequent deposition of eggs. Frequent 'blowing-in' of the nose is a common practice among children with nasal discharge, which results in re-introduction of the contaminated nasal secretions into the posterior nares. This could have been the probable mechanism of entry of eggs into the nasopharynx, thus favouring the completion of their life cycle high inside the nasal cavity.
This case is reported on account of the unusual pattern of inheritance of anhidrotic ectodermal dysplasia as well as the hitherto undescribed association with nasopharyngeal myiasis. The importance of oral and nasal hygiene in such patients is highlighted. A periodic nasopharyngeal examination may be helpful in their outpatient follow up. In addition, regular nasal irrigation seems to be more beneficial than antibiotics and nasal decongestants for the treatment of purulent rhinitis in such patients.
REFERENCES
1. Jones KL. Hypohidrotic ectodermal dysplasia syndrome. In : Smith's recognizable patterns of human congenital malformation 5th Ed, WB Saunders Co., Philadelphia, 1997; 540-541.
2. Anton-Lamprecht I, Schleiermacher E, Wolf M. Autosomal recessive anhidrotic ectodermal dysplasia : report of a case and discrimination of diagnostic features. Birth Defects 1988; 24 : 183.
3. Sybert VP. Hypohidrotic ectodermal dysplasia : argument against an autosomal recessive form clinically indistinguishable from X-linked hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). Pediatr Dermatol 1989; 6 : 76.
4. Reed WB, Lopez DA, Landing B. Clinical spectrum of anhidrotic ectodermal dysplasia. Arch Dermatol 1970; 102 : 134.
5. Al-Jassim AH, Swift AC. Persistent nasal crusting due to hypohidrotic ectodermal dysplasia. J Laryngol Otol 1996; 110 : 379.
FIGURE LEGENDS
1. Figure 1 : Girl with sparse hair and eyelashes with anodontia; the nose is swollen due to irritant
myiasis.
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2. Figure 2: Note that her uncles also have a similar facies.
3. Figure 3:Larval form of Musca domestica removed from the
nasopharynx.
| This
is a peer reviewed article. Accepted for publication on April
2,2004
Cite
as:
Riyaz A,Riyaz
N,Anoop P.Nasopharyngeal
Myiasis in Anhidrotic Ectodermal Dysplasia
Calicut
Medical Journal 2004;2(2):e7
URL: http://www.calicutmedicaljournal.org/2004/2/2/e7
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