Arnaz Bankwalla, Malesa Kazi, Anjum Kanjee.
Atopic Dermatitis in a patient with Kartegeners Syndrome.
J Pak Assoc Derma Jan ;11(1):64-8.

Kartegeners Syndrome or Primary Ciliary Dyskinesia is a rare condition. Its association with Atopic Dermatitis has not been reported previously. We report a case of Kartegeners Syndrome with Atopic Dermatitis from Pakistan, who presented with itchy pigmented lichenified lesions on the extremities.

CASE REPORT: A twenty one year old unmarried male presented with a ten year history of fluctuating episodes of intense itching and pigmented lesions involving both the upper and the lower extremities. The patient gave a history of respiratory tract infection since childhood, which would cause him to have low-grade fever and cough productive of mucoid and sometimes foul smelling sputum. Family history was significant in that he was born of consangious parents but the other siblings were unaffected. He did not give a family history of asthma or Tuberculosis. Physical examination revealed pigmented indilichenified lesions involving both the upper and lower extremities, the lichenification was marked in the popliteal and cubital fossae. The patient was found to be anemic prominent clubbing of fingers and toes. The respiratory system revealed increased an teroposterior diameter of the chest with recession of the tercostal spaces and supraclavicular fossae on inspiration. Coarse crepts were auscultated in both the lower lung zones. Apex beat was palpable on the right side. Liver dullness was not appreciated on the right side. Laboratory test results revealed Hemoglobin of 11 gms%, a white blood cell count of 14, 800/cumm, with neutrophil content of 75%, and ESR of 20 mm. X-rays chest (PA) view revealed Prominent bronchovascular markings in both lower lung zones indicating basal bronchiectatic changes with peribronchial cuffing indicating infective changes. On the right side multiple shadows of air fluid interfaces indicating cystic pulmonary changes with suspected basal pulmonary loss of volume (segmental collapse) was seen. Gastric air shadow was seen on the right indicating dextrocardia, with evidence of lateral transposition of liver. The patient has Situs Inverses Viscerum. This typical radiological appearance along with bronchiectasis and hypoplastic frontal sinus on X ray Paranasal Sinus helped confirmed the diagnosis of Kartegeners Syndrome. Pulmonary function tests showed a mixed limitation with restricted volumes 77% of predicted. The FEV1 was 70% suggesting mild obstructive airway limitation. This obstructive feature did not respond to bronchodilators, as there was no reversibility. The clinical features together with radiological appearance of Situs Inversus bronchiectasis and absent frontal sinus confirmed the diagnosis of Kartegeners Syndrome at this point. The histopathological examination of Skin biopsy showed features consistent with those of chronic eczema, hyperkeratosis acanthoses papillomatosis and lymphocytic infiltration of dermis. The clinical diagnosis of Atopic Dermatitis was confirmed on the histopathological examination of the skin lesions. The patient was treated for Atopic Dermatitis and his symptoms improved considerably.

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