A CURIOUS CASE OF COEXISTENT HEMOGLOBIN E DISEASE AND SPONGIOTIC DERMATITIS IN THREE INDIVIDUALS OF SAME ETHNIC ORIGIN

We present here three adult individuals, one male aged 25 years and two females aged 24 and 72 years, who presented to the dermatology outpatient department with complaints of generalized skin rash [diagnosed subsequently as spongiotic dermatitis] throughout the body. As part of routine investigations, blood of all three patients were sent for complete hemogram and HBA1c, among other routinely requisitioned laboratory investigations. Routine hemogram revealed anemia for all three subjects [Hemoglobin in the range of 6.5 -10.5 g/dL]. While attempting to ascertain HbA1c percentages, values were undetectable by HPLC. Since such a scenario is possible in the setting of hemoglobinopathy, particularly in homozygous condition, a hemoglobin variant study was conducted on the blood samples of all three subjects. The variant study revealed abnormally elevated HbA2 fractions of all individuals [89.0 % - 99.5%], strongly supporting the existence of hemoglobin E disease, as did the concomitant presence of anemia in routine hemogram. A detailed history taking revealed even more common features. Coincidentally all three individuals were residents of the same geographical area and shared a common ethnic background. One of the subjects hailed from Alipurduar district of West Bengal, India while the other two were residents of Dinajpur district of Bangladesh. Coincidentally, both these districts now belonging to two different countries, were one and the same district, in pre-independent India. The spongiotic dermatitis, a form of atopic dermatitis is also known to affect individuals residing in a common environment. None of the subjects shared any familial or marital ties with one another. We thus concluded that ethnic and environmental commonalities can very well lead to two apparently unrelated disease conditions manifesting in individuals who share no familial or marital ties with one another.

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INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH
Published
2025-04-01
DOI
https://doi.org/10.36106/ijsr/1805981
Primary Topic
Porphyrin Metabolism and Disorders
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article
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article

A CURIOUS CASE OF COEXISTENT HEMOGLOBIN E DISEASE AND SPONGIOTIC DERMATITIS IN THREE INDIVIDUALS OF SAME ETHNIC ORIGIN

Susruta Sen, Indrani Pathak, Pabitra Chandra Das, Sharmistha Choudhuri et al.
INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH
Porphyrin Metabolism and Disorders
article

A CURIOUS CASE OF COEXISTENT HEMOGLOBIN E DISEASE AND SPONGIOTIC DERMATITIS IN THREE INDIVIDUALS OF SAME ETHNIC ORIGIN

Susruta Sen, Indrani Pathak, Pabitra Chandra Das, Sharmistha Choudhuri, Animesh Sardar
article en

Abstract

We present here three adult individuals, one male aged 25 years and two females aged 24 and 72 years, who presented to the dermatology outpatient department with complaints of generalized skin rash [diagnosed subsequently as spongiotic dermatitis] throughout the body. As part of routine investigations, blood of all three patients were sent for complete hemogram and HBA1c, among other routinely requisitioned laboratory investigations. Routine hemogram revealed anemia for all three subjects [Hemoglobin in the range of 6.5 -10.5 g/dL]. While attempting to ascertain HbA1c percentages, values were undetectable by HPLC. Since such a scenario is possible in the setting of hemoglobinopathy, particularly in homozygous condition, a hemoglobin variant study was conducted on the blood samples of all three subjects. The variant study revealed abnormally elevated HbA2 fractions of all individuals [89.0 % - 99.5%], strongly supporting the existence of hemoglobin E disease, as did the concomitant presence of anemia in routine hemogram. A detailed history taking revealed even more common features. Coincidentally all three individuals were residents of the same geographical area and shared a common ethnic background. One of the subjects hailed from Alipurduar district of West Bengal, India while the other two were residents of Dinajpur district of Bangladesh. Coincidentally, both these districts now belonging to two different countries, were one and the same district, in pre-independent India. The spongiotic dermatitis, a form of atopic dermatitis is also known to affect individuals residing in a common environment. None of the subjects shared any familial or marital ties with one another. We thus concluded that ethnic and environmental commonalities can very well lead to two apparently unrelated disease conditions manifesting in individuals who share no familial or marital ties with one another.

INTERNATIONAL JOURNAL OF SCIENTIFIC RESEARCH
R. G. Kar Medical College and Hospital (IN), Art Research Centre of the Slovak Academy of Sciences (SK), Medical College and Hospital, Kolkata (IN)
Openalex Percentile: Top 93%
Porphyrin Metabolism and Disorders
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