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Biomedical subjects

D M Thappa

Publications and source records attributed to D M Thappa.

At least 37 records · Page 2Linked to original sources

An Indian case of keratoderma hereditarium mutilans (Vohwinkel's syndrome) associated with ichthyosiform dermatosis.

Keratoderma hereditarium mutilans (KHM) or Vohwinkel's syndrome (VS) is usually inherited as an autosomal dominant disease, but a recessive type has occasionally been described. We report a 19-year-old male born to consanguineous parents, who presented with KHM associated with generalized ichthyosiform dermatosis. On examination, his generalized ichthyosiform dermatosis was accompanied by severe 'honey comb' like palmoplantar keratoderma and marked hyperhidrosis. Bilateral fifth toes showed complete constricting bands just distal to the metatarsophalangeal joints leading to pseudoainhum. The histopathologic examination of a biopsy from the sole showed hyperkeratosis, marked parakeratosis, hypergranulosis, acanthosis, and a few mononuclear cells in the papillary dermis, consistent with the diagnosis of KHM. Several hereditary disorders may show varying degrees of hyperkeratosis and constriction of digits; their differentiating features are discussed. This case is reported for its rarity and interesting associated findings.

Adult↗

Alkaptonuric ochronosis presenting as palmoplantar pigmentation.

We describe a 37-year-old woman who presented with palmoplantar pigmentation, thickening and pitting of 4 years duration. Bluish pigmented patches were seen over the sclera of her eyes. Her lumbar spine showed typical calcification of the intervertebral discs. Addition of Benedict's reagent to a urine sample of the patient gave rise to greenish brown precipitate and brownish black supernatant. Alkalinization of urine turned it black. A biopsy of the palmar lesion demonstrated irregular breaking up, swelling and homogenization of collagen bundles in the reticular dermis. Yellow-brown (ochre coloured) pigment was seen lying within the collagen bundles and also freely in the deeper dermis confirming our clinical diagnosis of alkaptonuric ochronosis. To the best of our knowledge this is probably the second report of alkaptonuria presenting with palmoplantar pigmentation.

Adult↗

Primary pachydermoperiostosis: a case report.

Pachydermoperiostosis (PDP), a rare genodermatosis, occurred in a 38-year-old Indian male. He presented with progressive thickening of the skin on the face and scalp of 15 years duration. Widening of his wrists and ankles and broadening of the fingers and toes had also developed since then. He was born of a consanguineous marriage and had no family history of a similar disorder. He had the typical findings of complete form of PDP including cutis verticis gyrata, coarse facial features, clubbing of the digits in the skin, and periostosis and cortical thickening at the distal ends of long bones of the extremities and small bones of the hands and feet. PDP has two different forms--primary and secondary. These two entities are differentiated by family history and presence or absence of a primary lesion, usually in the lungs. Clinically, in secondary PDP, the cutaneous findings (pachydermia, seborrhoea, oiliness) are less severe than primary PDP; osteoarthropathy is more severe and painful in secondary PDP, especially with congenital heart disease. The present case was suffering from primary PDP that had expressed itself in its complete form.

Adult↗

The spectrum of mucocutaneous manifestations during the evolutionary phases of HIV disease: an emerging Indian scenario.

Third world countries, including India, lack sophisticated investigations to assess the progression of HIV disease. Hence, this study was undertaken to determine the clinical mucocutaneous markers of HIV disease and to establish its relationship with the stage of the disease. This was an observational institutional study of 75 patients with mucocutaneous disorders and HIV infection recruited over a period extending from September of 1996 to June of 1998. The patients with mucocutaneous lesions were staged according to the Centers for Disease Control classification system for HIV infection (1986). The most frequent mode of acquisition of HIV infection was heterosexual contact (96%). The patients were broadly categorized into two groups. The AIDS group was comprised of patients who were in group IV and the early HIV infection group included patients in group II and III; none were detected in group I. Forty-eight cases belonged to the AIDS group, and 27 belonged to the early HIV infection group. A total of 207 dermatoses were diagnosed and grouped as fungal, viral, bacterial, or miscellaneous. The common mucocutaneous disorders in order of frequency observed in this study were: candidiasis, dermatophytosis, herpes simplex, oral aphthae, xerosis/ichthyosis, scabies, HPV infection, molluscum contagiosum, and psoriasis. Xerosis/acquired ichthyosis and giant molluscum contagiosum were characteristically seen in group IV of HIV disease, whereas oral candidiasis, oral aphthae, papular dermatitis of HIV, and psoriasis were early warning signs. The mean number of dermatoses per patient in group IV was 3.15; in group III, it was 2.41; and in group II, it was 1.5. There was a statistically significant difference between the early HIV infection group and AIDS group with regard to number of dermatoses. Apart from syphilis and human papilloma virus infection, the treatment outcomes were satisfactory.

Adolescent↗