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Biomedical subjects

Devinder M Thappa

Publications and source records attributed to Devinder M Thappa.

14 recordsLinked to original sources
PubMed2005

Segmental neurofibromatosis of face.

A 38-year-old man presented with asymptomatic skin lesions over the left side of the face of 5-years duration. He had multiple discrete soft-to-firm papules and nodules on the left side of the face along the distribution of the mandibular division of the trigeminal nerve. Histopathology examination of one of the nodules (face) showed a non-encapsulated tumor of the dermis with normal overlying epidermis. The tumor consisted of loosely spaced spindle cells and wavy collagenous strands in a clear matrix. These features were consistent with our clinical diagnosis of segmental neurofibromatosis. This case is reported for its rarity and typical manifestations.

Adult↗
PubMed2005

Disseminated cutaneous rhinosporidiosis.

Rhinosporidiosis is a chronic granulomatous disorder caused by Rhinosporidium seeberi. It frequently involves the nasopharynx and occasionally affects the skin. We herewith report a 55-year-old man who has disseminated cutaneous rhinosporidiosis. He presents with multiple reddish lesions over the nose of 10 year's duration. In the past year, he develops skin lesions over the right arm and over back. Histopathological examination of the skin biopsy specimen from the representative cutaneous lesions shows hyperplastic epithelium with numerous globular cysts of varying shape, representing sporangia in different stages of development. His serology for HIV infection by ELISA is negative. On the basis of these clinical and histopathological findings, a diagnosis of nasal rhinosporidiosis with cutaneous dissemination is made.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2004

Subungual exostosis of the thumb.

A 19-year-old girl presented with painful raised skin lesions over the right thumb for the past one year that had ulcerated over the last one month. On examination, a lobulated nodule of bony-hard consistency, measuring around 2 cm was present over the lateral aspect of the tip of the right thumb. The lesion was tender and the overlying skin showed superficial erosions. Roentgenogram of the hand showed mature bone projecting from the distal end of the terminal phalanx of the right thumb forming a "Y"-like bifurcation. Histology from a bit of excised tissue from the lesion (thumb) showed evidence of mature trabecular bone with a fibrocartilaginous cap in the deep dermis. There was no evidence of malignant change on histology. Local excision of the entire lesion was done and there has been no recurrence till date.

Adult↗
PubMed2004

Leprous osteitis presenting as bone cyst and erosions.

A 30-year-old man presented to the Hansen outpatient department with swelling and ulceration of toes for 2 months and swelling of the right fifth and fourth fingers and the left second finger for 1 month. In addition to skin lesions of lepromatous leprosy (subpolar type), there was nontender, non-fluctuant swelling of the right fifth and fourth fingers and left second finger. Skin over the right fifth finger showed sinus-like openings with associated purulent discharge. He also had swelling and ulceration of second left toe. Slit-skin smear (SSS) showed a bacterial index of 6+ from the ear lobes and cutaneous nodules, 4+ from the patch, and 3+ from normal skin. Modified Ziehl-Neelsen staining of the discharge extruding from the sinuses on the right fifth finger also showed abundant acid-fast bacilli. Radiography of the hands and feet showed lytic lesions in the distal epimetaphyseal region o proximal phalanx of the right fifth finger and left second finger and erosion of distal end of proximal phalanges of both second toes. Histopathological examination of biopsy specimen from the patch (back) showed features of lepromatous leprosy, and Fite-Faraco stain for tissue acid-fast bacteria (AFB) was strongly positive. Fine-needle-aspiration cytology (FNAC) from the lytic lesion in the bone also showed predominantly foamy macrophages with strongly positive staining for AFB with a few interspersed lymphocytes, epithelioid cells and Langhans giant cells. On the basis of these features, a clinical diagnosis of subpolar lepromatous leprosy with leprous osteitis was made. In today's clinical era of improved case detection and prompt treatment with effective multidrug regimens, advanced bone changes are rarely encountered. We describe this case of lepromatous leprosy that developed cavitating lesions of the phalanges of the hand, seen on x-ray as well-defined bone cyst and erosions.

Adult↗
PubMed2003

Extracellular matrix protein 1 gene (ECM1) mutations in lipoid proteinosis and genotype-phenotype correlation.

The autosomal recessive disorder lipoid proteinosis results from mutations in extracellular matrix protein 1 (ECM1), a glycoprotein expressed in several tissues (including skin) and composed of two alternatively spliced isoforms, ECM1a and ECM1b, the latter lacking exon 7 of this 10-exon gene (ECM1). To date, mutations that either affect ECM1a alone or perturb both ECM1 transcripts have been demonstrated in six cases. However, lipoid proteinosis is clinically heterogeneous with affected individuals displaying differing degrees of skin scarring and infiltration, variable signs of hoarseness and respiratory distress, and in some cases neurological abnormalities such as temporal lobe epilepsy. In this study, we sequenced ECM1 in 10 further unrelated patients with lipoid proteinosis to extend genotype-phenotype correlation and to add to the mutation database. We identified seven new homozygous nonsense or frameshift mutations: R53X (exon 3); 243delG (exon 4); 507delT (exon 6); 735delTG (exon 7); 785delA (exon 7); 892delC (exon 7) and 1190insC (exon 8), as well as two new compound heterozygous mutations: W160X/F167I (exon 6) and 542insAA/R243X (exons 6/7), none of which were found in controls. The mutation 507delT occurred in two unrelated subjects on different ECM1 haplotypes and may therefore represent a recurrent mutation in lipoid proteinosis. Taken with the previously documented mutations in ECM1, this study supports the view that exons 6 and 7 are the most common sites for ECM1 mutations in lipoid proteinosis. Clinically, it appears that mutations outside exon 7 are usually associated with a slightly more severe mucocutaneous lipoid proteinosis phenotype, but neurological features do not show any specific genotype-phenotype correlation.

Adolescent↗
PubMed2003

Cutaneous variant of angiokeratoma corporis diffusum.

A case of angiokeratoma corporis diffusum (ACD) involving the skin of a 22-year-old patient presenting with normal physical and mental development is reported. ACD presenting with skin lesions alone is a rare but specific clinical entity, which differs from hereditary sphingolipidoses such as Fabry's disease.

Adult↗
PubMed2003

Peculiar pattern of nail pigmentation following cyclophosphamide therapy.

Cyclophosphamide is one of several cancer chemotherapy agents that can cause nail hyperpigmentation. We report a patient who began to have an unusual form of nail pigmentation after 8 months of receiving monthly pulses of dexamethasone-cyclophosphamide. The patient developed nail pigmentation that started proximally and spread distally but involved only the nails of the thumb, index finger, and half the middle finger of both hands.

Cyclophosphamide↗
PubMed2003

Clinical profile of molluscum contagiosum in children versus adults.

Molluscum contagiosum mainly affects children, but in adults the advent of HIV has modified its clinical spectrum. Our study is designed to ascertain the clinical profile of molluscum contagiosum in children and adults and to discover the effects of underlying HIV infection. During the study period of September 2000 to June 2002, 150 cases of molluscum contagiosum are screened and recruited at the Department of Dermatology and STD, JIPMER, Pondicherry. These include 137 children (85 male, 52 female) and 13 adults (10 male, 3 female). In children molluscum contagiosum is most commonly seen in the 5-10-year age group (58 cases), followed by the 1-5-year age group (53 cases), the 10-14-year age group (23 cases), and the younger-than-1-year age group (3 cases). In adults molluscum contagiosum is most commonly seen in the 14-20-year age group, followed by 2 cases each in the age groups 20-26 years, 26-34 years, and 34-40 years. The male-to-female sex ratio was 1.6:1 in children and 3.3:1 in adults. In both children and adults the most common sites affected are the head and neck, followed by trunk, upper extremity, genitalia, and lower extremity. It appears that the distribution of molluscum in our patients is significantly affected by the high proportion of HIV-positive adults in our sample. Seropositivity for HIV by ELISA is found in 1 of 137 children and 8 of 13 adults. In these HIV-positive patients, 2 cases of giant molluscum and one furuncle-like presentation are seen.

Adolescent↗
PubMed2003

Lipoid proteinosis in two siblings: a report from south India.

A 6-year-old girl and her 9-year-old brother, born of nonconsanguineous parents, had hoarseness and multiple, asymptomatic, raised skin lesions present since childhood. On examination, both siblings had hoarseness and numerous skin-colored, waxy papules distributed over the forehead, face, neck, axilla, groin, and extremities. Acneiform (pocklike) scars were present on the face, trunk, and extremities. Eyelid beading (moniliform blepharosis) was present over bilateral upper and lower eyelids. The tongue, lips, and frenulum were thickened and infiltrated, and the patients were unable to protrude the tongue out of the mouth. The scalp had patchy alopecia. Histological examination of representative skin specimens (from both siblings) showed deposition of pink, amorphous material in the papillary dermis, around blood vessels, and around appendages. These deposits stained positive with Periodic Acid-Schiff stain, were diastase resistant, and were negative for Congo red, confirming our clinical diagnosis of lipoid proteinosis. Over 250 cases of this rare disorder have been described in the literature, but occurrence of lipoid proteinosis in siblings is rare.

Basement Membrane↗
PubMed2002

Cutaneous anthrax: an endemic outbreak in south India.

Although human anthrax has become rare, endemic outbreaks still occur in tropical countries, parts of South America and Europe. We report 23 cases of cutaneous anthrax due to an endemic outbreak of animal and human anthrax in South India. These patients were admitted to our hospital between July 1998 and July 2001. Children outnumbered adults and most of them had lesions on the exposed sites. The majority of patients reported the death of infected animals in the neighbourhood without any direct contact with dead animals. Hence, vector borne transmission was suspected in most of the cases. Diagnosis was confirmed by the presence of a typical ulcer with eschar, Gram-stained smears from ulcers and epidemiological evidence. Except for one fatal case, all patients responded to treatment.

Adolescent↗