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Lewandowsky and lutz dysplasia: report of two cases in a family.

Bhutoria B, Shome K, Ghosh S, Bose K, Datta C, Bhattacharya S - Indian J Dermatol (2011)

Bottom Line: Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail.Cases were followed up for 7 years.This study reveals that the disease progression is variable among two individuals of the same family.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Burdwan Medical College, Burdwan, West Bengal, India.

ABSTRACT
Lewandowsky and Lutz dysplasia, also known as epidermodysplasia verruciformis (EV), is an inherited disorder in which there is widespread and persistent infection with human papilloma virus, defect in cell-mediated immunity and propensity for malignant transformation. Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail. Cases were followed up for 7 years. Detailed history, clinical features and investigations, including skin biopsy from different sites at different times, were examined. Generalized pityriasis versicolor like hypopigmented lesions in both the cases, together with variable pigmented nodular actinic keratosis like lesions on sun-exposed areas, were present. Multiple skin biopsies done from various sites on different occasions revealed features typical of EV along with lesions, i.e., actinic keratosis, Bowen's disease, basal and squamous cell carcinoma, in the elder sibling. However, skin biopsy of the other sibling showed features of EV and seborrheic keratosis only till date. This study reveals that the disease progression is variable among two individuals of the same family. Malignant lesions were seen only on sun-exposed areas and may be associated with other skin lesions or infections such as angiokeratoma of Fordyce and tinea cruris, as seen in this report.

No MeSH data available.


Related in: MedlinePlus

Photograph of younger sibling showing hypopigmented lesions all over the body and two scaly erythematous lesions over the chest wall
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Figure 2: Photograph of younger sibling showing hypopigmented lesions all over the body and two scaly erythematous lesions over the chest wall

Mentions: Two brothers [Figures 1 and 2], first presented at 16 and 18 years of age, respectively, came with a history of multiple, discreet, hypopigmented lesions all over the body since 8 years of age. Elder brother was also asthmatic since childhood. They gave history of consanguineous parentage (uncle–niece). Pedigree chart is shown in Figure 3. None of the living family members (total 18) had this disease.


Lewandowsky and lutz dysplasia: report of two cases in a family.

Bhutoria B, Shome K, Ghosh S, Bose K, Datta C, Bhattacharya S - Indian J Dermatol (2011)

Photograph of younger sibling showing hypopigmented lesions all over the body and two scaly erythematous lesions over the chest wall
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108519&req=5

Figure 2: Photograph of younger sibling showing hypopigmented lesions all over the body and two scaly erythematous lesions over the chest wall
Mentions: Two brothers [Figures 1 and 2], first presented at 16 and 18 years of age, respectively, came with a history of multiple, discreet, hypopigmented lesions all over the body since 8 years of age. Elder brother was also asthmatic since childhood. They gave history of consanguineous parentage (uncle–niece). Pedigree chart is shown in Figure 3. None of the living family members (total 18) had this disease.

Bottom Line: Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail.Cases were followed up for 7 years.This study reveals that the disease progression is variable among two individuals of the same family.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Burdwan Medical College, Burdwan, West Bengal, India.

ABSTRACT
Lewandowsky and Lutz dysplasia, also known as epidermodysplasia verruciformis (EV), is an inherited disorder in which there is widespread and persistent infection with human papilloma virus, defect in cell-mediated immunity and propensity for malignant transformation. Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail. Cases were followed up for 7 years. Detailed history, clinical features and investigations, including skin biopsy from different sites at different times, were examined. Generalized pityriasis versicolor like hypopigmented lesions in both the cases, together with variable pigmented nodular actinic keratosis like lesions on sun-exposed areas, were present. Multiple skin biopsies done from various sites on different occasions revealed features typical of EV along with lesions, i.e., actinic keratosis, Bowen's disease, basal and squamous cell carcinoma, in the elder sibling. However, skin biopsy of the other sibling showed features of EV and seborrheic keratosis only till date. This study reveals that the disease progression is variable among two individuals of the same family. Malignant lesions were seen only on sun-exposed areas and may be associated with other skin lesions or infections such as angiokeratoma of Fordyce and tinea cruris, as seen in this report.

No MeSH data available.


Related in: MedlinePlus