|Year : 2016 | Volume
| Issue : 4 | Page : 288-289
Huriez syndrome with superadded dermatophyte infection
Trupti Surana1, Bela Padhiar1, Umesh Karia2, Purna Pandya1
1 Department of Dermatology, GMERS Medical College, Civil Hospital, Gandhinagar, Gujarat, India
2 Department of Dermatology, B. J. Medical College, Ahmedabad, Gujarat, India
|Date of Web Publication||5-Jul-2016|
Department of Dermatology, GMERS Medical College, Civil Hospital, Gandhinagar - 382 012, Gujarat
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Palmoplantarkeratodermas (PPKs) are a heterogeneous group of hereditary and acquired disorders with underlying gene defects, and characterized by hyperkeratosis of palms and soles with or without other ectodermal and systemic abnormalities. Huriez syndrome is a rare autosomal dominant transgradient type of PPK with high frequency of squamous cell carcinoma in the affected skin. We hereby describe a case of a very rare autosomal dominant PPK in a 40-year-old male patient presenting since birth with PPK extending onto the dorsal aspects of hands and feet with peeling of the skin. The complaints were associated with sclerodactyly, hyperhidrosis, and nail abnormalities. Also superadded dermatophyte infection was observed involving abdomen. No history of loss of any digit. No mucosal, dental, or any systemic involvement was present. No sign of malignancy was noted. Baseline investigations, including ultrasonography of abdomen were normal. Histological findings were nonspecific with only orthohyperkeratosis and acanthosis. Diagnosis was mainly done on clinical grounds. The patient is better with oral retinoids and topical emollients and keratolytics along with antifungal treatment for dermatophyte infection. He is under follow up.
Keywords: Dermatophyte, Huriez, scleroatrophy
|How to cite this article:|
Surana T, Padhiar B, Karia U, Pandya P. Huriez syndrome with superadded dermatophyte infection. Indian Dermatol Online J 2016;7:288-9
|How to cite this URL:|
Surana T, Padhiar B, Karia U, Pandya P. Huriez syndrome with superadded dermatophyte infection. Indian Dermatol Online J [serial online] 2016 [cited 2020 Jun 3];7:288-9. Available from: http://www.idoj.in/text.asp?2016/7/4/288/185466
| Introduction|| |
Huriez syndrome is a transgradient palmoplantar keratoderma (PPK) with sclerodactyly and nail abnormalities. One of its distinctive features is the risk of development of squamous cell carcinoma on the affected skin. We hereby present a case of a 40-year-old male patient with Huriez syndrome since birth that later developed dermatophyte infection with no sign of malignancy.
| Case Report|| |
A 40 yearold male patient presented to us with thickened scaly skin lesions on both the extremities since birth and erythematous scaly lesions over abdomen since 1 month. The lesions initially affected palms and soles and gradually extended proximally in years to involve extremities, especially extensor aspect. Dense palmar keratoderma also resulted in tightening of the digits with nail changes.
On examinationPPK [Figure 1] extending onto the dorsal aspects of hands and feet with peeling of the skin was observed. Scleroatrophy of the skin of the extremities were also noted. Sclerodactyly and also superadded dermatophyte infection was observed involving the abdomen [Figure 2]. Nail changes included subungual hyperkeratosis, hypercurvature, koilonychia, and onychorrhexis.
|Figure 2: Abdomen showing lesions of dermatophyte infection (tinea corporis) and sclerodactyly with nail changes|
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No history of loss of any digit, and no mucosal, dental, or any other systemic involvement was present. Baseline investigations were normal, where as KOH examination of abdominal lesions was positive for fungal elements. Histological findings were nonspecific with only orthohyperkeratosis and acanthosis. Diagnosis was mainly done on clinical grounds as Huriez syndrome.
The patient is better with oral retinoids and topical emollients and keratolytics along with antifungal treatment for dermatophyte infection. He is under follow up.
| Discussion|| |
Huriez syndrome is a very rare autosomal dominant transgradient scleroatrophic keratodermal genodermatosis of the extremities first described by Huriez et al. in 1968. A mutation in the gene mapped to chromosome 4q23 is implicated. This condition is characterized by diffuse PPK with atrophic skin over the dorsal aspect of the extremities, sclerodactyly, nail abnormalities, and high frequency of squamous cell carcinoma (SCC) in the affected skin.,,
In 1960s, Claude Huriez and colleagues reported two families from Northern France with a rare genodermatosis later known as Huriez syndrome. It is a cancerprone genodermatosis. There are very few cases of this syndrome reported in the literature since then. Man et al. reported a study on a patient having Huriez syndrome with SCC. Riggio et al. and Dumont et al. also reported the association of SCC with Huriez syndrome in their patients along with surgical approach and possible ways of surgical management in such cases.
Our case presented with almost all the features of this condition since birth but no signs of SCC. The patient rather presented with extensive dermatophyte infection involving abdomen. Fungal superinfection has been known to be associated with other keratodermas but never been reported in cases with Huriez syndrome.
Histopathologic findings are nonspecific and diagnosis is mainly done clinically. Treatment includes systemic retinoids with topical keratolytics with mixed results. Other complaints are treated symptomatically. SCC, if developed, may need surgical approach.,
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]