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Year : 1996  |  Volume : 62  |  Issue : 4  |  Page : 254-255

Mutilating keratoderma with deaf-mutism

Correspondence Address:
S Rastogi

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Source of Support: None, Conflict of Interest: None

PMID: 20948071

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A 30 year old woman presented with typical lesions of mutilating keratoderma. The patient was deaf and dumb. Hyperkeratosis of palms and soles was present since infancy. Constriction of digits started by the age of 5 years. The clinical diagnosis was supported by histopathological examination.

Keywords: Keratoderma, Mutilating, Deaf-mutism

How to cite this article:
Rastogi S, Kumar P, Mukhija R D. Mutilating keratoderma with deaf-mutism. Indian J Dermatol Venereol Leprol 1996;62:254-5

How to cite this URL:
Rastogi S, Kumar P, Mukhija R D. Mutilating keratoderma with deaf-mutism. Indian J Dermatol Venereol Leprol [serial online] 1996 [cited 2019 Sep 15];62:254-5. Available from: http://www.ijdvl.com/text.asp?1996/62/4/254/4408

  Introduction Top

Mutilating keratoderma (Vohwinkel's syndrome) is a rare syndrome of hereditary palmo-plantar keratoderma. It is inherited in an autosomal dominant fashion. The keratoderma begins in infancy and is diffuse but "honeycombed" by small depressions. Later on constricting fibrous bands lead to progressive strangulation of digits. Star shaped keratoses on the dorsa of fingers and knees are distinctive.[1] Associated features may include alopecia, deafness, spastic paraplegia, myopathy and ichthyosiform dermatoses.[2] Mental retardation, bony changes and congenital deformities have also been reported in association with mutilating keratoderma.[3],[4] We report a case of mutilating keratoderma with deaf-mutism.

  Case Report Top

A 30-year-old woman developed hyperkeratosis of palms and soles extending to distal part of forearm and legs respectively. The lesion began in infancy with thickening of palms and soles [Figure - 1], but since the aqe of 5 years the disease rapidly progressed. Fibrous bands around fingers mostly on the little fingers were also noticed. These bands led to the constriction of digits. In the last 5 years, thickening had extended to dorsum of hands, feet and flexural aspects of both forearms. Star fish shaped keratosis on the knuckles along with isolated keratotic warty papules on the dorsum of elbows were also seen.

The patient was deaf and dumb since birth. Hair were sparse, thin and lustreless. No other abnormality could be detected. Detailed family history did not reveal any other family member affected with similar disease.

Histology of the palmar lesion showed changes of keratoderma ie, hyperkeratosis, hypergranulosis and moderate acanthosis along with mild perivascular infiltrate of mononuclear cells.

  Discussion Top

A few cases of mutilating keratoderma have been described in the literature but extension of keratoderma into adjacent areas of dorsum of hands and feet and forearm has not been reported earlier. Diffuse thickening of palms and soles along with constriction bands on little fingers of both hands suggested mutilating keratoderma. Absence of erythema and scaling on palms and soles ruled out  Mal de Meleda More Details.

  References Top

1.Chang Sing Pang AF, Oranje AP, Vuzevki VD, et al. Successful treatment of keratoderma heredetaria mutilans with an aromatic retinoid. Arch Dermatol 1981;117:225-8.  Back to cited text no. 1  [PUBMED]  
2.Camisa C, Rossana C. Variant of keratoderma heredetaria mutilans (Vohwinkel's syndrome): treatment with orally administered isotretinoin. Arch Dermatol 1984;120:1328-9.  Back to cited text no. 2  [PUBMED]  
3.Krishnaram AS, Reddy BSN, Garg BR, et al. Vohwinkel's disease. Ind J Dermatol Venereol Leprol 1984;50:115-8.  Back to cited text no. 3    
4.Chakravorty N, Gupta CM. Mutilating palmoplantar keratoderma with congenital deformities. Ind J Dermatol Venereol Leprol 1987;53:309.  Back to cited text no. 4    


[Figure - 1]


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