Unna Thost syndrome: a case report

Geetha M., Rajeswari Bhat, Kiran B., Madhusmitha Jena, Libni D. Angel


Unna thost syndrome is Palmo Plantar Keratoderma (PPK) of diffuse non epidermolytic type inherited in autosomal dominant fashion. Authors had a seven-year-old boy born to second degree consanguineous parents had palmoplantar keratoderma with hyperhidrosis with positive family history. He was promptly diagnosed and treated with acitretin and showed improvement.


Hyperhidrosis, Epidermolysis, Palmo plantar keratoderma, Unna Thost Syndrome, Vorner

Full Text:



Karadag AS, Simsek GG. A family of unna-thost disease with one of them showing findings of epidermolytic keratoderma. Indian J Dermatol Venereol Leprol. 2010;76:85.

Dhillon KS, Agarwal V, Singh T, Sharma D, Khan A, Srivastava R, Srivastava S, Yadav S. Unna-Thost type of palmoplantar keratoderma: a case report. Int J Adv Med. 2015;2:288-90.

Thost A. Ueber Erbliche Ichthyosis Palmaris et Plantaris Cornea. Dissertation. Heidelberg, Germany: University of Heidelberg; 1880.

Hinterberger L, Pföhler C, Vogt T, Müller CSL. Diffuse epidermolytic palmoplantar keratoderma (Unna-Thost-). BMJ Case Rep. 2012;10.1136/bcr-2012-006443.

Costa OG. Acroceratoses (ceratodermiaspalmo-plantares). Tese. Belo Horizonte: Minas Gerais (MG); 1962.

Krol AL. Keratodermas. In: Bolognia JL, Jorizzo JL, Rapini R, editors. Dermatology. 2 nd ed. London: Mosby; 2008:777-790.

Cesarini LV, Pegas JR, Reis VM, Müller H, Oliveira MA, Pires MC. Unna-Thost type palmoplantar keratoderma associated with pseudoainhum: a case report. An Bras Dermatol. 2004;79(1):61-7.

Jones BJ, Hutchinson PE. A family with palmoplantar epidermolytic hyperkeratosis. Clin Exp Dermatol. 1989;14:313-6.

Kόster W, Becker A. Indication for the identity of palmoplantar keratoderma type Unna-Thost with type Vorner-Thost's family revisited 110 years later. Acta Derm Venereol. 1992;72:120-2.

Magro CM, Baden LA, Crowson AN, Bowden PE, Baden HP. A novel nonepidermolytic palmoplantar keratoderma: a clinical and histopathologic study of six cases. J Am Acad Dermatol. 1997;37:27-33.

Gulati S, Thappa DM, Garg BR. Hereditary palmoplantar keratodermas in South India. J Dermatol. 1997;24:765-8.

Christiano AM. Frontiers in keratodermas: pushing the envelope. Trends Genetic. 1997;13:227-33.

Stevens HP, Kelsell DP, Bryant SP, Bishop DT, Spurr NK, Weissenbach J, et al. Linkage of an American pedigree with palmoplantar keratoderma and malignancy (palmoplantar ectodermal dysplasia type III) to 17q24: literature survey and proposed updated classification of the keratodermas. Arch Dermatol. 1996;132(6):640-51.

Burgdorf WHC. Ainhume Pseudoainhum. In: Fitzpatrick TB, Freedberg IM, Eisen AZ, Wolff K, eds. Dermatology in General Medicine. 5th ed. New York, Mc Graw-Hill; 1999: 1208-1209.

Rickman L, Šimrak D, Stevens HP, Hunt DM, King IA, Bryant SP, et al. N-terminal delection in a desmossomal cadherin causes the autossomal dominant skin disease striate palmoplantar keratoderma. Hum Mol Gen. 1999;8:971-3.

Ramesh V, Misra RS, Mahaur BS. Pseudoainhum in porokeratosis of Mibelli. Cutis. 1992;49(2):129-30.

Park BS, Cho HK, Youn JI, Chung JH. Pseudoainhum associated with linear scleroderma (letter). Arch Dermatol. 1996;132(12):1520-1.

Tajima S, Suzuki Y, Inazumi T. A case of atypical localized scleroderma presenting with pseudoainhum: treatment with tranilast an anti-fibrotic agent (letter). Acta Derm Venereol. 1996;76(2):162.

McLaurin CI. Psoriasis presenting with peudoainhum. J Am Acad Dermatol. 1982;7(1):130-2.

Kansky A, Stanimirovic A, Juzbasic BA. Isolated cases of palmo plantar Keratoderma, Unna-Thost type. Cutis. 1992;49:406-8.