Indian Journal of Dermatology
: 2021  |  Volume : 66  |  Issue : 4  |  Page : 424--425

Hereditary Distal Onycholysis: A New Affected Family

Fernando Garcia-Souto, Isabel Maria Coronel-Pérez, Sandra Cases-Mérida, Jeronimo Escudero-Ordoñez 
 Deparment of Dermatology, Valme University Hospital, Seville, Spain

Correspondence Address:
Fernando Garcia-Souto
Deparment of Dermatology, Valme University Hospital, Seville

How to cite this article:
Garcia-Souto F, Coronel-Pérez IM, Cases-Mérida S, Escudero-Ordoñez J. Hereditary Distal Onycholysis: A New Affected Family.Indian J Dermatol 2021;66:424-425

How to cite this URL:
Garcia-Souto F, Coronel-Pérez IM, Cases-Mérida S, Escudero-Ordoñez J. Hereditary Distal Onycholysis: A New Affected Family. Indian J Dermatol [serial online] 2021 [cited 2023 Feb 6 ];66:424-425
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Full Text

Inherited nail disorders are a rare and heterogeneous group of diseases. Hereditary distal onycholysis (HDO) is classified into the group of isolated hereditary nail disorders and is characterized by a slow growth of nails, thick and hard nails, and a straight or concave proximal edge of detachment.[1]

We report an 18-year-old patient who has suffered from onycholysis of all the nails since birth. He has no relevant medical history. Clinical examination revealed nails were smooth, thick, and showed a concave proximal edge of detachment [Figure 1]. Apart from cosmetic disability, our patient complained about the difficulty to cut them and a marked sensibility to cold, particularly on the hands and feet and especially after exposure to cold water. No skin changes were noticed so Raynaud's phenomenon was discarded. The patient did not complain of hyperhidrosis. Examination of skin, teeth, hair, and mucosae proved no abnormalities. Blood analysis and X-rays of hands and feet were normal. Nails evaluation with direct microscopy as well as mycological cultures were negative. Several relatives showed similar abnormalities [Figure 2]. The inheritance appeared to be autosomal dominant [Figure 3].{Figure 1}{Figure 2}{Figure 3}

The features of the family we describe are comparable to the families reported with HDO. Shulze (1966) described a family where several members showed onycholysis since childhood.[2] It was associated with a slow nail growth, scleronychia, and absent lunulae. Hypersensitivity to cold and palmoplantar hyperhidrosis were frequently linked symptoms. No changes in the skin, teeth, hair, or mucosae were seen. Subsequently, Burg (1975) reported a family with similar characteristics.[3] Affected individuals did not present palmoplantar hyperhidrosis or sensitivity to cold, but itchy fingers. Finally, Bazex et al.[4] described a new family with onycholysis of all nails in several members, decreased nail growth, and scleronychia. In addition, all affected members except one showed palmoplantar hyperhidrosis and sensitivity to cold.

There are various hereditary disorders that could resemble the nails of the family we describe.[5] Hidrotic ectodermal dysplasia is an autosomal dominant disorder characterized by nails that gradually become thickened, often with onycholysis, together with hypotrichosis and palmoplantar keratoderma. Pachyonychia congenita is an autosomal dominant inherited disease associated with nail thickening, more marked in toenails, and painful plantar keratoderma. The main challenge is to differentiate HDO from other isolated hereditary nail disorders. A decreased growth rate, thick nails, and a straight or concave proximal edge of detachment since childhood, in patients with a positive familiar history highly suggest the diagnosis. In addition, cold sensitivity and palmoplantar hyperhidrosis are key features that may be associated. Excessive sensitivity to cold expresses an exaggerated response with pain and tenderness when affected patients are exposed to a cold environment. In contrast to Raynaud phenomenon, affected patients do not manifest skin color changes.

Diagnosis of HDO is clinical. An appropriate anamnesis including past medications and family history in combination with physical examination with emphasis to ectodermal structures are helpful in diagnosing HDO. None effective therapy has been reported and nail distrophy may persist with no or little improvement.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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1Khan S, Basit S, Habib R, Kamal A, Muhammad N, Ahmad W. Genetics of human isolated hereditary nail disorders. Br J Dermatol 2015;173:922-9.
2Schulze HD. [Hereditary partial onycholysis with scleronychia]. Dermatol Wochenschr 1966;152:766-75.
3Burg G. [Procceedings: Hereditary onycholysis mit scleronychia]. Hautarzt 1975;26:386.
4Bazex J, Baran R, Monbrun F, Grigorieff-Larrue N, Marguery MC. Hereditary distal onycholysis-a case report. Clin Exp Dermatol 1990;15:146-8.
5Zaias N, Escovar SX, Zaiac MN. Finger and toenail onycholysis. J Eur Acad Dermatol Venereol 2015;29:848-853.