- Email: [email protected]
Idiopathic familial onychomadesis
Idiopathic familial onychomadesis Anu Mehra, Richard J. Murphy, MD, and Barbara B. Wilson, MD Charlottesville, Virginia Onychomadesis is defined as spontaneous separation of the nail plate from the nail bed, beginning at its proximal end and resulting in the shedding of the nail. It has been associated with a variety of clinical settings, including systemic illnesses, generalized skin diseases, and drug therapies. There have been a small number of reports in the older literature of idiopathic onychomadesis occurring in a familial pattern. We report the case of a healthy 12-year-old girl and her mother, both of whom have experienced recurrent onychomadesis affecting multiple digits, and in the absence of any causal disease or medication. (J Am Acad Dermatol 2000;43:349-50.)
O
nychomadesis is a condition of the nail characterized by spontaneous separation of the nail plate from the nail bed beginning from the proximal nail end. It may be idiopathic or associated with disease or drug therapy. Onychomadesis has been reported in the literature to occur in association with a number of diseases and clinical settings, including systemic lupus erythematosus, pemphigus vulgaris, mycosis fungoides, alopecia areata universalis, epidermolysis bullosa, keratosis punctata palmaris et plantaris, thrombocytopenia, neurologic disease, peritoneal dialysis, penicillin anaphylaxis, chemotherapy, retinoids, and carbamazepine. We report a case of idiopathic onychomadesis not associated with any disease or drug therapy, and presenting in both a child and her mother.
CASE REPORT A healthy 12-year-old girl presented to the University of Virginia Dermatology clinic with a 4month history of a gradual, asymptomatic shedding of several finger and toenails. Her mother, in her late 30s, reported that she, too, had developed a similar nail abnormality at approximately the same age. She has experienced the periodic sloughing of her nails since that time. Both patients described loss of the
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Department of Dermatology, University of Virginia Health Sciences Center. Reprint requests: Barbara B. Wilson, MD, Department of Dermatology, Box 134, University of Virginia Health Sciences Center, Charlottesville, VA 22908. E-mail: Email: blw7u@avery. med.virginia.edu. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/101588 doi:10.1067/mjd.2000.101588
Fig 1. Onychomadesis of both third fingernails in 12-yearold girl.
nails in the proximal to distal direction, with subsequent regrowth of normal-appearing nails over 6 to 8 months. The medical histories of both mother and daughter are unremarkable, and neither has been on any regular medications. The daughter has no siblings, and no other family members are affected. Examination of the daughter’s nails revealed proximal separation of the nail plate from the nail bed occurring on the third digits of each hand (Fig 1). The fourth toenail of the left foot and the great toenail of the right foot also showed similar changes (Fig 2). There was no tenderness, inflammation, or evidence of trauma surrounding any of the involved nails. In addition, examination of the patient’s mother demonstrated proximal onycholysis with a single deep transverse groove of the right fourth toenail. Neither patient displayed any associated abnormalities of the skin, hair, or teeth.
DISCUSSION Onychomadesis is defined as spontaneous separation of the nail plate from the nail bed, beginning 349
350 Mehra, Murphy, and Wilson
Fig 2. Onychomadesis of right great toe. In both figures, new nail can be observed growing out behind old nail.
at its proximal end and resulting in shedding of the nail. The characteristic early finding is symmetric proximal onycholysis, as demonstrated in this case. Onychomadesis, when associated with systemic illness, generalized skin disease, or drug therapy may be considered a severe form of Beau’s lines.1 That is, temporary slowing or cessation of nail plate production, resulting in a transverse groove across the nail in patients with Beau’s lines, might persist long enough for a full thickness transverse split of the nail plate to occur. There are a number of such cases reported in the literature. Two adults with malignant lymphoma experienced reversible loss of their nails after beginning chemotherapy.2 Both had Hodgkin’s disease and developed onychomadesis of most of their nails within a few months of beginning treatment with cyclophosphamide, vincristine, and prednisone. The lost nails subsequently regrew in both persons. A 31-year-old man with a generalized partial seizure disorder developed onychomadesis of all of his finger and toenails after initiation of carbamazepine therapy.3 The drug was discontinued 4 months after it was started, when signs of nail dystrophy began to appear. One month after the drug therapy was stopped the patient lost all his nails; they grew back a few months later with normal texture, but with some mild discoloration. In an unusual presentation, onychomadesis of several fingernails on both hands was reported in a 24-year-old man with chronic renal failure on peritoneal dialysis 2 months after Acinetobacter peritonitis developed.4 He had spontaneous recovery of all fingernails affected 4 months after the condition developed. Onychomadesis has also been reported in a 69-year-old man with mycosis fungoides and in 3 adults with various neurovascular accidents.1,5
J AM ACAD DERMATOL AUGUST 2000
There is scant literature describing onychomadesis in children. A study of nail abnormalities in 272 children (ages 1-14) with alopecia areata identified 3 pediatric patients who demonstrated onychomadesis.6 In all 3 patients, the onset of the onychomadesis coincided with the acute development of alopecia areata universalis. All 20 nails in each patient were affected, and only 1 patient had regrowth of normal nails. The remaining 2 patients demonstrated severe pitting of the new nails: 1 child developed a peculiar form of punctate leukonychia characterized by numerous white dots arranged in a geometric pattern in all fingernails. In most cases of onychomadesis reported in the literature, the development of the nail abnormality has been preceded by the onset of a systemic illness or has coincided with use of a new drug therapy. In most of these cases, the condition is described as a temporary event with eventual regrowth of the nails. Idiopathic onychomadesis without associated disease or drug therapy, and occurring in a familial pattern, appears to be a much rarer entity; we could find no reports in the recent literature. There have been isolated reports of this condition in the older literature, occurring in families and suggesting a dominant inheritance pattern.7,8 Oliver8 described 3 families, each with a parent and child affected in a very similar way as our case. The duration of nail shedding in his patients varied from 2 to 35 years. We report another case of recurrent onychomadesis presenting in childhood and lasting months, as seen in our patient, and decades, as is the case with her mother, in the absence of underlying disease or drug therapy. We suggest calling this rare entity “idiopathic familial onychomadesis.” REFERENCES 1. Fleming CJ, Hunt MJ, Barnetson R. Mycosis fungoides with onychomadesis. Br J Dermatol 1996;135:1012. 2. Kochupillai V, Bhide NK, Prabhu M. Cancer chemotherapy and nail loss (onychomadesis). Acta Haematol 1983;70:137. 3. Mishra D, Gurmohan S, Pandey S. Possible carbamazepine induced reversible onychomadesis. Int J Dermatol 1989;28:4601. 4. Caputo R, Gelmetti C, Cambiaghi S. Severe self-healing nail dystrophy in a patient on peritoneal dialysis. Dermatology 1997; 195:274-5. 5. Baran R, Goettmann S. Nail bleeding associated with neurologic diseases: all that uncommon? Dermatology 1993;187:197-9. 6. Tosti A, Morelli R, Bardazzi F, Peluso AM. Prevalence of nail abnormalities in children with alopecia areata. Pediatr Dermatol 1994;11:112-5. 7. Baran R, Dawber RPR, editors. Diseases of the nail and their management. Oxford: Blackwell Scientific Publications; 1984. p. 52-53, 230, 238, 264. 8. Oliver WJ. Recurrent onychoptosis occurring as a familial disorder. Br J Dermatol Syph 1927;39:297-9.
O
nychomadesis is a condition of the nail characterized by spontaneous separation of the nail plate from the nail bed beginning from the proximal nail end. It may be idiopathic or associated with disease or drug therapy. Onychomadesis has been reported in the literature to occur in association with a number of diseases and clinical settings, including systemic lupus erythematosus, pemphigus vulgaris, mycosis fungoides, alopecia areata universalis, epidermolysis bullosa, keratosis punctata palmaris et plantaris, thrombocytopenia, neurologic disease, peritoneal dialysis, penicillin anaphylaxis, chemotherapy, retinoids, and carbamazepine. We report a case of idiopathic onychomadesis not associated with any disease or drug therapy, and presenting in both a child and her mother.
CASE REPORT A healthy 12-year-old girl presented to the University of Virginia Dermatology clinic with a 4month history of a gradual, asymptomatic shedding of several finger and toenails. Her mother, in her late 30s, reported that she, too, had developed a similar nail abnormality at approximately the same age. She has experienced the periodic sloughing of her nails since that time. Both patients described loss of the
This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology. From the Department of Dermatology, University of Virginia Health Sciences Center. Reprint requests: Barbara B. Wilson, MD, Department of Dermatology, Box 134, University of Virginia Health Sciences Center, Charlottesville, VA 22908. E-mail: Email: blw7u@avery. med.virginia.edu. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/4/101588 doi:10.1067/mjd.2000.101588
Fig 1. Onychomadesis of both third fingernails in 12-yearold girl.
nails in the proximal to distal direction, with subsequent regrowth of normal-appearing nails over 6 to 8 months. The medical histories of both mother and daughter are unremarkable, and neither has been on any regular medications. The daughter has no siblings, and no other family members are affected. Examination of the daughter’s nails revealed proximal separation of the nail plate from the nail bed occurring on the third digits of each hand (Fig 1). The fourth toenail of the left foot and the great toenail of the right foot also showed similar changes (Fig 2). There was no tenderness, inflammation, or evidence of trauma surrounding any of the involved nails. In addition, examination of the patient’s mother demonstrated proximal onycholysis with a single deep transverse groove of the right fourth toenail. Neither patient displayed any associated abnormalities of the skin, hair, or teeth.
DISCUSSION Onychomadesis is defined as spontaneous separation of the nail plate from the nail bed, beginning 349
350 Mehra, Murphy, and Wilson
Fig 2. Onychomadesis of right great toe. In both figures, new nail can be observed growing out behind old nail.
at its proximal end and resulting in shedding of the nail. The characteristic early finding is symmetric proximal onycholysis, as demonstrated in this case. Onychomadesis, when associated with systemic illness, generalized skin disease, or drug therapy may be considered a severe form of Beau’s lines.1 That is, temporary slowing or cessation of nail plate production, resulting in a transverse groove across the nail in patients with Beau’s lines, might persist long enough for a full thickness transverse split of the nail plate to occur. There are a number of such cases reported in the literature. Two adults with malignant lymphoma experienced reversible loss of their nails after beginning chemotherapy.2 Both had Hodgkin’s disease and developed onychomadesis of most of their nails within a few months of beginning treatment with cyclophosphamide, vincristine, and prednisone. The lost nails subsequently regrew in both persons. A 31-year-old man with a generalized partial seizure disorder developed onychomadesis of all of his finger and toenails after initiation of carbamazepine therapy.3 The drug was discontinued 4 months after it was started, when signs of nail dystrophy began to appear. One month after the drug therapy was stopped the patient lost all his nails; they grew back a few months later with normal texture, but with some mild discoloration. In an unusual presentation, onychomadesis of several fingernails on both hands was reported in a 24-year-old man with chronic renal failure on peritoneal dialysis 2 months after Acinetobacter peritonitis developed.4 He had spontaneous recovery of all fingernails affected 4 months after the condition developed. Onychomadesis has also been reported in a 69-year-old man with mycosis fungoides and in 3 adults with various neurovascular accidents.1,5
J AM ACAD DERMATOL AUGUST 2000
There is scant literature describing onychomadesis in children. A study of nail abnormalities in 272 children (ages 1-14) with alopecia areata identified 3 pediatric patients who demonstrated onychomadesis.6 In all 3 patients, the onset of the onychomadesis coincided with the acute development of alopecia areata universalis. All 20 nails in each patient were affected, and only 1 patient had regrowth of normal nails. The remaining 2 patients demonstrated severe pitting of the new nails: 1 child developed a peculiar form of punctate leukonychia characterized by numerous white dots arranged in a geometric pattern in all fingernails. In most cases of onychomadesis reported in the literature, the development of the nail abnormality has been preceded by the onset of a systemic illness or has coincided with use of a new drug therapy. In most of these cases, the condition is described as a temporary event with eventual regrowth of the nails. Idiopathic onychomadesis without associated disease or drug therapy, and occurring in a familial pattern, appears to be a much rarer entity; we could find no reports in the recent literature. There have been isolated reports of this condition in the older literature, occurring in families and suggesting a dominant inheritance pattern.7,8 Oliver8 described 3 families, each with a parent and child affected in a very similar way as our case. The duration of nail shedding in his patients varied from 2 to 35 years. We report another case of recurrent onychomadesis presenting in childhood and lasting months, as seen in our patient, and decades, as is the case with her mother, in the absence of underlying disease or drug therapy. We suggest calling this rare entity “idiopathic familial onychomadesis.” REFERENCES 1. Fleming CJ, Hunt MJ, Barnetson R. Mycosis fungoides with onychomadesis. Br J Dermatol 1996;135:1012. 2. Kochupillai V, Bhide NK, Prabhu M. Cancer chemotherapy and nail loss (onychomadesis). Acta Haematol 1983;70:137. 3. Mishra D, Gurmohan S, Pandey S. Possible carbamazepine induced reversible onychomadesis. Int J Dermatol 1989;28:4601. 4. Caputo R, Gelmetti C, Cambiaghi S. Severe self-healing nail dystrophy in a patient on peritoneal dialysis. Dermatology 1997; 195:274-5. 5. Baran R, Goettmann S. Nail bleeding associated with neurologic diseases: all that uncommon? Dermatology 1993;187:197-9. 6. Tosti A, Morelli R, Bardazzi F, Peluso AM. Prevalence of nail abnormalities in children with alopecia areata. Pediatr Dermatol 1994;11:112-5. 7. Baran R, Dawber RPR, editors. Diseases of the nail and their management. Oxford: Blackwell Scientific Publications; 1984. p. 52-53, 230, 238, 264. 8. Oliver WJ. Recurrent onychoptosis occurring as a familial disorder. Br J Dermatol Syph 1927;39:297-9.