Familial median canaliform nail dystrophy

Median canaliform nail dystrophy typically appears as a central nail groove, beginning at or distal to the proximal nail fold, from which small lateral fissures may be found. Although the onset of this nail dystrophy has occasionally been associated with either prior local trauma or the initiation of treatment with isotretinoin, the etiology of this condition remains elusive for most affected individuals. We describe a man with median canaliform nail dystrophy whose brother and mother had the same nail dystrophy, review the other reported cases, and discuss the differential diagnosis of this nail disorder.     

Nail cosmetics in nail disorders

The clinical features of nail dystrophies depend on the part of the nail that has been damaged. Due to the important functions of fingernails and toenails, any abnormality of the nail causes impaired function of the hand or foot. Moreover, the aesthetic aspect of the nail may affect employability, self-esteem, and interaction with other people. Because the nails are often difficult to treat, cosmetology may be an effective support to medical treatment. Nail cosmetics may help the patient to cope with his or her nail dystrophy while waiting for treatment to show its efficacy. It may also be the only choice to hide nail dystrophy where the nail is irreversibly damaged. Nail cosmetics may also function at treatment for onychtillomania, nail biting, and nail ingrowing.     

Trachyonychia (rough nails)

The term trachyonychia is a useful one to describe roughening of the surface of many nails due to various causes. Psoriasis and alopecia areata are responsible for some cases. Lichen planus can roughen nails but the change is usually limited to a few nails and accompanied by discomfort. In many cases no cause can be found and these cases are somewhat arbitrarily listed either as twenty nail dystrophy of childhood or severe nail dystrophy.     

Twenty-nail dystrophy of childhood—two cases in one family

Two siblings suffering from the so-called twenty-nail dystrophy are described. This syndrome is characterized by the appearance in early childhood of changes affecting all the nails: moderate koilonychia, marked accentuation of longtitudinal ridges and loss of lustre of the nail plates. These two cases, the first reported in the same family, confirm that the twenty-nail syndrome is a clinical entity which can be differentiated from other diseases involving all twenty nail plates.     

An association of twenty-nail dystrophy with vitiligo

A rare association of twenty-nail dystrophy with segmental vitiligo is described in two patients. Vitiligo preceded the nail dystrophy. In both cases, all twenty nails were uniformly affected with the nail plates showing longitudinal striations and loss of luster. Longitudinal nail biopsy revealed a histological picture suggestive of eczematous changes and lichen planus respectively. Intramatrix injections of triamcinolone acetonide into the proximal and lateral nail folds were administered with considerable improvement.     

Nail dystrophy in congenital cutaneous candidiasis

Congenital cutaneous candidiasis (CCC) is usually a benign condition characterized by various skin manifestations and is rarely associated with nail changes. We report a premature infant with CCC who developed dystrophy of all 20 nails at about 1 month of age. Nail dystrophy due to Candida albicans in the young infant may be differentiated from other congenital or hereditary nail malformations by appearance, recovery of the organism in nail culture, and complete resolution over a period of several months.     

Atypical crusted scabies in an Iranian man

Crusted scabies has been defined as a scabies-induced psoriasiform dermatosis on the hands and feet with dystrophy of the nails and an erythematous scaling eruption that may be generalized. This case report demonstrates a patient who presented with widespread psoriasiform plaques that were teeming with scabies mites and eggs, but who had absence of nail and palmar involvement. While patients with crusted scabies may have nail dystrophy and involvement of the palms, this involvement is not necessary.     

Twenty-nail dystrophy in a girl with incontinentia pigmenti

A 9-year-old girl with incontinentia pigmenti (IP) diagnosed soon after birth subsequently developed 20-nail dystrophy at the age of 3 years. This persists unchanged 6 years later, and she has no other evidence of any other cutaneous disease, nor a family history of IP or nail dystrophy. Twenty-nail dystrophy has been associated with a number of diseases including eczema, lichen planus, alopecia areata and psoriasis and a number of other congenital associations, but the association with IP appears to be unique.     

几种甲病诊疗的思考

【摘要】 甲病种类繁多,近年其诊断与治疗取得一些进展,但仍面临诸多挑战,如纵行黑甲、罕见的甲单元特异性肿瘤、甲营养不良等。纵行黑甲存在黑素瘤可能,而活检与手术本身是肢端黑素瘤的诱发因素,因此是否手术以及如何把握手术原则很重要。此外,在诊疗过程中应结合年龄、临床评估、皮肤镜表现以及病理检查进行评估,最终做到恶性黑甲不漏诊,良性黑甲不畸形。临床上对特发于甲单元的少见肿瘤临床表现和病理学特征的总结较少,诊断难度高,尤其甲母质细胞癌和甲鞘癌属恶性肿瘤,损害大,本文重点描述从而提醒临床医生避免漏诊。甲营养不良病因复杂,由炎症性疾病导致的甲营养不良可选用局部或系统药物治疗,而由遗传、足趾畸形、行走姿势不当、机械性损伤、甲沟慢性炎症导致的各种甲畸形则需进行物理或手术矫正。本文将对这几种甲病诊疗中存在的挑战和相应对策进行探讨。     

Bowen's disease of the nail bed

A 77-year-old man presented with a 2-year history of left thumbnail dystrophy, purulent discharge and aching pain. There was no history of trauma. On examination there was a linear area of nail dystrophy and dyschromia of the nail bed. The nail plate was thinned proximally. No other digits were affected. There was no lymphadenopathy. X-ray of the digit was normal. Longitudinal nail biopsy revealed Bowen's disease along the length of the nail bed and nail matrix specimen. After avulsion of the nail plate, the nail matrix and tumour were fully excised. The defect was repaired with a split-skin graft. His symptoms were relieved.     

Twenty nail dystrophy in vitiligo

Twenty nail dystrophy (TND) is a manifestation of several dermatoses and its association with vitiligo is rare. It may be consequent upon a common autoimmune insult to the melanocytes and the nail matrix. We report a patient of acrofacial vitiligo who developed TND. Nail matrix biopsy revealed focal lichenoid reaction and chronic inflammatory infiltrate in the dermal papillae and around blood vessels. Hematological, biochemical and serological investigations showed no evidence of other autoimmune diseases.     

20甲营养不良患者的甲病理诊断分析

目的探讨临床诊断的20甲营养不良患者的病理改变特征,明确病因,指导治疗。方法 23例20甲营养不良患者,临床表现为获得性单纯性20甲损害,甲板表面有许多纵嵴,失去光泽不透明,似砂纸样外观,甲板增厚或变薄,真菌检查阴性。采用纵行甲活检手术方法获取甲组织标本,观察甲单位病理改变。结果 23例患者中病理改变符合甲银屑病4例,甲扁平苔藓3例,甲非特异性湿疹样变16例。发病年龄以青中年发病人数占绝对优势(73.91%);病程<2年者占21.74%,2～10年者占69.57%,>10年者占8.70%。结论甲单位病理检查能对20甲营养不良患者进行病理诊断,其病因包括甲银屑病、甲扁平苔藓以及甲非特异性湿疹样变,为下一步选择治疗药物提供重要依据。     

Lesiones melanocíticas subungueales: claves clínico-patológicas y técnicas de biopsia

Both dermatologists and pathologists sometimes find daunting the evaluation of melanonychia (especially subungual melanocytic lesions) because of the fear of performing nail surgery due to the risk of dystrophy, difficulties processing and interpreting nail biopsy specimens, and a general lack of experience in the field. Nevertheless, mastery of nail biopsy techniques, correct processing and orientation of specimens, and familiarity with the histologic particularities of the nail apparatus can attenuate the undoubted complexity and facilitate the tasks involved. Longitudinal excision is the biopsy technique that ensures the simplest histologic interpretation, and it is associated with a low risk of nail dystrophy when performed correctly. Clinical and epidemiological data are crucial. Subungual melanoma in childhood, for instance, is very rare and even lesions with atypical clinical and/or histologic features are probably benign. The presence of suprabasal melanocytes and other findings that would suggest malignancy at other sites are considered normal in the nail apparatus. Subungual melanoma shows a lentiginous pattern in the early stages of disease, and detection of an inflammatory infiltrate accompanying atypical lentiginous subungual lesions would appear to be one of the first diagnostic findings.     

Intralesional triamcinolone injections for the treatment of nail dystrophy: A case series

Nail dystrophy can be temporary or permanent and affects quality of life for many patients. It can be secondary to an inflammatory condition; however, many cases are idiopathic. Despite many efforts, there is no promising treatment. Local steroid injection is one of the standard therapies offered for nail dystrophy, but there have been few formal open trials or case reports of its efficacy. This study investigated the impact of intralesional triamcinolone injections on the management and safety of nail dystrophy. Overall, 12 patients with 55 nails affected by nail dystrophy were enrolled. All lesions were injected with triamcinolone acetonide (2.5 mg/mL, 0.1 cc) through the proximal nail fold with a 30 g needle. Injections were administered every 4 weeks. Efficacy was retrospectively evaluated using physician's global assessment of clinical photographs graded on a 5‐point scale as 0, no improvement; 1, slight improvement; 2, moderate improvement; 3, marked improvement; and 4, almost resolved. All adverse events that occurred during treatment were recorded. All patients presented with slight improvement of nail dystrophy after 1 to 3 months. The treatment durations varied from 6 to 12 months (mean, 8.58 months). The average time to observed therapeutic effect was 1.91 months after first treatment. The mean outcome assessment score was 2.8 points, with two patients each showing slight and moderate improvement and five showing marked improvement; in three, the dystrophy was almost resolved. Side effects of this regimen were minimal. Intralesional triamcinolone injections are an effective and safe method for the treatment of nail dystrophy.     

Dermatopathia pigmentosa reticularis

An unusual dyschromatosis associated with nail dystrophy and partial alopecia is described in a 16-year-old girl. The condition has been previously described as dermatopathia pigmentosa reticularis and is contrasted with other similar disorders.     

Polycythaemia rubra vera with lamellar dystrophy of the nails: a report of two cases

Two cases of polycythaemia rubra vera with lamellar dystrophy of the nails are described. In one case the nail changes improved markedly when the polycythaemia was treated, but the nail dystrophy has persisted in the second patient.     

The ‘PDA Nail’

All-in-one devices with mobile phone, web browser, and organizer are now owned by over 6 million people and their popularity is increasing. These devices are often called personal digital assistants (PDAs) or ‘BlackBerry®’ devices, after a popular brand name of these appliances. The use of PDAs is associated with exposure of distal thumbs and nails to repeated pressure with a frequency of up to a few thousand times per hour and several tens of thousands of times per day. We describe two cases of traumatic thumb nail dystrophy associated with using a PDA keyboard for several hours per day. Both patients developed median nail plate dystrophy after 4–8 months of habitual PDA use. One patient also developed thumb nail psoriasis and paronychia. All symptoms resolved a few months after discontinuing PDA use. Analysis of nail biomechanics, performed by using a finite element fingertip model, showed that maximal stress reaches approximately 3 MPa and appears near the root on the internal surface of the nail, while it reaches approximately 2 MPa and appears around one-third from the root on the outside surface. In conclusion, biomechanical stress resulting from overuse of PDAs may result in various types of nail dystrophy. We suggest the general term ‘PDA nails’ for these nail abnormalities.     

Giant onychomatricoma: report of two cases with rare clinical presentation

BACKGROUND: Onychomatricoma was reported for the first time by Baran and Kint as a rare nail matrix tumor with specific clinical and histologic features, including a macroscopic appearance of filiform digitations originating from the nail matrix which are inserted in the nail plate.(1) The appearance of the lesion may resemble that of an "anemone." All previous reports have been mostly from Europe, with only one case from North America. These are the first case reports from Mexico. They show rare clinical characteristics, in particular tumor involving the entire nail matrix. METHODS: Two cases are presented: Case 1, a 59-year-old man with a 2-year history of deformity of the entire nail apparatus of the right thumb, clinically suggestive of onychomatricoma; Case 2, a 45-year-old woman with a 3-year history of gradual nail dystrophy, initially misdiagnosed as onychomycosis and treated unsuccessfully for several months with antimycotics by a general physician. In both cases, the entire nail plate was affected and, because of the clinical appearance, nail exploration and excisional biopsy of the tumors at the nail matrix were performed. RESULTS: During nail matrix surgical exploration, including avulsion of the nail plate, characteristic digitiform projections that were firmly attached to the nail plate were observed. In both cases, the entire nail matrix was affected, producing a giant form of onychomatricoma. Hematoxylin and eosin stain showed characteristic filiform projections that corresponded to the clinical appearance, with elongated epithelium from the nail matrix and fibromyxoid stroma with multiple basophilic cells, and typical clefts in between. CONCLUSIONS: Onychomatricoma has a classical clinical appearance; however, it is difficult to identify, as it is not until surgery, when the typical filiform projections are more visible, that the diagnosis can be made. Onychomatricoma is a rare tumor. The involvement of the entire nail matrix, with secondary nail dystrophy, in both cases presented here makes them interesting case reports of this unusual form of tumor.