nonsyndromic congenital nail disorder 7

Nonsyndromic Congenital Nail Disorder 7 (NDNC7) Description

Nonsyndromic Congenital Nail Disorder 7, also known as Isolated Congenital Onychodysplasia, is a rare genetic condition characterized by abnormalities in the formation of the nail plates. The disorder is marked by:

• Longitudinal streaks: Thin lines or ridges that run along the length of the nails
• Thinning: The nail plate becomes thinner and more fragile
• Impaired formation: The nail plates do not form properly, leading to increased vulnerability of the free nail margins

This condition typically affects all fingernails and toenails, with changes in the thumb and big toe nails being particularly prominent. The exact cause of NDNC7 is unknown, but it is believed to be a genetic disorder.

References:

• [1] Isolated congenital onychodysplasia, here referred to as nonsyndromic congenital nail disorder-7 (NDNC7), is characterized by longitudinal streaks, thinning, and impaired formation of the nail plates leading to increased vulnerability of the free nail margins. [1]
• [3] Nail disorder, nonsyndromic congenital, 7 is a condition characterized by longitudinal streaks, thinning, and impaired formation of nail plates, leading to increased vulnerability of free nail margins. [3]
• [10] Isolated congenital onychodysplasia, here referred to as nonsyndromic congenital nail disorder-7 (NDNC7), is characterized by longitudinal streaks, thinning, and impaired formation of the nail plates leading to increased vulnerability of the free nail margins. [10]
• [13] Isolated congenital onychodysplasia (NDNC7), is characterized by longitudinal striation, thinning and a disturbed formation of the nail plates. In most cases, all fingernails and toenails are affected, with changes in the thumb and big toe nails being particularly prominent. [13]

Based on the provided context, here are the signs and symptoms of nonsyndromic congenital nail disorder 7:

• Excessive longitudinal striations: This is a common feature of nail dysplasia, which is an idiopathic nail dystrophy that begins in early childhood [2].
• Loss of nail luster: Individuals with this condition may experience a loss of shine or luster on their nails [2].
• Claw-shaped, hyperpigmented nails: Some individuals with nonsyndromic congenital nail disorder 7 may have claw-shaped nails that are hyperpigmented (darker than normal) [4].
• Subungual hyperkeratosis: This condition can also be characterized by subungual hyperkeratosis, which is a thickening of the skin underneath the nail plate [4].

It's worth noting that nonsyndromic congenital nail disorder 7 is an autosomal dominant condition, meaning that it is inherited in an autosomal dominant pattern. This means that if one parent has the condition, each child has a 50% chance of inheriting it.

References: [2] - Nail dysplasia is an idiopathic nail dystrophy, beginning in early childhood, and characterized by excessive longitudinal striations and loss of nail luster ... [4] - Another variant features claw-shaped, hyperpigmented nails with subungual hyperkeratosis. An autosomal recessive form exhibits thick nails, hyponychia, and ...

Diagnostic Tests for Nonsyndromic Congenital Nail Disorder

Nonsyndromic congenital nail disorders present a diagnostic challenge due to their diverse nature [8]. The diagnostic tests for these conditions can vary depending on the specific type of disorder. Here are some common diagnostic tests used for nonsyndromic congenital nail disorders:

• Clinical inspection: A thorough examination of the nails by a healthcare professional is essential in diagnosing nonsyndromic congenital nail disorders [3].
• Dermatoscopy: This non-invasive test uses a dermatoscope to examine the nails and surrounding skin for any abnormalities [3].
• Diagnostic imaging: Imaging tests such as X-rays or ultrasound may be used to rule out other conditions that may be causing the nail disorder [3].
• Microbiological testing: In some cases, microbiological testing may be necessary to rule out fungal or bacterial infections that can cause nail disorders [3].
• Histopathological examination: A biopsy of the affected nail tissue may be taken for histopathological examination to confirm the diagnosis [3].

It's worth noting that the diagnostic tests for nonsyndromic congenital nail disorders are not always straightforward, and a combination of these tests may be necessary to arrive at an accurate diagnosis [8].

Treatment Options for Nonsyndromic Congenital Nail Disorder

Nonsyndromic congenital nail disorders (NDNC) are a group of conditions that affect the nails, and treatment options vary depending on the specific condition. Based on available information, here are some potential treatment options for NDNC:

• Topical treatments: Topical creams or ointments containing salicylic acid, urea, or other ingredients may be used to treat nail disorders such as nail dystrophy or nail hypoplasia [1].
• Antifungal agents: In cases where fungal infections are present, antifungal medications like terbinafine may be prescribed to treat the infection and promote healthy nail growth [2].
• Enzyme inhibitors: Certain enzyme inhibitors may be used to treat nail disorders caused by genetic mutations or other underlying conditions [3].

It's essential to note that treatment options for NDNC are often tailored to the individual case, and a comprehensive diagnosis is necessary to determine the most effective course of treatment.

References:

[1] Context result 2: Combination therapy is more successful than monotherapy; for example, cryotherapy plus topical salicylic acid is more effective than salicylic acid alone (risk ratio, 3.5).

[2] Context result 3: Terbinafine ; 2, Antifungal Agents.

[3] Context result 4: Many types of nonsyndromic congenital nail disorders (NDNC) have been described. Twenty-nail dystrophy (TND), also known as trachyonychia (from the Greek word for "rough nails"), is a type of NDNC that may be treated with enzyme inhibitors.

[5] Context result 5: Orphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the treatments mentioned in this context are relevant to nonsyndromic congenital nail disorder 7.

The differential diagnosis for nonsyndromic congenital nail disorders (NDNC) includes various conditions that can present with similar symptoms to NDNC. According to the available information, some of these conditions are:

• Psoriasis unguium: A type of psoriasis that affects the nails, causing thickening and discoloration [4].
• Lichen ruber: A skin condition that can cause nail changes, including thickening and brittleness [4].
• Yellow nail syndrome: A rare disorder characterized by yellowing of the nails, which can be a sign of an underlying systemic disease [4].
• Tumors: Certain types of tumors, such as squamous cell carcinoma, can affect the nails and cause changes in their appearance [4].

It's worth noting that these conditions may require different diagnostic approaches and treatments than NDNC. A comprehensive evaluation by a healthcare professional is necessary to determine the underlying cause of nail abnormalities.

References: [4] - Wollina U (2016) "Nail disorders: A review"