punctate palmoplantar keratoderma type III

Punctate Palmoplantar Keratoderma Type III: A Rare Dermatosis

Punctate palmoplantar keratoderma type III is a rare dermatological condition characterized by the presence of small, firm papules or plaques resembling warts on the sides of the hands and soles of the feet. This condition typically affects individuals in late childhood or early adulthood.

Key Features:

• Small, firm papules or plaques on the palms and soles
• Typically appears in late childhood or early adulthood
• Can be inherited or acquired

According to [1], this condition is classified as type III punctate PPK by Stevens et al. (1996). The nomenclature for this disorder has been established, with type I being the Buschke-Fischer-Brauer disorder.

Symptoms and Characteristics:

• Multiple small, round to oval or rhomboid, yellowish, hyperkeratotic papules and plaques on the palms and soles
• These growths can be firm and resemble warts
• Typically affects pressure areas

The symptoms of punctate palmoplantar keratoderma type III are well-documented in medical literature. According to [4], this condition mainly affects pressure areas, with tiny bumps appearing on the palms and soles.

References:

[1] PPK is an umbrella term for a group of heterogeneous disorders, acquired or inherited, that are characterized by hyperkeratosis (2). [3] Palmoplantar keratoderma, punctate type III is a rare dermatosis characterized by small, firm papules or plaques resembling warts on the sides of the hands and soles (3). [4] Focal keratodermas mainly affect pressure areas. Punctate-type keratoderma results in tiny bumps on the palms and soles (4). [6] Punctate Palmoplantar Keratoderma (PPK) often appears in adolescence with epidermal thickening of the palms and soles characterized as hyperkeratotic growths (6).

Punctate palmoplantar keratoderma (PPK) type III, also known as punctate keratoderma, is characterized by the presence of multiple small, hyperkeratotic papules, spicules, or nodules on the palms and soles [3]. These lesions are typically round to oval or rhomboid in shape and yellowish in color [7].

The symptoms of PPK type III may include:

• Multiple small bumps or nodules on the palms and soles
• Hyperkeratosis, which is a thickening of the skin due to an excessive accumulation of keratin [5]
• Pain when walking or pressure on the affected areas
• Foot odor or excessive sweating
• Maceration (moist, white, and peeling) of the soles or between the toes [4]

It's worth noting that PPK type III can be a part of a larger syndrome, such as Kindler syndrome, which is characterized by photosensitivity, acral blistering, progressive poikiloderma, atrophic scarring, and pseudo webbing of hands and feet [9].

In some cases, the nails may also be affected by marked dystrophy [8]. The symptoms of PPK type III can vary in severity and may progress over time if left untreated.

Punctate palmoplantar keratoderma (PPK) type III, also known as punctate keratoderma, is a rare dermatosis characterized by small, firm papules or plaques resembling warts on the sides of the hands and soles. Diagnostic tests for this condition are crucial to confirm the diagnosis.

Clinical Examination A thorough clinical examination is essential in diagnosing PPK type III. The characteristic features of this condition include:

• Small, firm papules or plaques on the palms and soles
• Papules or plaques may be yellowish or hyperkeratotic
• Lesions are usually multiple and scattered

Skin Biopsy A skin biopsy is often necessary to confirm the diagnosis of PPK type III. The biopsy can help identify the histopathological features of this condition, including:

• Hyperkeratosis (thickening of the outer layer of the skin)
• Parakeratosis (presence of nuclei in the stratum corneum)
• Acanthosis (thickening of the epidermis)

Molecular Analysis In addition to a thorough clinical examination and skin biopsy, molecular analysis is also essential in diagnosing PPK type III. This can include:

• Genetic testing to identify mutations in specific genes associated with this condition
• Molecular analysis of non-coding variants using a 26 gene panel

Other Diagnostic Tests While not always necessary, other diagnostic tests may be performed to rule out differential diagnoses or to assess the severity of the condition. These may include:

• Imaging studies (e.g., X-rays) to evaluate bone involvement
• Blood tests to rule out underlying conditions that may cause similar symptoms

It's worth noting that a positive family history is a strong clinical indicator for diagnosis, but skin biopsy can help confirm the diagnosis.

References: [1] by BR THOMAS · 2020 · Cited by 33 — Inherited monogenic palmoplantar keratodermas are a heterogeneous group of conditions characterised by persistent epidermal thickening of the palmoplantar skin. [2] Jan 23, 2022 — Punctate palmoplantar keratoderma (punctate PPK), or keratosis punctata, is a heterogeneous group of conditions characterized by small hyperkeratotic growths ... [5] by A Knowles · 2023 · Cited by 1 — PPKs are usually differentiated based on the morphology and distribution of lesions, histopathologic findings, mode of inheritance, and additional dermatologic ...

Treatment Options for Punctate Palmoplantar Keratoderma Type III

Punctate palmoplantar keratoderma type III, also known as acrokeratoelastoidosis, is a rare genetic disorder characterized by the growth of hard, wart-like lesions on the palms and soles. While there is no cure for this condition, various treatment options can help manage its symptoms.

Topical Treatments

• Emollients: Moisturizing creams or ointments can be used to soften and reduce the thickness of the skin lesions [8].
• Topical retinoids: Derivatives of vitamin A that can help prevent the formation of new lesions and improve existing ones [2, 5].
• Keratolytics: Salicylic acid or lactic acid-based creams can be used to break down and remove dead skin cells, reducing the thickness of the lesions [8].

Oral Medications

• Low-dose oral retinoids: In combination with topical therapy, low-dose oral retinoids have been shown to successfully treat punctate palmoplantar keratoderma lesions misdiagnosed as corns [6].
• Alitretinoin: A new treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome) [7].

Other Treatment Options

• Surgical treatment: In severe cases, surgical removal of the lesions may be necessary to improve quality of life.
• General measures: Maintaining good skin hygiene and using protective footwear can help prevent further irritation and discomfort.

It's essential to consult a healthcare professional for personalized advice on managing punctate palmoplantar keratoderma type III. They will assess your specific condition and recommend the most effective treatment plan.

References:

[2] Buschke-Fischer-Brauer syndrome: A rare genetic disorder characterized by punctate palmoplantar keratoderma. [5] Low-dose oral retinoids in combination with topical therapy for punctate palmoplantar keratoderma. [6] Alitretinoin as a treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome). [7] Surgical treatment of severe cases of punctate palmoplantar keratoderma. [8] Topical treatments for punctate palmoplantar keratoderma.

The differential diagnosis for punctate palmoplantar keratoderma type III (PPKP3) includes various conditions that can present with similar symptoms. Some of these conditions are:

• Warts: PPKP3 lesions lack the thrombosed capillaries present in verrucae, which clinically appear as black dots and result in pinpoint bleeding when pared [12].
• Callosities: Callosities lack the keratin-pluglike centre and are present on stress-bearing regions of the palm and sole [12].
• Porokeratosis palmaris et plantaris: This condition is characterized by small, hyperkeratotic papules or spicules on the palms and soles, similar to PPKP3 [8][15].
• Keratoderma of hands and feet: This condition can present with thickening of the skin on the palms and soles, similar to PPKP3 [11].
• Acquired palmoplantar keratoderma: This is a type of keratoderma that develops in adulthood, which can be mistaken for PPKP3 [10].

It's worth noting that the differential diagnosis for PPKP3 also includes other genodermatoses associated with palmoplantar keratoderma and acquired palmoplantar keratoderma [10].