Case of punctate palmoplantar keratoderma type I treated with combination of low-dose oral acitretin and topical salicylic acid and steroid.

Palmoplantar keratodermas (PPK) are heterogeneous disorders characterized by abnormal keratinization. Especially, punctate PPK (PPPK), one of the subtypes of hereditary PPK, is a rare punctate keratoderma characterized by tiny "raindrop" keratoses having a tendency to coalesce on the edge of soles, which are exposed to sustained pressure. If typical punctate lesions are confined to the palms and soles and the patient has a family history and late onset, it can be considered as PPPK type I (PPKP1), also called Buschke-Fisher-Brauer disease. The exact etiology of PPPK has not been fully understood. Furthermore, no standardized treatment for PPPK has been established and treatment options are limited. Above all, traditional systemic retinoids have been used in several cases, but dose-related adverse effects are common. Therefore, combination of low-dose systemic retinoids and adjuvant topical therapy can be an alternative treatment option for PPPK. Herein, we report a case of PPKP1 treated with combination of low-dose oral acitretin (10 mg/day) and topical salicylic acid and steroid. Despite low capacity, low-dose acitretin showed excellent regression of the lesions by combined use of topical ointments. The supplementary topical therapy may be useful in reducing the dose of systemic retinoids and preventing potential toxicity.

Disseminated punctate keratoderma: a rare case report and review of the literature.

We report a rare case of a 53-year-old womanpresenting with diffuse, late-onset disseminatedhyperkeratotic papules. Biopsy showed massivehyperkeratosis overlying a crateriform epidermaldepression and hypergranulosis with mild epidermalhyperplasia. There was no parakeratosis, cornoidlamella, or dyskeratosis. Based on the clinical findingsand histopathological features, a diagnosis ofdisseminated punctate keratoderma was made. Thisis a rare subtype of palmoplantar keratoderma, whichhas a putative increased risk of malignancy. This casereport emphasizes the importance of identifyingthe clinical and histological presentation of this rarecondition; referral of the patient for age-appropriatemalignancy screening is appropriate. We also presenta concise review of treatment options.

Retrospective long-term follow-up in patients with chronic palmoplantar dermatoses after good response to bath PUVA therapy.

BACKGROUND: Numerous studies have confirmed the short-term effectiveness of 8-methoxypsoralen bath PUVA therapy in patients with chronic palmoplantar dermatoses; however, little is known about long-term results. PATIENTS AND METHODS: In this retrospective study we examined the long-term results in 79 patients (mean age: 48 years) with chronic palmoplantar dermatoses who were treated with bath PUVA three times a week over an 8-year period. A good clinical response (a reduction of more than 50% of the skin lesions) occurred after a mean of 23 treatments and a mean cumulative UVA dose of 39 J/cm(2) in 51 patients (65%). In 2007 a questionnaire was sent to these 51 patients to assess the long-term outcome. RESULTS: With bath PUVA treatment, the best results were found in patients with hyperkeratotic eczema (17/22; 77% good clinical response) followed by patients with palmoplantar psoriasis (26/41; 63%) and patients with dyshidrotic eczema (8/16; 50%). Thirty-four patients (67%) answered the questionnaire after a mean follow-up interval of 4.3 years (10-87 months). Among these patients, 36% reported an improved course of disease after PUVA therapy with reduced frequency and/or intensity of the skin rash, and 29% of patients reported continued complete clearance. 79% of our patients reported a long-term reduction in the use of topical corticosteroids during the follow-up period (mean: 4.3 years). In addition, 67% of patients reported a lasting improvement in quality of life. CONCLUSIONS: These data show that bath PUVA may have a long-term, beneficial influence on the course of disease in a majority of patients with recalcitrant chronic palmoplantar dermatoses.

Aquagenic acrokeratoderma.

BACKGROUND: Aquagenic acrokeratoderma (AA) is a rare condition with female predilection that occurs after brief water exposure and disappears minutes to an hour after drying. The pathogenesis remains unclear. METHODS: Four Chinese patients with AA were reported and analyzed. RESULTS: There were 2 males and 2 females (age range: 14-33 years) who presented with a 2-week to more than 10-year history of small white papules coalescing into edematous plaques on the hands; lesions appeared within 5-10 minutes of water exposure, began to regress in 3-20 minutes and disappeared within 5 minutes to one hour after drying. Warm water provoked the lesions more rapidly than cold water. Lesions of a female patient could be triggered by detergent. In another female patient, lesions also involved the feet and were associated with palmoplantar erythema and hyperhidrosis. Biopsy from the lesion of one patient after water exposure revealed hyperkeratosis, mild hypergranulosis, and dilatated eccrine ducts. Biopsy from the lesion of another patient after drying showed normal epidermis and dermis. Two patients were treated with topical formalin 3% in alcohol, and two with 3% potassium aluminium sulfate solution with partial relief without any adverse effects. CONCLUSION: AA may occur in both males and females and may involve the feet. Warm water triggers the lesions more rapidly than cold. Topical formalin 3% in alcohol and 3% potassium aluminium sulfate solution may be optional therapy.

Chronic arsenic toxicity from Ayurvedic medicines.

BACKGROUND: Ayurvedic medicines are known to contain arsenic and concentrations up to toxic levels have been reported in certain formulations. However, clinical disease due to arsenic containing ayurvedic medicines has rarely been reported. We seek to highlight the existence of toxic levels of arsenic in certain ayurvedic preparations that can produce serious systemic manifestations. METHODS: An 11-year-old girl developed manifestations of arsenical keratosis (punctuate palmoplantar keratoderma and leucomelanoderma) and non-cirrhotic portal hypertension, 6 months and 18 months respectively after intake of ayurvedic medications, prescribed for epilepsy. The eight ayurvedic preparations consumed by the patient and her serum levels were analyzed for arsenic content. RESULTS: Arsenic content of ayurvedic medicines ranged from 5 mg/L to 248 mg/L. The serum arsenic level was 202.20 microg/L (normal < 60 microg/L). Skin manifestations improved after the discontinuation of ayurvedic medications. CONCLUSIONS: Ayurvedic medications should be consumed under strict guidance and supervision of qualified practitioners to prevent such catastrophies.

[Pityriasis rubra pilaris]. / Pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on the scalp and spreads caudally within a few weeks. It often results in a generalized erythroderma with sharply demarcated islands of sparing ("nappes claires"). A 65-year-old patient with severe PRP showed good clinial improvement after 8 months of treatment when treated with acitretin in combination with phototherapy and systemic gluocorticosteroids.

Hereditary palmoplantar keratoderma (four cases in three generations).

A 39-year-old man reported with progressive thickening of the skin of the hands and feet and an inability to flex his hand. It was largely asymptomatic; however, brisk walking caused excessive sweating, pain, and widening of the fissures on the soles of the feet. He was unable to walk barefooted. According to his mother, the first episode presented with blistering at 7 days of age. Ever since, the condition has steadily worsened to acquire the current status. He was married at the age of 18 years, and had a stillborn child 18 months afterwards. Presently, he has three children, two girls aged 14 and 12 years and a son aged 10 years. Both the daughters are similarly affected. While cataloguing the details of the pattern of inheritance, the mother of the index case was also found to be affected (Fig. 1). The natural history of the disease was identical. Examination of the palms was marked by pronounced thickening of the skin resulting in the masking of palmar creases. The thickening was well demarcated and its margins were prominent and surrounded by an erythematous halo. The color of the skin was yellow and waxy (Fig. 2a). Contractures were present on all the fingers; nevertheless, the deformity of the middle and distal interphalangeal joints of the little finger was prominent. The soles of the feet had a similar morphology. In addition, marked fissuring was obvious (Fig. 2b). His daughters had an identical affliction of the palms and soles. The texture and morphology of the nails were normal. Light microscopy performed on scrapings from the fissures, mounted on 10% potassium hydroxide, revealed mycelia (hyphae) and spores. Hematoxylin and eosin-stained microsections from the palms and soles showed exquisite changes in the epidermis characterized by considerable uniform orthohyperkeratosis. Hypergranulosis and acanthosis were other associated changes. In addition, perinuclear vacuolization and keratohyalin granules of varying sizes and shapes were located at the periphery of the cells. A sparse mononuclear infiltrate was located at the dermo-epidermal junction. Hyphae and spores of fungi were also identified in the stratum corneum (Fig. 3). Itraconazole, 400 mg/day in two equally divided doses, was administered with major meals for 7 days. In addition, high doses of vitamin A (100,000 IU) were given daily for 2 weeks, supplemented by 12% salicylic acid (Salicylix SF12) ointment for daytime application and an ointment containing 6% coal tar and 3% salicylic acid (Salytar) for night-time application. This treatment is useful in recalcitrant cases.

Spiny keratoderma: case report, classification, and treatment of music box spine dermatoses.

The classification of palmoplantar lesions resembling music box spines is confusing, and nearly all attempts at treatment have been futile. We present the case of a 62-year-old white man with symptomatic music box spine lesions on his palms and fingers composed of parakeratotic columns over a hypogranular epidermis. Lesions and symptoms disappeared after a short course of 6 percent salicylic acid gel applied under occlusion. We suggest a classification scheme for this and similar-appearing conditions under the title spiny keratoderma, based on their histologic pattern (parakeratotic or hyperkeratotic) and anatomical location (palmoplantar, diffuse, or associated with epidermal appendages).