Treatment of disseminated superficial actinic porokeratosis (DSAP) with the Q‐switched ruby laser

Background: Disseminated superficial actinic porokeratosis (DSAP) is one clinical subtype of porokeratosis, a cutaneous disorder of keratinization. A variety of approaches may be used to treat DSAP. The ruby laser appears to be a promising option for DSAP treatment. Traditionally, the ruby laser is used to treat hair removal and lesions involving hyperpigmentation. Its use may be further applied to treat the hyperpigmented lesions of DSAP. Objective: This study examines the efficacy of the ruby laser in treating a case of DSAP. Methods: A 48‐year‐old female, with a history of pseudoxanthoma elasticum and DSAP, received three Q‐switched ruby laser treatments (694?nm) to over 50 sites on the lower and upper extremities. Clinical outcome and patient satisfaction was followed‐up. Results: Minimal to moderate erythema and appropriate whitening was noted after each treatment. The patient tolerated treatments well and hyperpigmentation and erythema of the majority of the lesions decreased. The patient was very pleased with the results and reports satisfactory cosmetic outcome 3 months later. Conclusion: The results obtained from the current case suggests that the ruby laser is moderately successful in treating DSAP and may still provide a good alternative to other available treatments. Further studies are needed to investigate the potential of combined ruby laser treatment for DSAP and to determine the appropriate laser pulse duration and fluence for effective treatment.     

Disseminated superficial actinic porokeratosis: a treatment review

Disseminated superficial actinic porokeratosis (DSAP) is a chronic disorder of keratinization characterized by numerous papules and plaques distributed over sun-exposed sites. Treatments are poorly standardized and several investigational therapies have demonstrated limited success in treating DSAP. To our knowledge, there have been no systematic reviews of the literature regarding the treatment of this disease. Herein, we review recent studies pertaining to the treatment of DSAP and evaluate the level of evidence for each of these therapeutic modalities.     

Disseminated superficial actinic porokeratosis treated effectively with topical imiquimod 5% cream

A 68-year-old man presented with a 3-year history of asymptomatic red marks on his limbs. On physical examination, he was found to have numerous circular, erythematous macules with a fine peripheral rim of scale on his upper arms and legs. A clinical diagnosis of disseminated superficial actinic porokeratosis (DSAP) was made. On histopathological examination of the lesional skin on the hands, an angulated parakeratotic tier was seen extending up through the epidermis. The patient was offered a trial of topical imiquimod 5% cream. He was instructed to apply it five times weekly for 6 weeks to the right forearm. This produced a dramatic inflammatory response with painful superficial ulceration of the affected skin after 4 weeks. At follow-up a further 4 weeks later, the treated areas were noted to have healed with slight superficial scarring and residual erythema, but no evidence of the original condition. DSAP is a disorder affecting areas exposed to sunlight, appearing mainly on the distal surfaces of the limbs. Treatment of DSAP is often difficult. Therapies such as cryotherapy with liquid nitrogen or use of topical 5-fluorouracil cream are generally either impractical or unsuccessful. Topical imiquimod 5% cream may prove to be a useful treatment option for DSAP. Our limited experience suggests that treatment should be introduced cautiously to avoid excessive inflammation. Further study is needed to confirm whether imiquimod should have a place in the management of DSAP.     

A novel locus (DSAP2) for disseminated superficial actinic porokeratosis maps to chromosome 15q25.1-26.1

BACKGROUND: Disseminated superficial actinic porokeratosis (DSAP) is a chronic cutaneous disorder characterized by multiple superficial keratotic lesions surrounded by a slightly raised keratotic border. It develops in teenagers in sun-exposed areas of skin and usually follows an autosomal dominant inheritance pattern. The first locus for DSAP was localized to chromosome 12q23.2-24.1, but no gene responsible for porokeratosis has been identified to date. OBJECTIVES: To determine whether DSAP is a genetically heterogeneous disorder and to identify the disease gene locus in a three-generation Chinese family with DSAP. METHODS: Genetic linkage analysis was carried out in this family using 15 microsatellite markers between D12S1671 and D12S369 on chromosome 12q, followed by a genome-wide scan with 382 microsatellite markers from the autosomes. RESULTS: Genetic linkage analysis with chromosome 12q markers suggested that the locus in this family is not linked to chromosome 12q. A genome-wide scan and fine mapping finally localized the locus for DSAP in this family to a 6.4-cM region between markers D15S1023 and D15S1030 at chromosome 15q25.1-26.1. This DSAP locus was named DSAP2. CONCLUSIONS: The previous results and this study have shown that DSAP is a genetically heterogeneous disorder; a novel locus for DSAP, termed DSAP2, was mapped to a 6.4-cM region between markers D15S1023 and D15S1030.     

A mutation in SART3 gene in a Chinese pedigree with disseminated superficial actinic porokeratosis

BACKGROUND: Disseminated superficial actinic porokeratosis (DSAP) is an uncommon autosomal dominant chronic disorder of keratinization, characterized by multiple superficial keratotic lesions surrounded by a slightly raised keratotic border. Thus far, although two loci for DSAP have been identified, and the genetic basis and pathogenesis of this disorder have not been elucidated. OBJECTIVES: To determine the locus of DSAP and identify the candidate gene(s) of the disease. METHODS: Genome-wide scan and linkage analysis were performed in a six-generation Chinese family with DSAP. The coding exons of the candidate genes were sequenced to analyse and detect the nucleotide variations. RESULTS: Linkage analysis showed that the maximum two-point lod score of 5.56 was obtained with the marker D12S79 at a recombination fraction theta of 0.00. Haplotype analysis defined the critical region for DSAP between D12S330 and D12S1612 on 12q24.1-24.2. By sequence analysis, we found a Val591Met mutation in SART3 in all affected individuals of the family. CONCLUSION: SART3 is a candidate gene for DSAP, and is possibly involved in the pathogenesis of DSAP.     

Diclofenac sodium 3% gel as a potential treatment for disseminated superficial actinic porokeratosis

Background   Disseminated superficial actinic porokeratosis (DSAP) is a chronic cutaneous disorder of keratinization for which there is no known cure. Current therapies are often ineffective, painful, or unappealing.
Objective   To investigate the efficacy and safety of diclofenac sodium 3% gel for the treatment of DSAP.
Methods   Seventeen adult patients with a diagnosis of DSAP applied diclofenac sodium 3% gel to a target area (forearm) twice daily for 3 months up to a maximum of 6 months in an open-label, multicentre pilot study. Target area lesion counts were performed monthly, and global lesion counts were performed at baseline and at weeks 12 and 24. A treatment satisfaction questionnaire was completed at weeks 12 and 24.
Results   Thirteen patients completed 12 weeks of treatment and 10 completed 24 weeks. Among patients who completed 12 weeks, there was a mean decrease of 4% in target area lesions, while a mean increase of 12% was noted in global lesions. Among patients who completed 24 weeks, there was a mean increase of 19% in global lesions, but only a 10% increase noted in the target area. Seven of 13 patients had a decrease in target area lesions at week 12 and 3 of 10 patients at week 24. Questionnaire responses indicated 6 out of 10 patients would use the medication again.
Conclusion   Target area DSAP lesions in the majority of patients treated with diclofenac sodium 3% gel (both 12 and 24 weeks) progressed to a lesser extent as compared to the global lesion count.     

Disseminated superficial actinic porokeratosis with sterile spongiform pustular dermatitis

A 55-year-old woman with disseminated superficial actinic porokeratosis (DSAP) with sterile spongiform pustular dermatitis is described. The pustular dermatitis may have developed from friction of the DSAP lesions with clothes, gloves and/or stockings.     

Squamous Cell Carcinoma Developing within Lesions of Disseminated Superficial Actinic Porokeratosis

Disseminated superficial actinic porokeratosis (DSAP) consists of multiple annular, hyperkeratotic lesions that have a bilateral distribution on sun-exposed areas, particularly the extremities. DSAPs have a wider distribution than porokeratosis of Mibelli and usually develop during the 3rd or 4th decade of life. Squamous cell carcinoma that arises in the classical type of porokeratosis of Mibelli is well-documented, but there are only a few reports of squamous cell carcinoma in DSAP. Here, we describe a 62-year-old man with DSAP who developed squamous cell carcinoma on his right forearm.     

Amelanotic melanoma arising within a lesion of disseminated superficial actinic porokeratosis: An unusual presentation leading to a novel therapeutic approach

Disseminated superficial actinic porokeratosis (DSAP) is the most common variant of porokeratosis with a potential for malignant transformation. Its association with malignant melanoma, however, is exceedingly rare. Treatment of DSAP is often ineffective. We report a unique case of amelanotic melanoma arising within a lesion of DSAP. The melanoma was managed surgically, and her DSAP were treated successfully with a novel approach utilizing 5‐fluorouracil chemowraps.     

Cultured skin fibroblasts derived from three patients with disseminated superficial actinic porokeratosis (DSAP) are hypersensitive to the lethal effects of X-radiation but not to those of ultraviolet (UV) light

Abstract Disseminated superficial actinic porokeratosis (DSAP), skin lesions of which are known to be induced by ultraviolet (UV) light, does not develop into skin cancer as frequently as other types of porokeratosis (PK) To establish the cellular basis of this characteristic of DSAP. we examined the colony-forming ability of UVC light- or X-ray-irradiated cultured fibroblasts derived from DSAP patients' skin. Sensitivity to the lethal effects of UV light was not significantly different between 3 DSAP and 5 control cell strains. In contrast, DSAP cell strains were significantly hypersensitive to the lethal effects of X-radiation, although the sensitivity was less than that of cell strains from other types of PK patients. The results indicate that the actinic character of DSAP is not reflected in the cellular response to the lethal effects of UV light, but suggest that DSAP shares X-ray sensitivity, which is probably associated with the cancer-prone nature of PK.     

Bildgebende Diagnostik der aktinischen Porokeratose mittels optischer Kohärenztomographie (OCT)

Disseminated superficial actinic porokeratosis (DSAP) is a rare, genetically heterogeneous skin disorder. We report a case of a 73-year-old female patient who was diagnosed with DSAP by optical coherence tomography (OCT) and histology. During the last 4 years prior to diagnosis, she had developed numerous (pre)malignant lesions of the skin of the lower legs including actinic keratoses, squamous cell carcinomas and Bowen's disease. DSAP lesions and actinic keratoses were resistant to topical treatment with imiquimod and retinoids, but improved with photodynamic therapy (PDT).     

ED2000®: 585 nm collagen remodelling pulsed dye laser

The wavelength of 585nm corresponds to an absorption peak of haemoglobin. The heating effect in these skin layers triggers the release of various growth factors that stimulate collagen remodelling and tightening. We report our experience with a 585nm collagen remodelling, double flashlamp excited pumped dye laser was used (ED2000^®, Deka MELA, Calenzano, Italy), spot size 5?mm, energy density (fluence J/cm²) from 2 to 4?J/cm², emission modality (repetition rate) at 0.5?Hz, with a short pulse duration of 250?μsec. The efficiency of 585?nm collagen remodelling pulsed dye laser is controversial in only one session. It is probably reasonable to inform patients that 3–4 treatment sessions are necessary, and that 10% of the patient have no response to nonablative photorejuvenation.

Because of its low fluence and is shorter pulse duration, the 585?nm collagen remodelling pulsed dye laser has limited efficacy for the treatment of port wine stains. However, it may offer patients with erythematous, raised or hypertrophic acne scars or striae distensae a permanent cosmetic solution. This laser is safe and effective in the treatment of surgical scars starting as soon as possible, on the day of suture removal if possible. We found that 96.3% of molluscum contagiosum healed after the first treatment, the other 3.7% after the second.     

“Drug‐resistant granuloma faciale”: treatment with carbon dioxide‐GaAs laser

Granuloma faciale (GF), also known as “eosinophilic granuloma,” is a rare benign leukocytoclastic vasculitis which most commonly occurs on the face of middle‐aged Caucasian males. Clinically, GF appears as single or multiple, slowly growing, reddish‐brown papules, nodules or plaques which may be cosmetically unpleasant. Its pathogenesis is unknown and GF is notoriously resistant to treatments. Both medical (dapsone, colchicine, gold injections, isoniazid, clofazimine, corticosteroids, psoralen ultraviolet radiation, and topical tacrolimus) and surgical therapies (excision, graft, dermabrasion, argon laser, carbon dioxide laser, pulsed dye laser, cryotherapy, and electrosurgery) have been used for GF but no effective treatment has yet been found. Furthermore, the typical facial location of GF requires an acceptable cosmetic result. We report two cases of drug‐resistant GF which were successfully treated with laser vaporization combining two different wavelengths: carbon dioxide (CO₂) 10,600 nm and GaAs 1540 nm.     

Laser resurfacing of the skin for the improvement of facial acne scarring: a systematic review of the evidence

This review presents and evaluates the evidence of the effectiveness of laser resurfacing for facial acne scars. Primary studies of all types of design in any language were identified from MEDLINE, EMBASE, the Cochrane database, Science Citation Index and various internet sites. Studies were accepted if they included patients treated by any laser for atrophic or ice-pick acne scars. The quality of the studies was assessed and data extracted by two independent researchers. There were no controlled trials but 14 case series were found which reported the effects of either the carbon dioxide or erbium:YAG laser. All of the studies were of poor quality. The types and severity of scarring were poorly described and there was no standard scale used to measure scar improvement. There was no reliable or validated measure of patient satisfaction; most improvement was based on visual clinical judgement, in many cases without blinded assessment. The inaccurate use of ordinal scales meant that any improvement was impossible to quantify with any validity, although the evidence suggested that laser treatment had some efficacy (a range in individual patients of 25-90% for both the carbon dioxide laser and the erbium:YAG laser). Changes in pigmentation as a side-effect were common (in up to 44% of patients), although lasting only a few weeks. Laser resurfacing technology is increasingly used in clinical practice to treat acne scars. Despite the poor quality evidence, it is plausible that there is some improvement of acne scarring; there is insufficient information, however, for patients to make informed decisions on whether to opt for treatment and there is not enough evidence to compare the two types of laser. There is a particular lack of information about the psychological effects of acne scar improvement. Good quality randomized controlled trials are needed with standardized scarring scales and validated patient outcome measures in order to assess the effectiveness of laser resurfacing in this group of patients.     

Confirmation and refinement of a genetic locus for disseminated superficial actinic porokeratosis (DSAP1) at 12q23.2-24.1

BACKGROUND: Our previous study has identified two loci for disseminated superficial actinic porokeratosis (DSAP), but the genes responsible are still unknown. OBJECTIVES: To narrow down the candidate regions and to assess candidate genes. METHODS: A genome-wide scan and linkage analysis were carried out in a newly collected five-generation Chinese family with DSAP. In addition, six candidate genes were screened for possible DSAP-associated mutations. RESULTS: DSAP in this family was associated with chromosome 12q. Fine mapping and haplotype construction refined the DSAP1 locus to a 4.4-cM interval. No disease-associated mutation was detected in CRY1, C4ST1, TXNRD1, HCF2, CMKLR1 or KIAA0789 genes. CONCLUSIONS: The DSAP1 locus was localized to a 4.4-cM interval at chromosome 12q23.2-24.1. CRY1, C4ST1, TXNRD1, HCF2, CMKLR1 and KIAA0789 genes were not associated with DSAP1.     

Nd:YAG Q-switched laser for the treatment of a hemicorporal epidermal nevus: A safe and effective option

Epidermal nevi are benign proliferations of the epidermis for which different treatments have been used with disappointing results due to their recurrences and anesthetic scars. Topical therapies have generally been ineffective and surgical treatment provides more definitive results, but with high risk of scarring. In recent years, multiple laser modalities have been described for the treatment of these lesions. In the literature, there are no reported cases of treatment of these lesions with Neodymium-doped Yttrium aluminum garnet (Nd:YAG) laser. We present the case of a 3-year-old patient with a hemicorporal epidermal nevus treated with Nd:YAG laser at an early stage with good results.     

Treatment of disseminated superficial actinic porokeratosis with topical diclofenac gel: a case series

Disseminated superficial actinic porokeratosis (DSAP) is the most common of the of five clinical variants of porokeratosis. These are disorders of keratinization and the distinctive pathological feature is the cornoid lamella at the margin. DSAP usually manifests in the third or fourth decades of life with a female preponderance and with multiple lesions over sun‐exposed areas. A diverse range of treatments is employed though evidence of efficacy remains largely anecdotal. We report a series of eight patients with DSAP treated with 3% diclofenac gel (Solaraze® gel).     

Ultrapulse carbon dioxide laser treatment for bilateral facial nevus comedonicus: A case report

Nevus comedonicus (NC), a rare skin ailment with an aggregation of dilated follicular orifices filled with keratinous material, is difficult to treat. Several drugs have been assessed for the treatment of NC, but with limited success. Surgery requires much experience and the recurrence rate is high. Various types of laser have been tried, with promising outcomes. A 54‐year‐old male patient with bilateral facial NC was admitted on July 8, 2014. A coin‐sized area was first treated successfully with ultrapulse CO₂ laser. The remaining lesions were treated during three subsequent sessions at 2‐week intervals. There were no complications. There was no recurrence after 2 years. This case suggests that ultrapulse CO₂ laser could efficiently alleviate NC. Ultrapulse CO₂ laser treatment should be further studied for its application in the treatment of NC.     

Disseminated superficial actinic porokeratosis. Coexistence with other porokeratotic variants

The coexistence of disseminated superficial actinic porokeratosis (DSAP) with other variants of porokeratosis is rare. We report three such cases: DSAP with porokeratosis of Mibelli; DSAP with linear porokeratosis; and DSAP occurring in the mother of a girl with linear porokeratosis. Although different areas of skin and different family members usually express the same morphological variant, we suggest that the simultaneous expression of two closely linked gene loci could explain the coexistence of different porokeratotic variants.     

Disseminated superficial actinic porokeratosis: Experimental induction and exacerbation of skin lesions

A 55-year-old woman with disseminated superficial actinic porokeratosis (DSAP) had lesions on sun-exposed skin areas that were exacerbated during the summer months and involuted in winter. This is the third report in which induction and exacerbation of DSAP lesions were achieved by irradiation with artificial ultraviolet light sources. Our data show that UVB plus UVA is more effective in inducing new or exacerbating preexisting skin lesions than either wavelength alone. We believe that testing with the appropriate ultraviolet light sources is a practical means to differentiate between DSAP and disseminated superficial porokeratosis.