Invasive squamous cell carcinoma within a plaque of circumscribed palmar hypokeratosis.

Circumscribed palmar hypokeratosis (CPH) is a localized disorder of epidermal keratinization that is presented as a well-delimited, depressed, erythematous plaque on the palms or on the soles. It is histopathologically characterized by an abrupt thinning of the corneal epidermal layer. CPH is considered a benign condition, but a few cases with dysplastic changes/carcinoma in situ in the hypokeratotic epidermis have been described. We report hereby a case of invasive squamous cell carcinoma developed within a plaque of CPH. The pathogenesis of the malignant changes in this disorder remains to be clarified. Clinicians should be aware of the potential for developing malignancy in CPH and carry out a closer follow-up of this disorder.

[Atypical leiomyoma in a patient with cutaneous leiomyomatosis and mutation of the enzyme fumarate hydratase]. / Leiomioma cutáneo atípico en un paciente con leiomiomatosis cutánea y mutación de la enzima fumarato hidratasa.

We report the case of a 56 year-old male with an atypical leiomyoma in the context of a cutaneous leiomyomatosis and a family history of uterine leiomyomatosis. The genetic study revealed a mutation in the gene for the enzyme fumarate hydratase, but he has not had any renal malignancy so far. Atypical leiomyoma is a rare tumor that usually presents as a single lesion and is exceptional in patients with cutaneous leiomyomatosis. The relation between fumarate hydratase enzyme mutations with multiple leiomyomas, uterine leiomyomatosis and an increased risk of developing kidney cancer is widely known. However, the role of these mutations in the development of atypical leiomyomas is still impossible to clarify given the few cases reported in the literature.

Unilateral and pruritic papules: segmental Darier-White disease.

Darier-White disease is an uncommon disorder, which presents in a localized pattern in about 10% of patients, usually without nail, mucosa, or acral involvement. Type-1 is the most common of the segmental Darier-White disease types: papules have unilateral distribution along Blaschko lines. A 36-year-old woman diagnosed with type-1 segmental Darier-White disease is reported herein.

Dermoscopy of acral angioma serpiginosum.

Angioma serpiginosum (AS) is an unusual vascular disorder that typically affects female patients, begins in childhood and stabilizes in adulthood and not frequently involve acral skin. We herein present a 13 year-old girl with an asymptomatic erythematous punctuate first noticed on the right palm three years ago, with a proximal serpiginous progression up to the forearm. On examination there was a nonblanching erythematous punctuate on the palm and the inner aspect of right hand and forearm. Dermoscopy showed an erythematous parallel ridge pattern with some red globules and dots spreading on a linear arrangement, and the acrosyringia openings were not affected. Histopathological study showed dilated capillaries in the dermal papillae. This feature is consistent with angioma serpiginosum (AS). To the best of our knowledge, this is the first report that shows a dermoscopic image of a palmar AS. The dermoscopic pattern described in this case could aid in the diagnosis of AS and could add a value in the differential diagnosis with vascular lesions on acral skin.

Acral persistent papular mucinosis: is it an under-diagnosed disease?

Acral persistent papular mucinosis is a subtype of localized lichen myxedematosus. It presents as acrally located papules with a benign, but persistent course. It is a scarcely reported disease. We present a female with both the clinical and histopathological described criteria.

Self-regressing S100-negative CD1a-positive cutaneous histiocytosis.

In the skin, the antigen-presenting cells are mainly represented by Langerhans cells, indeterminate cells, and interstitial dendritic cells, which show distinctive immunophenotype and/or ultrastructure. We report a case of a cutaneous-limited self-regressing histiocytosis with a peculiar immunohistochemical profile (CD1a-positive and S100 protein-negative) that is not observed in any of the known cutaneous antigen-presenting cell or nowadays recognized neoplasm. This lesion is probably related to indeterminate dendritic cell tumors, but very few cases with such immunoprofile have been reported up-to-date, and their exact nosologic position and outcome remain to be clarified.

Dermoscopic changes in melanocytic nevi after depilation techniques.

Depilation techniques have gain popularity in the last few decades. Nowadays there are available a wide variety of lasers as well as intense pulsed light for depilation. However, little is known about the long-term effects of these procedures when melanocytic nevi are affected by them. We report the cases of three patients where we observed clinical, dermoscopic and histopathological changes secondary to laser therapy and intense pulsed light depilation, respectively. We believe that it is necessary to perform further studies to prove the absence of malignant transformation so that we will be able to set up recommendations in those patients with a personal or family history of malignant melanoma and/or dysplastic nevi.

Clinically relevant pain relief with an ibuprofen-releasing foam dressing: results from a randomized, controlled, double-blind clinical trial in exuding, painful venous leg ulcers.

The objective of this 6-week, 120-patient, double-blind, randomized, controlled trial was to investigate if a foam dressing with ibuprofen provided clinically relevant pain relief (PAR) for exuding, painful venous leg ulcers in comparison with a similar foam dressing without ibuprofen. Primary outcome parameter was PAR compared with baseline pain during the first 5 days of the investigation. PAR was registered by the patient morning and evening. Main end point was proportion of patients reporting a summed PAR score of at least 50% of the total maximum PAR (i.e., responders) and the corresponding number needed to treat (NNT). Wound-related parameters such as ulcer healing, ulcer area reduction, and peri-ulcer skin condition as well as adverse events were recorded during all 6 weeks of the investigation. PAR was significantly greater in the ibuprofen foam group than the comparator group (p = 0.0438). There were 34% responders in the ibuprofen foam group vs. 19% in the comparator group (NNT = 6.8). When evening data were analyzed separately to evaluate PAR over daytime, NNT was 5.3. Wound healing parameters and adverse events were comparable. In conclusion, in this study, the ibuprofen foam dressing provided clinically relevant PAR for patients with exuding, painful venous ulcers.

Photodynamic therapy for the treatment of folliculitis decalvans.

Folliculitis decalvans is a chronic form of deep folliculitis that occurs on the scalp as patches of scarring alopecia at the expanding margins of which are follicular pustules. Treatment of folliculitis decalvans is extremely difficult with a resultant poor prognosis. Photodynamic therapy has been reported to be effective in disorders as acne or folliculitis. We report one patient with folliculitis decalvans who was successfully treated with photodynamic therapy.

Cutaneous leishmaniasis with histopathologic pattern of non-necrotizing granulomatous dermatitis in patients treated with adalimumab.

Human leishmaniasis produced by Leishmania infantum is endemic in Mediterranean countries. In the context of a leishmaniasis outbreak in the town of Fuenlabrada, Madrid, Spain, we had two patients with cutaneous leishmaniasis that developed non-necrotizing cutaneous granulomas. They had both been receiving anti-TNF treatment with adalimumab for rheumatic diseases. Neither of them developed visceral disease and did not require anti-TNF treatment withdrawal to control the cutaneous disease. It is well known that anti-TNF therapy is associated with opportunistic diseases, especially with those in which granuloma formation is an important part of the host defence, as in tuberculosis. We think that granuloma formation through activation of Toll-like receptor-9 and via induction of a Th17 response may be precipitated by the parasites in the dermis.

Unsuccessful treatment of recalcitrant cutaneous discoid lupus erythematosus with photodynamic therapy.

Discoid lupus erythematosus (DLE) can be a therapeutic challenge. Antimalarials and topic steroids are the first-line standard therapies, while systemic steroids, immunomodulators (as azathioprine, methotrexate, cyclosporine), retinoids (acitretin), thalidomide, auranofin and dapsone are used as second-line therapies. We report two patients with recalcitrant DLE who were treated with three and two sessions of 5-aminolevulinic photodynamic therapy without an improvement and with a bad tolerance to the therapy.

Long-term management of erythrodermic psoriasis with anti-TNF agents.

Erythrodermic psoriasis is a chronic condition that is difficult to treat. Biological agents offer a new alternative, but there are no controlled trials to support their use; there are a few reports of patients treated with these agents, but often only with short term results. We report a 68-year-old man with erythrodermic psoriasis and ankylosing spondylitis, treated with infliximab for 48 weeks and then low-dose etanercept monotherapy for 34 additional months. Excellent results were obtained for both conditions without significant side effects. We think etanercept can be a good therapeutic option for long-term control of erythrodermic psoriasis.