Efficacy of alitretinoin in the treatment of Darier disease: a case report.

Darier disease is a rare autosomal dominant genodermatosis that initially first presents in adolescence with scaly reddish brown keratotic papules and plaques with a seborrheic and intertriginous distribution. The absence of specific targeted medications complicates the treatment process, and managing resistant cases can prove challenging due to recurrent exacerbations that may result in serious complications such as secondary bacterial and viral infections. Treatments of choice include antiseptics, topical corticosteroids, and systemic retinoids, mainly acitretin and isotretinoin. We report the case of a female patient with Darier disease that was unsuccessfully treated with acitretin and isotretinoin but showed significant improvement with alitretinoin. Previous reports on the efficacy of alitretinoin in Darier disease are reviewed.

Pemphigus vulgaris with refractory gingival ulcerations, successfully treated with rituximab-A case report.

A patient presented with ulcerations of the buccal mucosae, palate and gingiva. A gingival biopsy confirmed the diagnosis as pemphigus vulgaris. Despite medication with systemic corticosteroids and mycophenolate mofetil, desquamative gingivitis persisted. Adjunct treatment with rituximab was therefore introduced. Regular follow-ups revealed no inflammatory gingival changes even 6 years later.

Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.

BACKGROUND: Inherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting. METHODS: Almost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus. RESULTS: Recommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed. CONCLUSION: The recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

A case of inverse psoriasis successfully treated with adalimumab.

Inverse psoriasis is a rare form of psoriasis characterized by the involvement of skin fold areas rather than the more common psoriatic involvement of the extensor surfaces of the extremities, trunk, and scalp. In addition, it requires a modified therapeutic approach because it is often less responsive to standard treatment regimens. Current treatment recommendations for inverse psoriasis mainly consist of topical agents, including corticosteroids, calcipotriol, and immunomodulating agents, whereas systemic medications remain insufficiently studied. Although adalimumab, a TNF-α inhibitor, has been approved for the treatment of moderate to severe plaque psoriasis, some reports indicate that TNF-α inhibitors may sometimes trigger psoriatic lesions, including inverse psoriasis. However, we present a case of inverse psoriasis and psoriatic arthritis unresponsive to standard treatment that was successfully treated with adalimumab.

Figurate erythema in a patient with bullous pemphigoid and Toxocara infection.

Figurate erythema can appear in a wide spectrum of dermatological diseases. Rarely, it can present as an atypical manifestation of bullous pemphigoid. Among eosinophilic dermatoses, figurate erythema may appear in Wells syndrome, which has been occasionally reported in association with Toxocara infection. We present the case of an older female patient diagnosed with bullous pemphigoid, who presented with an unusual combination of blisters and figurate erythema outside the area of blister formation. In addition, high blood eosinophilia associated with lymph node and bone marrow eosinophilia was diagnosed and was causally related to Toxocara canis infection. The patient was treated with dapsone for bullous pemphigoid and with albendazole for toxocariosis, with complete regression of all skin lesions and blood eosinophilia. This paper discusses the possible etiopathogenesis of figurate erythema in our patient and summarizes previous clinical and histological findings in bullous pemphigoid and eosinophilic dermatoses presenting with figurate erythema lesions.

Infliximab for treatment of resistant pyoderma gangrenosum associated with Crohn's disease.

We present the case of an 18-year-old woman with Crohn's disease manifested by diffuse abdominal pain, bloody diarrhea accompanied by arthralgia, and swelling of large joints. On the lateral aspect of her right ankle there was an hemorrhagic, necrotic bullous lesion measuring 3 x 4 cm, surrounded by cutaneous inflammation and erythema. Biopsy showed a neutrophilic abscess-like ulcerative skin inflammation, which was diagnosed as pyoderma gangrenosum. The patient was treated with high doses of parenteral methylprednisolone, but her condition failed to improve and infliximab, a TNF-alpha blocking agent, was instituted. An immediate response of Crohn's disease was observed and, over the next 5 weeks, the ulcer on her right ankle also healed completely.

Density of Demodex folliculorum in perioral dermatitis.

The role of Demodex folliculorum in perioral dermatitis is not satisfactory explained. Our purpose was to assess the density of D. folliculorum in perioral dermatitis and evaluate the relationship of the mite count to previous therapy with topical steroids. A standardized skin surface biopsy of the chin was performed in 82 female patients with perioral dermatitis and in 70 control female subjects. Patients who received previous topical steroid therapy had a significantly higher mite density than the patients who had received no topical steroids (p<0.001). In the latter group of patients, the mite density did not differ significantly from that of the control group (p=0.629). Mite density increased significantly with the length of treatment with topical steroids (p<0.001). Our results suggest that increased density of D. folliculorum in perioral dermatitis is a secondary phenomenon, associated with topical steroid therapy.