Background:Erosive oral lichen planus and desquamative gingivitis are uncommon but severe debilitating variants of oral lichen planus. Treatment of these presentations is difficult and challenging.Main observation:A 44-year-old woman was referred to the dermatology clinic with chronic painful lichen planus-related gingivitis and buccal erosions. She has failed multiple treatments including topical clobetasol and tacrolimus, intralesional corticosteroids and several systemic and immunosuppressive agents. Following completion of three months of treatment with oral apremilast at a dose of 30 mg twice daily, significant improvement was noted in her disease activity.Conclusion:Oral apremilast may be a safe and effective treatment for erosive oral lichen planus.

Background:Hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC) is an autosomal dominant syndrome due to mutation in fumarate hydratase. Patients with HLRCC frequently develop cutaneous and uterine leiomyomata and are at risk for renal cell carcinoma. Rarely, other malignancies have been reported.Main observations:We report the development of basal cell carcinoma and melanoma in two siblings with genetically-confirmed HLRCC.Conclusions:It is unclear whether the development of melanoma and basal cell carcinoma in our patients is due directly to their mutations in the gene encoding fumarate hydratase, or genetic susceptibility at another unrelated locus, or whether these are incidental lesions. However this observation has implications for careful and routine skin surveillance in patients with HLRCC for lesions other than cutaneous leiomyomata.

Background:Nevogenesis is a complex process involving several pathogenetic mechanisms, including genetic factors, hormonal influences and UV-radiation. Trauma has been described as a triggering factor for an alternative pathway of nevogenesis. Eruptive melanocytic nevi (EMN), related either to immunosuppression or to blistering disorders, represent a special type of nevi probably induced by the disruption of the dermo-epidermal junction and consequent proliferation of quiescent pigment cells during re-epithelization.Main observations:We report two patients with three melanocytic nevi that developed de novo along the direction of surgical suturing, following surgical operation for other reason. The lesions exhibited special dermoscopic characteristics and histology revealed features of acquired melanocytic nevi.Conclusions:Such cases may represent a new type of eruptive nevus, the surgical suturing-induced nevus, which should be included in the differential diagnosis of new pigmentation developing within a scar.

Background:Epidermolysis bullosa simplex associated with muscular dystrophy is a genetic skin disease caused by plectin deficiency. A case of a 19-year-old Czech patient affected with this disease and a review all previously published clinical cases arepresented.Main observations:In our patient, skin signs of the disease developed after birth. Bilateral ptosis at the age of 8 years was considered as the first specific symptom of muscular dystrophy. Since then, severe scoliosis, urological and psychiatric complication have quickly developed. The signs of plectin deficiency were found by histopathological studies, electron microscopy and antigen mapping of the skin and muscular samples. Two autosomal recessive mutations in the plectin gene leading to premature termination codon were disclosed by mutation analysis. By review of all published clinical cases, 49 patients with this disease were found. 54 different mutations in the plectin gene were published, p.(Arg2319*) in exon 31 being the most frequently found. Median age of muscular dystrophy development was 9.5 years. Hoarseness and respiratory complications were the most often complications beside skin involvement.Conclusion:Epidermolysis bullosa simplex with muscular dystrophy was diagnosed based on clinical, histopathological (skin and muscle biopsy) and mutation analysis of the plectin gene. Overview of the genetic and clinical characteristic of this disease could be presented by review of all previously published clinical cases.