Epidermolysis Bullosa Pruriginosa successfully treated with concomitant topical and systemic agents.

Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, manifests at birth or late in life and is characterised by intense pruritus, resulting in lichenified or nodular prurigo-like lesions and scarring most prominent on the shins. Treatment is unsatisfactory. We report a patient treated with success using a combination of topical and systemic agents.

Case report of recalcitrant allergic contact eczema successfully treated with teriflunomide.

Allergic contact dermatitis (ACD) is a type IV, delayed-type reaction caused by skin contact with low-molecular-weight organic chemicals and metal ions that activate antigen-specific T cells, primarily T-helper 1 (Th1), in a sensitized individual, leading to skin eczema.First-line treatments are based on avoidance of causal agents and topical corticosteroids/immunomodulators. In recalcitrant cases, chronic oral immunosuppressive agents may be used, but they may have serious adverse effects and do not address the immunological disfunction. We report a case of severe ACD, unresponsive to topical or oral immunosuppressive therapy, which resolved itself after treatment with teriflunomide (TF) 14 mg/daily used for multiple sclerosis. TF is a once-daily, oral selective and reversible dihydroorotate dehydrogenase inhibitor, revealing a new treatment option for ACD.

Pyodermatitis vegetans of the vulva.

Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. No standardized treatment plan is available for PV. Its evolution tends to follow the progression of the underlying disorder. We report a patient with vulvar PV with good response to systemic antibiotics and corticosteroids.

Photoallergic reaction to cyamemazine.

A 50-year-old man presented with a scaly erythema of the face, upper chest, forearms, and dorsum of the hands. He has been treated with cyamemazine for 6 months. Photopatch tests were performed and the patient was diagnosed with photoallergic reaction to cyamemazine. The drug was discontinued and a course of oral steroids was prescribed. The patient was advised to avoid light exposure. There has been no evidence of recurrence during a six-month follow-up period. Photoallergic reactions are much less frequent than phototoxic disorders. It is well known that several drugs including neuroleptics of the phenothiazine family may produce a skin eruption on light-exposed areas by dose-dependent (phototoxic) or photoallergic mechanisms. It is believed that photopatch testing, which is the clinical investigation of choice for suspected photoallergic reactions, is significantly underused in Europe and probably world-wide.

Vesico-bullous subacute cutaneous lupus erythematosus--an uncommon entity successfully treated with dapsone and hydroxychloroquine.

Vesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquine.

Letter: Pellagra as the initial presentation of Crohn disease.

Pellagra is a nutritional disease caused by the deficiency of niacin. We describe a case of pellagra as the initial presentation of Crohn disease, which has been rarely described in the literature.

Treatment of recalcitrant generalized granuloma annulare with adalimumab.

Granuloma annulare is a benign, usually self-limited, dermatosis of unknown cause. Generalized lesions occur in approximately 15 percent of patients with GA and may cause mild to severe cosmetic disfigurement. The treatment of generalized granuloma annulare can be challenging. We report the case of a 36-year-old male patient with a generalized granuloma annulare who had failed topical and systemic glucocorticoids, systemic retinoids, dapsone, minocycline, PUVA therapy, and hydroxicloroquine and was successfully treated with adalimumab, an anti-TNF-α monoclonal antibody. Adalimumab may be an additional option in the treatment of recalcitrant forms of granuloma annulare.

Lichen planus pemphigoides induced by a weight reduction drug.

Lichen planus pemphigoides is a rare autoimmune dermatosis characterized by bullous lesions arising on lichen planus (LP) papules and on clinically uninvolved skin, coexistence of histological features of LP and bullous pemphigoid and linear deposits of IgG and/or C3 along the basal membrane zone on direct immunofluorescence of peribullous skin. LPP has been reported to be associated with several medications such as ramipril, cinnarizine, simvastatin, captopril, psoralen ultraviolet A therapy and antituberculous medications. We report a case of a 41-year-old woman with LPP associated with a weight reduction drug.

Letter: Penile Kaposi sarcoma: A case of complete resolution with highly active antiretroviral therapy alone.

Kaposi sarcoma remains an important cause of morbidity in HIV-infected patients. Regardless of the recent pharmacological progress, treatment of this malignancy is still disappointing. We report the case of a patient with Kaposi sarcoma in an unusual localization, the penis, which completely resolved with highly active antiretroviral therapy alone.

Persistent varicella as the initial manifestation of systemic lymphoma.

Varicella is a common benign childhood disease that often presents in adolescents and adults in a more severe form. We report a previously healthy 50-year-old man who developed multiple necrotic cutaneous ulcers associated with fever, asthenia and anorexia. Physical examination revealed few tense hemorrhagic vesicles on the trunk and necrotic cutaneous ulcers scattered over the entire cutaneous surface. After the diagnosis of varicella with varicella pneumonia was established, treatment with acyclovir was instituted. His poor response to treatment was indicative of immune compromise; an underlying peripheral T-cell lymphoma was discovered.

Late onset pityriasis rubra pilaris type IV treated with low-dose acitretin.

Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. We report a 19-year-old male patient with a 2-week history of pruritic, scaling dermatosis of the hands, feet, elbows, and knees. He had no family history of skin disease. On physical examination, we observed circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma. The skin biopsy showed acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of pityriasis rubra pilaris. The patient started treatment with oral acitretin, 25 mg every other day. The treatment was tolerated well, and after 6 months the lesions had resolved completely. Pityriasis rubra pilaris is a chronic papulosquamous disorder of unknown pathogenesis, characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. There is still no consensus regarding the treatment, but therapeutic options include systemic retinoids, particularly acitretin in the recommended dose of 0.5 to 0.75 mg/kg/day. In our case, the patient was treated with a low-dose regimen of acitretin, which was effective and well tolerated.