Cutaneous Nodular Amyloidosis: A Disfiguring Aspect of the Face.

Primary localized cutaneous nodular amyloidosis is a rare plasma cell dyscrasia in which an amorphous material consisting of light chain amyloid is produced and deposited in the dermis, with varied clinical presentation. We describe the case with unusual and tumor lush clinical presentation in the face with no progression to systemic disease and no evidence of extracutaneous commitment.

[Disseminated necrobiotic xanthogranuloma]. / Disseminiertes nekrobiotisches Xanthogranulom.

Necrobiotic xanthogranuloma is a rare non-Langerhans-cell histiocytosis. A 62-year-old woman presented with yellowish erythematous plaques, nodules, and papules in the periorbital region and the extremities. She had a nodular tumor grown on the left upper lid that clinically resembled a keratoacanthoma. Histologically it was a xanthogranulomatous lesion. She suffered from monoclonal gammopathy of unknown significance of κ­type. Treatment was realized with a combination of systemic dapsone/prednisolone and topical corticosteroids.

Ingenol mebutate gel for actinic keratosis.

BACKGROUND: Actinic keratosis is a common precursor to sun-related squamous-cell carcinoma. Treating actinic keratoses and the surrounding skin area (i.e., field therapy) can eradicate clinical and subclinical actinic keratoses. Topical field therapy currently requires weeks or months of treatment. We investigated the efficacy and safety of a new topical field therapy for actinic keratosis, ingenol mebutate gel (0.015% for face and scalp and 0.05% for trunk and extremities). METHODS: In four multicenter, randomized, double-blind studies, we randomly assigned patients with actinic keratoses on the face or scalp or on the trunk or extremities to receive ingenol mebutate or placebo (vehicle), self-applied to a 25-cm(2) contiguous field once daily for 3 consecutive days for lesions on the face or scalp or for 2 consecutive days for the trunk or extremities. Complete clearance (primary outcome) was assessed at 57 days, and local reactions were quantitatively measured. RESULTS: In a pooled analysis of the two trials involving the face and scalp, the rate of complete clearance was higher with ingenol mebutate than with placebo (42.2% vs. 3.7%, P<0.001). Local reactions peaked at day 4, with a mean maximum composite score of 9.1 on the local-skin-response scale (which ranges from 0 to 4 for six types of reaction, yielding a composite score of 0 to 24, with higher numbers indicating more severe reactions), rapidly decreased by day 8, and continued to decrease, approaching baseline scores by day 29. In a pooled analysis of the two trials involving the trunk and extremities, the rate of complete clearance was also higher with ingenol mebutate than with placebo (34.1% vs. 4.7%, P<0.001). Local skin reactions peaked between days 3 and 8 and declined rapidly, approaching baseline by day 29, with a mean maximum score of 6.8. Adverse events were generally mild to moderate in intensity and resolved without sequelae. CONCLUSIONS: Ingenol mebutate gel applied topically for 2 to 3 days is effective for field treatment of actinic keratoses. (Funded by LEO Pharma; ClinicalTrials.gov numbers, NCT00742391, NCT00916006, NCT00915551, and NCT00942604.).

Dichotomous long-term response to isotretinoin in two patients with fordyce spots.

Fordyce spots are benign, enlarged sebaceous glands that commonly present as tiny yellow papules on a variety of body surfaces, including the lips. Although these lesions are asymptomatic, in some patients, they are abundant and conspicuous, leading to concerns regarding their cosmetic appearance. The response of Fordyce spots to isotretinoin therapy is poorly documented in the literature, which contains only one case report of an affected patient treated with a short course of isotretinoin (35 mg/kg in total). Herein, we report dichotomous long-term response to a standard course of isotretinoin in two patients with Fordyce spots of the lips and concomitant acne.

Vitiliginous lesions induced by amyl nitrite exposure.

Chemical (or contact) leukoderma is a condition induced by local cutaneous exposure to chemicals or medicaments that are toxic to melanocytes and/or koebnerize preexisting vitiligo vulgaris. Chemicals known to induce leukoderma include phenol/catechol derivatives (eg, hydroquinone), sulfhydryls, contact sensitizing agents (eg, squaric acid dibutylester), and more recently imiquimod, among others. We report the case of a 37-year-old black man with human immunodeficiency virus who developed chemical leukoderma in the nasal and perioral areas within 4 weeks of spilling liquid amyl nitrite, which he had been inhaling as a recreational drug, on his lower face. The depigmented regions were treated with a biweekly regimen of 308-nm excimer laser treatment for a total of 78 sessions. More than 90% cutaneous repigmentation was achieved. Amyl nitrite-induced vitiliginous lesions are rare. We also discuss potential mechanisms of hypopigmentation from chemical agents and therapeutic options for chemical leukoderma.

[Successful treatment of granulomatous rosacea with dapsone]. / Erfolgreiche Therapie einer granulomatösen Rosazea mit Dapson.

Granulomatous rosacea is an uncommon variant of rosacea. It is characterized by disseminated, red-brown papules and nodules especially in periocular and centrofacial locations. This form of rosacea causes considerable distress among patients because of the inflammatory facial lesions and represents a therapeutic challenge. A 62-year old man with granulomatous rosacea failed to improve with systemic doxycycline. Systemic isotretinoin and corticosteroids produced only a temporary reduction in cutaneous findings. Finally, systemic treatment with dapsone resulted in remission of the skin lesions and long-term stabilization.

[Morbihan disease as a special form of rosacea: review of pathogenesis and new therapeutic options]. / Morbus Morbihan als Sonderform der Rosazea: Einblick in die Pathogenese und neue Therapieoptionen.

Morbihan disease is classified as a special form of rosacea, which presents with persistent facial erythema and solid edema because of marked involvement of the lymphatic vessels. The cheeks, nose and forehead are particularly affected. Currently, the treatment options of this cosmetically very disturbing disease are limited. However, every attempt should be made to provide treatment because of the great emotional suffering of the patients. We review some new currently available therapeutic options for Morbihan disease. In our patient, we were able to achieve a significant improvement with systemic isotretinoin 30 mg/day over a period of 12 months.

[Lupoid perioral dermatitis as a special form of perioral dermatitis: review of pathogenesis and new therapeutic options]. / Lupoide periorale Dermatitis: Einblick in die Pathogenese und neue Therapieoptionen.

Lupoid perioral dermatitis is classified as a special form of perioral dermatitis with dense aggregations of red-brown papules which display a lupoid infiltrate on diascopy. There are various treatment options ranging from total avoidance of all topical substances to different topical treatment. Cases resistant to therapy require systemic treatment, which, however, is off-label use. In our case we were able to achieve a significant improvement with systemic therapy with isotretinoin over a period of 6 months.

[Lupoid rosacea as a special form of rosacea: review of pathogenesis and therapeutic options]. / Lupoide Rosazea als Sonderform der Rosazea: Einblick in die Pathogenese und Therapieoptionen.

Lupoid or granulomatous rosacea is classified as a special form of rosacea which along with the typical rosacea symptoms features monomorphic yellow-brown and red papules and nodules on the cheeks and periorificial areas. Histology shows noncaseating epithelioid cell granulomas with surrounding mononuclear cell infiltrates. It is difficult to treat. Currently, the only officially approved systemic treatment is tetracycline. However, there is a broad experience with off-label use of isotretinoin (10-20 mg daily). We describe a patient with an excellent response to 20 mg isotretinoin daily.

Childhood actinic lichen planus: successful treatment with antimalarials.

Actinic lichen planus (ALP) is a photosensitive variant of lichen planus, affecting mainly young adults of Middle Eastern descent. We report a case of ALP in a 9-year-old Colombian girl with an excellent response to hydroxychloroquine and photoprotection.

Acquired localized cutis laxa of the face: a rare presentation.

We report a rare case of acquired localized cutis laxa in a teenage boy, without any preceding skin lesions. The area affected was the midface, extending to the chin, and involving the ears, leading to a prematurely aged appearance. Only five such cases have been previously published in the literature.