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[Lupus miliaris disseminatus faciei: 6 cases].

BACKGROUND: Lupus miliaris disseminatus faciei is recognized by all authors as a unique entity, despite many appellations for this disease. In fact, the real origin and the treatment of this affection are unknown. In this study we try to specify the major histological and clinical points of diagnosis, the evolution in the mid-term and the different possibilities of the treatment. PATIENTS AND METHODS: This is a retrospective study of the patients diagnosed with lupus miliaris and followed-up by one of us (P.M.) between 1990 and 2000. RESULTS: There were 6 patients in this study, 5 males and 1 female. The mean age was 37 years old at the time of diagnosis. The patients exhibited a facial eruption, notably in the middle of the face, eyelids and peri-oral area. All the patients had a polymorph eruption of inflammatory and smooth papules. Many of these papules progressed in several weeks to round scars with a perpendicular carved shape. Histologically and depending of the stage of evolution of the lesion, there were some epithelioid cell and giant cell granulomas organized around the necrosis. All of the trial treatments led to failure or semi-failure. Four of the patients followed-up for several years spontaneously recovered from this disease within 2 to 4 years. DISCUSSION: Based on the anatomical and clinical similarities observed in these 6 patients, our study confirms the authenticity of the "lupus miliaris". It did not correspond to a particular form of acne, rosacea, tuberculosis or sarcoidosis. The scars are pathognomonical of this type of eruption. No treatment seems to be efficient. However our study seems to confirm the general idea of spontaneous recovery with 2 to 4 years.

Adult↗

[Facial rash].

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Adult↗

[Facial granuloma].

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Diagnosis, Differential↗

Varied clinical spectrum of necrobiotic xanthogranuloma.

Four cases are presented that illustrate a wide spectrum of ophthalmologic and systemic features of necrobiotic xanthogranuloma (NXG). Case 1 initially had signs of Cogan syndrome, and then developed chronic lymphocytic leukemia. Case 2, the first case of NXG to undergo autopsy, had progressive cicatricial lid retraction and corneal perforation. Case 3 had a more typical presentation of diplopia and blepharoptosis caused by orbital and periorbital infiltrative masses. Case 4 had nondeforming periocular skin lesions over a 6-year period. In all four cases, the diagnosis was made on the basis of characteristic histopathologic and laboratory findings. Although the cause of NXG is still obscure, in many cases it appears to be a forerunner of lymphoproliferative diseases.

Aged↗

The combination of 2% 4-hydroxyanisole (Mequinol) and 0.01% tretinoin is effective in improving the appearance of solar lentigines and related hyperpigmented lesions in two double-blind multicenter clinical studies.

BACKGROUND: Solar lentigines are a chronic condition of the aging population resulting from years of cumulative sun exposure. A topical treatment that is both safe and effective would be welcome and useful. Combinations of therapeutic agents are often used and allow synergy of mechanisms with tolerability. A tyrosinase inhibitor in use in Europe, 4-hydroxyanisole (Mequinol), and the retinoid tretinoin have been used singly as depigmenting agents. OBJECTIVE: The efficacy and safety of the combination product of 2% 4-hydroxyanisole (4HA [mequinol]) /0.01% tretinoin solution (tradename Solagé) were evaluated in two phase III, randomized, controlled, double-blind trials. METHODS: Subjects were randomized to treatment with 4HA/tretinoin solution, one of the active components (4HA or tretinoin), or vehicle. Subjects applied the test solution with a wand applicator twice daily to all solar lentigines and related hyperpigmented lesions on the face, forearms, and backs of hands for up to 24 weeks. Trial 1 had a 24-week no-treatment regression phase and trial 2 had a 4-week no-treatment regression phase. Information collected included clinical assessments of Target Lesion Pigmentation, Physician's Global Assessment of Improvement/Worsening, an Assessment of Overall Cosmetic Effect, and a Subject's Self-Assessment Questionnaire. RESULTS: The 4HA/tretinoin combination was clinically superior to each of its active components and to the vehicle in the treatment of solar lentigines. At the end of treatment, in trial 1 and trial 2, 4HA/tretinoin was statistically superior to each of its active components and vehicle on the forearms and face (P </=.03), except versus tretinoin on the face in trial 2 (P =.2). In trial 2, a trend toward greater efficacy of 4HA/tretinoin over tretinoin on the face was demonstrated at the end of treatment (P =.2), which was also increasingly evident at the end of the 4-week follow-up (P =.06). Most skin-related adverse events were mild and were similar for both the 4HA/tretinoin and tretinoin treatment groups. CONCLUSION: For the treatment of solar lentigines and related hyperpigmented lesions, the topical combination product containing 2% 4HA/0.01% tretinoin solution is well tolerated and superior to either active component.

Administration, Cutaneous↗

Pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent.

Pemphigus foliaceus is an autoimmune blistering disease of unknown origin with antibodies produced against desmoglein 1, an adhesive protein found in the desmosomal cell junction in the suprabasal layers of the epidermis. The disease is primarily treated with corticosteroids and corticosteroid-sparing immunosuppressive agents. We report a case of pemphigus foliaceus successfully treated with mycophenolate mofetil. It remains to be seen whether this agent has a significant effect on the course of the disease and remission induction.

Adult↗

Facial erythema as a result of benzophenone allergy.

Benzophenones are common causes of photoallergy and are increasingly used in products other than traditional sunscreens. Patients may be unaware of any sunscreen exposure when using a product such as shampoo containing benzophenone. Benzophenones also may produce photoallergic contact urticaria, in addition to delayed contact and photocontact dermatitis, which may complicate the clinical presentation. Allergy to benzophenone should be considered in the diagnosis of patients with patchy erythema of the face and neck that is not typically eczematous and that may otherwise be attributed to a rosacea diathesis, lupus erythematosis, or simple flushing. Patch and photopatch testing are indicated to evaluate these patients for allergy to benzophenone.

Adult↗

Juvenile colloid milium associated with conjunctival and gingival involvement.

Juvenile colloid milium is an uncommon cutaneous disease characterized by translucent papules distributed on sun-exposed areas with early onset. Association of juvenile colloid milium with conjunctival and gingival deposits is uncommon and interesting. We report a case of juvenile colloid milium associated with conjunctival and gingivai deposits of an amyloid-like homogeneous eosinophilic material. It seems that all 3 of these in our patient may be different expressions of the same pathologic disease.

Adult↗

Pseudoxanthoma elasticum: Temporary treatment of chin folds and lines with injectable collagen.

Pseudoxanthoma elasticum is characterized by calcification of dermal connective tissues. Typical rhytides and horizontal creases of the chin are cutaneous manifestations that begin at an early age and are often overlooked. We report 2 patients with pseudoxanthoma elasticum who had characteristic rhytides and horizontal creases of the chin ("mental" creases). Rhytides and horizontal creases were successfully treated with injectable collagen.

Collagen↗

Arcuate plantar plaques as the initial sign of chronic cutaneous lupus in a child.

A 9-year-old girl was initially seen with bilateral mildly pruritic plantar skin lesions. Skin biopsy demonstrated a superficial and deep perivascular lymphoid infiltrate with mucin but was not specific. Laboratory evaluation revealed a mildly elevated antinuclear antibody and mild leukopenia. Over the following year, an annular skin lesion developed in the preauricular area, her antinuclear antibody titer rose, and she was found to have positive anti-SS-B antibodies. She responded to oral hydrochloroquine with resolution of her skin lesions. This report highlights the unusual presentation of a case of chronic cutaneous lupus with a plantar skin eruption.

Antirheumatic Agents↗

Demodex abscesses: clinical and therapeutic challenges.

A 53-year-old man had a 6-week history of confluent erythematous papules, pustules, and abscesses of the face initially limited to the right nasolabial fold. Histologic investigation revealed a perifollicular infiltrate with lymphocytes, histiocytes, and many Demodex folliculorum mites. A large number of mites were seen in skin scrapings. The skin manifestations progressed and persisted for the following 5 years and were unresponsive to numerous antiparasitic treatments, including repeated oral administration of ivermectin and external application of lindane, permethrin, and benzoyl benzoate. Therapy with oral administration of 250 mg metronidazole 3 times a day for 2 weeks resulted in rapid and lasting recovery. Demodex folliculitis remains a diagnostic and therapeutic challenge. Antiparasitic therapies used against lice or scabies may fail in control of D folliculorum mites. In the presence of clinical and microscopic findings typical of Demodex folliculitis or abscesses, treatment failure with acaricidal agents does not exclude the diagnosis. Oral metronidazole seems to work in the management of this chronic mite infestation.

Administration, Oral↗

Hypocomplementemic urticarial vasculitis with angioedema, a rare presentation of systemic lupus erythematosus: rapid response to rituximab.

We report a case of hypocomplementemic urticarial vasculitis and recurrent angioedema in a patient with systemic lupus erythematosus unresponsive to mycophenolate mofetil, high-dose methylprednisolone, and intravenous immunoglobulin that responded rapidly to rituximab. Rituximab is a monoclonal antibody against CD20 transmembrane protein on the surface of mature and malignant B cells. No adverse effects occurred during or after therapy, and the patient was discharged from the hospital for outpatient rituximab infusion and follow-up care.

Adult↗

Idiopathic eruptive macular pigmentation: a case of 21 years' duration.

Idiopathic eruptive macular pigmentation is a rare condition characterized by asymptomatic pigmented macules involving the neck, trunk, and proximal portions of the extremities. Age at onset usually varies from 1 to 20 years. The lesions usually appear abruptly and remit spontaneously over months to years. An unusual case of a 24-year-old woman with idiopathic eruptive macular pigmentation lasting 21 years was characterized by several periods of spontaneous resolution followed by recurrences.

Adult↗