Hypopigmented mycosis fungoides: a retrospective clinicohistopathologic study.

IMPORTANCE: Hypopigmented mycosis fungoides is a rare variant of mycosis fungoides with limited published clinicohistopathologic data available. OBJECTIVE: To characterize our patient group, to provide additional information and insight into this malignancy. DESIGN: A 16-year retrospective medical records review (from 1992 to 2009) was conducted of patients with a diagnosis of hypopigmented mycosis fungoides. SETTING: All patients were seen in the department of dermatology at Howard University Hospital, an outpatient clinic in an urban academic institution. PARTICIPANTS: The review comprised of 20 patients. Inclusion required presence of hypopigmented skin lesions and a skin biopsy diagnostic for hypopigmented mycosis fungoides. INTERVENTIONS: Treatment modalities, including oral psoralen with UVA, narrow-band UVB and/or topical medications such as nitrogen mustard and topical corticosteroids were employed. RESULTS: Patients ranged from 4 to 57 years old. Fifteen were African American, three African, one Afro-Caribbean and one Hispanic. The interval from disease onset to diagnosis ranged from 7 months to 24 years. Patients presented at Stage 1A or 1B. Treatment included phototherapy and topical medications. In four patients with pre- and post-treatment biopsies, the original histological diagnosis of hypopigmented mycosis fungoides and the subsequent complete resolution were shown. There was no associated mortality in the patients studied. CONCLUSIONS AND RELEVANCE: Hypopigmented mycosis fungoides affected skin of colour patients in this study. This variant differs from classic mycosis fungoides: younger population, slower progression and the majority of patients remaining in Stage I with treatment. We observed that any repigmentation of lesions suggests an effective treatment regimen, complete repigmentation correlates with clinical and histopathologic resolution, and new hypopigmented lesions during remission suggest relapse. A limitation of this study is the small sample size. This is the first study to correlate the histological resolution of hypopigmented mycosis fungoides with clinical repigmentation of lesions.

Topical treatment and combination approaches for vitiligo: new insights, new developments.

Despite much research done involving elucidation of the pathogenesis of vitiligo, a precise cause is still not known. Prevalent hypotheses include the autoimmune, genetic, neural, self-destruction, growth factor deficiency, viral, and convergence theories, which have served as the basis for treatment formulation. Topical therapies have been a mainstay of vitiligo treatment, with or without phototherapy. Topical treatments used in the treatment of vitiligo include steroids, calcineurin inhibitors, vitamin D analogues, pseudocatalase, and depigmenting agents. Combination therapies are used to improve the success rate of repigmentation. In this article, we have examined randomized controlled trials utilizing topical treatments used as monotherapy or combination therapy. Although psoralen and khellin can be used as topical agents, used in conjunction with UV radiation, we have not included them in the review due to their inability to be used as monotherapy. We have also excluded less used or ineffective topical agents, such as melagenina, topical phenylalanine, topical L-DOPA, coal tar, anacarcin forte oil and topical minoxidil. According to current guidelines, a less than two month trial of potent or very potent topical corticosteroids or topical calcineurin inhibitors may be used for therapy of localized vitiligo (<20% skin surface area). Combinations of topical corticosteroids with excimer laser and UVA seem to be more effective than steroids alone. Pseudocatalase plus NB-UVB does not seem to be more effective than placebo with NB-UVB. Combinations of vitamin D analogues have varied efficacy based on which type is used and the type of UV light. Efficacy of calcineurin inhibitor combinations also vary based on the type used and UV light combined, with tacrolimus being more effective with excimer laser. Pimecrolimus has been effective with NB-UVB and excimer laser on facial lesions, and microdermabrasion on localized areas.

8-Methoxypsoralen levels in blood of vitiligo patients and in skin, ophthalmic fluids, and ocular tissues of the guinea pig.

8-Methoxypsoralen (8-MOP) levels in the blood of vitiligo patients were determined through the use of a reverse-phase high-performance liquid chromatographic method. The overall recovery of the internal standards was 85-94%, with the lower detection limit of 8-MOP at 2 ng. Peak blood levels as low as 130 ng/ml and as high as 3892 ng/ml were obtained in patients at 1-3 h following the oral administration of 0.6 mg/kg body weight of Oxsoralen capsules (Elder Pharmaceuticals Co.). These results are consistent with the clinical observation that maximum response in phototherapy is obtained at about 2 h after oral administration of the drug. Two hours after oral administration of 0.6 mg/kg of Oxsoralen, 8-MOP levels in the epidermis, dermis, and whole skin of the guinea pig (in ng/g) were: epidermis, 330 +/- 20; dermis, 89 +/- 16; whole skin, 379 +/- 19. Also detected were 8-MOP levels of 441 +/- 22 ng/ml in aqueous humor, 166 +/- 18 ng/ml in vitreous gel, 355 +/- 15 ng/g in lens, and 410 +/- 26 ng/g in retina. These results point to the fact that the eyes of the patient must be protected from exposure to sunlight after psoralen UV treatment, and that 8-MOP is absorbed in blood unevenly and varies from patient to patient. The fact that only 50-60% of the patients responded to psoralen photochemotherapy for vitiligo may be related to the variation of absorption of the drug in individual patients.

Vitiligo is associated with HLA-DR4 in black patients. A preliminary report.

We have determined the HLA-DR and HLA-DQ phenotypes of 24 black patients with vitiligo and compared these with phenotypes of 143 local black controls. HLA-DR4 was significantly increased in patients, 38% vs 11% for controls. HLA-DQw3 was also increased in patients, 58% vs 32% for controls and may be explained in part by linkage disequilibrium with HLA-DR4. When patients were subgrouped according to family history of autoimmune disease and compared with controls, the increase in HLA-DR4 and HLA-DQw3 segregated with a positive family history. HLA-DRw6 in patients with a negative family history of autoimmune disease (64%) was significantly greater than the 10% in patients with a positive family history. When patients were subgrouped according to age at onset of disease, HLA-DR4 was increased in those with early onset of disease (younger than 20 years) while HLA-DRw6 was greater in patients who were older at onset of disease. These findings support the hypothesis of an immunogenetic influence on the expression of vitiligo in black patients with vitiligo.

Autoantibodies and their clinical significance in a black vitiligo population.

The frequency of autoantibodies was determined in 70 black vitiligo patients and controls. Both groups were screened for antithyroid, antinuclear, antigastric parietal cell, anti-smooth muscle cell, and antimitochondrial autoantibodies. The significance of autoantibodies was determined in vitiligo patients by correlating their presence or absence with various clinical features of the patients. The overall frequencies of autoimmune and endocrine diseases were also assessed in vitiligo patients, controls, and their respective families. Vitiligo patients had an increased frequency of antithyroid antibodies and an increased frequency of autoimmune and/or endocrine diseases. These diseases included, especially, hyperthyroidism, hypothyroidism, and alopecia areata. Autoantibody-positive vitiligo patients had an increased frequency of first- and second-degree relatives having autoimmune and/or endocrine diseases. These findings tend to support an autoimmune cause of vitiligo in black patients.

Pseudofolliculitis barbae and related disorders.

Pseudofolliculitis barbae, although not a serious medical problem, is certainly a distressing one for the affected patient. Its pathogenesis lies in an ingrown hair arising from the curved hair and follicle common in black men and women. Improper shaving techniques cause ingrown hairs through both transfollicular and extrafollicular mechanisms. Various treatment modalities exist, but there is no cure. Treatment must be individualized, as not all regimens will work for each patient. With diligence, pseudofolliculitis barbae can in many instances be controlled. Dermatitis papillaris capillitii is related to pseudofolliculitis barbae because its pathogenesis also lies in a curved hair and follicle. The treatment differs, however. Mild to moderately severe cases can be kept under good control with intralesional injections of steroid and a topical chloramphenicol and steroid cream mixture. Scarred or keloidal lesions may require surgery.

Skin cancer in blacks in the United States.

Skin cancer is rare in blacks compared with whites in the United States. The most common form is squamous-cell carcinoma, not basal-cell carcinoma, as it is in whites. Sunlight does not appear to be an important etiologic factor in skin cancer in blacks, as most lesions occur on covered areas. Malignant melanoma is low in frequency but commonly affects acral areas and has a poor prognosis. Mycosis fungoides and dermatofibrosarcoma protuberans appear to have a high frequency among skin cancers. Squamous-cell carcinoma, malignant melanoma, and mycosis fungoides have a relatively high mortality rate in blacks. Bowen's disease and Kaposi's sarcoma occur in blacks but are rare. As there is a high frequency of squamous-cell carcinoma of the skin in blacks, prevention and early detection should benefit the patient. Considering the difficulties encountered in applying epidemiologic methods to skin cancer on a national scale, etiologic studies should be conducted in carefully selected areas. Future investigations of skin cancer in blacks should include an examination of risk factors such as burns, trauma, and diet and familial and immunologic aspects as well.

New and emerging therapies for vitiligo.

Vitiligo is a common skin disease; however, it still remains a difficult disease to treat. Not all patients respond to current forms of treatment. There are several new treatments, surgical and nonsurgical, and immunologic, that appear to either have higher success rates than past therapies or have potential as future developments for therapy of vitiligo.

Management of vitiligo.

Vitiligo is a disease of unknown origin that causes destruction of melanocytes in the skin, mucous membranes, the eyes, and occasionally in hairbulbs and in the ears. The loss of melanocytes alters both structure and function of these organs. The goals of therapy are multifold. The primary goal is to restore melanocytes to the skin so that the epidermis has a normal morphology. Such repigmented skin regains its normal immune/inflammatory functions.

Skin cancer in black Americans: a review of 126 cases.

Primary cancer of the skin is rare in blacks. The records of 126 black patients with skin cancer were reviewed. Histopathologic findings included squamous cell carcinomas (43) basal cell carcinomas (39) malignant melanomas (8) dermatofibrosarcomas (16) Bowen's disease (6) mycosis fungoides (14) and sebaceous cell carcinoma (1).There is a higher percentage of skin cancer involving covered areas in blacks than among whites. Squamous cell carcinoma was the most common skin cancer in blacks. The distribution of basal cell carcinoma in blacks was 30 percent in this series, as compared with 80 percent in whites in the 1977 to 1978 survey. The majority of patients with squamous cell carcinoma had associated predisposing conditions and lesions on non-sun-exposed skin. Sunlight and occupational chemical exposure did not appear to be associated with skin cancer in blacks in this series.

Hair and scalp disorders in blacks.

Hair and scalp disorders in blacks and the properties of hair in blacks that make it behave differently are described. Hair disorders commonly seen now are the result of permanent and relaxer damage, heat, and traction alopecias. Presently, the most common scalp disorders in blacks are keloidal folliculitis and dissecting cellulitis of the scalp. Treatment modalities for these disorders are reviewed.

Incidence of common dermatoses in a predominantly black dermatologic practice.

A study was done to determine the frequency of common dermatoses seen in private dermatology practices composed of predominantly black patients. This was then compared to similar previous studies. New trends in common dermatoses in private black patients were found. The most common dermatosis noted was acne vulgaris, followed by eczema, pigmentary disorders, seborrheic dermatitis, and alopecias.

Topical agents used in the management of hyperpigmentation.

Disorders of hyperpigmentation are difficult to treat, particularly in dark-skinned individuals. The goal is to reduce the hyperpigmentation without causing undesirable hypopigmentation or irritation in the surrounding normally pigmented skin. The psychosocial impact caused by these disorders must be considered. Although there are many effective therapeutic modalities available, there are potentially significant side-effects associated with treatment. The most commonly used treatment is topical hydroquinone. There are other phenolic agents, such as N-acetyl-4-cystaminylphenol (NCAP), that are currently being studied and developed. The non-phenolic agents, which include tretinoin, adapalene, topical corticosteroids, azelaic acid, arbutin, kojic acid, and licorice extract, are also used for hyperpigmentation disorders.

Topical PUVA therapy for vitiligo.

Vitiligo, a pigmentary disorder of the skin, causes the appearance of white patches which are profoundly devastating to the person afflicted with this disease. This article outlines the safe use of topical PUVA therapy in the office for treating those patients with less than 20% skin surface involvement. This treatment has a response rate of approximately 50%.

Skin cancer in African Americans.

Skin cancer is the most common type of malignancy in the United States. Incidence within the African American population remains relatively low, but data is limited for this racial group, making accurate determination of incidence and mortality difficult. Factors implicated as causative in the pathogenesis of cutaneous malignancy in African Americans include, but are by no means limited to, sunlight, albinism, burn scars, X-rays, preexisting pigmented lesions, chronic inflammation, and chronic discoid lupus erythematosus. Anatomic distribution of lesions may be similar to that seen in whites for basal cell carcinoma but not for other skin cancers. For squamous cell carcinoma, melanoma, and cutaneous T-cell lymphoma, African Americans do not as well in terms of mortality as do whites. This difference probably is due either to the fact that African Americans have more advanced stages of disease at diagnosis than do whites or, in some cases, because the course of the disease is more aggressive in African Americans for reasons yet unknown. There is a need for heightened awareness of skin cancer in African Americans by patients and physicians. Emphasis should be on education and early diagnosis with the primary goal in mind being the reduction of incidence of and mortality due to skin cancer in African Americans. In addition, because of environmental factors, African Americans will be exposed to more solar ultraviolet radiation in the future. Strategies should be developed for public education to keep this exposure to low levels in this racial group.

Micropigmentation for the treatment of vitiligo.

A technique of permanent dermal micropigmentation using a nonallergenic iron oxide pigment to cover recalcitrant areas of vitiligo is described. The areas included in this study were the distal digits, the lips, hands, wrists, axillae, elbows, hairline, perioral area, and lower legs. The immediate postmicropigmentation results invariably showed dramatic aesthetic improvements. There was a moderate degree of fading in the majority of cases, most of which occurred within the first six weeks. The pigment that remained usually persisted with minimal to no further fading. Short- and long-term complications, which were few, mild, and resolved fully, are discussed. No allergic reactions to the pigment or koebnerization of the vitiligo have been noted.

Palmoplantar psoriasis: experience with 8-methoxypsoralen soaks plus ultraviolet A with the use of a high-output metal halide device.

The use of 8-methoxypsoralen water soaks plus ultraviolet A was evaluated retrospectively in 11 patients with moderate to severe palmoplantar psoriasis that had been resistant to previous topical therapies. Patients received twice- or thrice-weekly 30-minute hand and/or foot soaks in 8-methoxypsoralen, 2.5 mg/L, followed by UVA irradiation. The condition of one patient cleared completely, and seven showed good improvement. The range of treatments required for maximal improvement was 14 to 43 with a mean of 28. The range of maximum UVA dosage per treatment was 3.5 to 17.5 joules/cm2, and the range of cumulative UVA required for maximal improvement was 45 to 388 joules/cm2 with a mean of 165 joules/cm2. The mean serum level of 8-methoxypsoralen 60 minutes after completion of hand and foot soaks was 2.5 +/- 0.5 ng/ml compared with 95.75 +/- 10.43 ng/ml after oral 8-methoxypsoralen, 0.5 mg/kg. In our experience this treatment has been a safe and relatively effective form of therapy for resistant palmoplantar psoriasis.

Childhood vitiligo.

Eighty-two children (ages 6 months to 12 years) with clinical and/or histopathologic diagnoses of vitiligo were evaluated; 35 were male and 47, female. Fifty-six were black, 25 white, and 3 classified as "other." Children were compared with control groups of children with other skin diseases and with adults with vitiligo. Children had an increased incidence of segmental vitiligo (p less than 0.01). Children had an increased incidence of autoimmune and/or endocrine disease and also of premature graying in their immediate and extended family members (p less than 0.001). Six of 33 children with vitiligo tested had positive organ-specific serum autoantibodies, which was a higher incidence than in the control group of children (p less than 0.05). Eighteen percent of children treated with topical psoralens and long-wave ultraviolet light (PUVA) therapy had an acceptable response, which was less than an adult group similarly treated. We have found childhood vitiligo to be a distinct subset of vitiligo, showing increased segmental presentation; strong autoimmune and/or endocrine disease background and high incidence of premature graying in the families of affected children; the presence of organ-specific serum autoantibodies and a poor response to topical PUVA therapy.