Nevus depigmentosus: review of a mark of distinction.

Nevus depigmentosus (ND), also known as nevus achromicus or achromic nevus, is an uncommon congenital hypomelanosis of the skin that is often characterized as being nonprogressive and having serrated borders. It needs to be distinguished from other hypopigmented skin conditions such as nevus anemicus, hypomelanosis of Ito, Fitzpatrick patches (ash leaf spots) of tuberous sclerosis, vitiligo, indeterminate leprosy, and pigment demarcation lines. Treatment may be desired for aesthetic and possible psychosocial considerations. We review and update knowledge about ND and its simulants.

The Medical Treatment of Vitiligo: An Historical Review.

Discolorations of the skin, such as vitiligo, were recognized thousands of years ago. White spots caused by vitiligo and other disorders have caused significant social opprobrium to those disfigured by these pigmentary disorders, throughout history and still in the present day. Treatments have been desperately sought with only partial success. Recent advances suggest that vitiligo and other pigmentary disorders might soon be curable.

Atypical presentations of eosinophilic fasciitis.

Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus. In view of the overlapping clinical and histopathological features of these disorders, MRI may be helful in delineating the conditions by detecting involvement of fascia.

Guidelines for bath PUVA, bathing suit PUVA and soak PUVA.

BACKGROUND: The aim of these guidelines is to encourage dermatologists to use bath psoralen plus ultraviolet A (PUVA), bathing suit PUVA and soak PUVA in the treatment of psoriasis vulgaris and other conditions. METHODS: Evidence was collected using searches of the PubMed, MEDLINE and COCHRANE databases using the keywords "bath PUVA," "soak PUVA," "bathing suit PUVA" and "turban PUVA." Only publications in English were reviewed. RESULTS: One hundred and thirty-eight studies were evaluated, 57 of which fulfilled the criteria for inclusion. CONCLUSIONS: Both bath PUVA and bathing suit PUVA are very effective and safe treatments for generalized stable plaque psoriasis (strength of recommendation, A). Soak PUVA is very effective in the treatment of both palmoplantar psoriasis and chronic palmoplantar eczema (strength of recommendation, A).

Phototherapy for mycosis fungoides.

BACKGROUND: Both phototherapy and photochemotherapy have been used in all stages of mycosis fungoides since they improve the symptoms and have a favourable adverse effect profile. MATERIALS AND METHODS: We performed an extensive search of published literature using keywords like "phototherapy", "photochemotherapy", "NBUVB", "PUVA", "UVA1", "mycosis fungoides", and "Sezary syndrome", and included systematic reviews, meta-analysis, national guidelines, randomized controlled trials (RCTs), prospective open label studies, and retrospective case series. These were then arranged according to their levels of evidence. RESULTS: Five hundred and forty three studies were evaluated, of which 107 fulfilled the criteria for inclusion in the guidelines. CONCLUSIONS AND RECOMMENDATIONS: Photochemotherapy in the form of psoralens with ultraviolet A (PUVA) is a safe, effective, and well tolerated first line therapy for the management of early stage mycosis fungoides (MF), that is, stage IA, IB, and IIA (Level of evidence 1+, Grade of recommendation B). The evidence for phototherapy in the form of narrow-band UVB (NB-UVB) is less robust (Level of evidence 2++, Grade of recommendation B) but may be considered at least as effective as PUVA in the treatment of early-stage MF as an initial therapy. In patients with patches and thin plaques, NB-UVB should be preferentially used. PUVA may be reserved for patients with thick plaques and those who relapse after initial NB-UVB therapy. For inducing remission, three treatment sessions per week of PUVA phototherapy or three sessions per week of NB-UVB phototherapy may be advised till the patient achieves complete remission. In cases of relapse, patients may be started again on PUVA monotherapy or PUVA may be combined with adjuvants like methotrexate and interferon (Level of evidence 2+, Grade of recommendation B). Patients with early-stage MF show good response to combination treatments like PUVA with methotrexate, bexarotene or interferon-α-2b. However, whether these combinations hold a significant advantage over monotherapy is inconclusive. For late stage MF, the above-mentioned combination therapy may be used as first-line treatment (Level of evidence 3, Grade of recommendation C). Currently, there is no consensus regarding maintenance therapy with phototherapy once remission is achieved. Maintenance therapy should not be employed for PUVA routinely and may be reserved for patients who experience an early relapse after an initial course of phototherapy (Level of evidence 2+, Grade of recommendation B). Bath-water PUVA may be tried as an alternative to oral PUVA in case the latter cannot be administered as the former may show similar efficacy (Level of evidence 2-, Grade of recommendation C). In pediatric MF and in hypopigmented MF, both NB-UVB and PUVA may be tried (Level of evidence 3, Grade of recommendation D).

Photochemotherapy (PUVA) in psoriasis and vitiligo.

Phototherapy with photochemotherapy (PUVA) is a well-known and well-studied modality for the treatment of psoriasis, which involves systemic or topical administration of chemicals known as psoralens and administration of ultraviolet light in increasing dosages after requisite time gap. PUVA is also used in the treatment of widespread vitiligo with moderately good results, though it is being surpassed by ultraviolet B (UVB), which is equally or slightly more efficacious with fewer side effects. PUVA induces repigmentation by varying mechanisms such as stimulation of melanogenesis, immunomodulation and activation of growth factors, though the exact mechanism is still speculative. There are various studies evaluating the efficacy of PUVA in psoriasis as well as in vitiligo, either alone or in combination with other immunosuppressants like azathioprine and calcipotriene.

Comparative evaluation of NBUVB phototherapy and PUVA photochemotherapy in chronic plaque psoriasis.

BACKGROUND: Psoralen UV-A (PUVA) is an established therapy for psoriasis, but there is a well-documenated risk of melanoma and nonmelanoma skin cancer. Narrow-band Ultraviolet-B (NBUVB) therapy has a lower carcinogenic risk, has equal therapeutic potential and is considerably safe in the long term than PUVA. AIM: The aim of present study was to compare the efficacy and side-effects of PUVA and NBUVB in chronic plaque psoriasis. METHODS: Sixty patients of chronic plaque psoriasis were taken up for the study and were randomly divided into two groups of 30 each. They were well matched in terms of age, sex, psoriasis extent and pretreatment psoriasis area severity index (PASI) scoring. One group was treated with twice-weekly narrow-band UV-B (TL-01) phototherapy and the other group received twice-weekly oral 8-Methoxsalen PUVA for a period of 3 months. RESULTS: Both the groups achieved >75% reduction in the PASI score or complete clearance at the end of 3 months, but PUVA group patients required significantly fewer number of treatment sessions and fewer number of days to clear the psoriasis as compared to the NBUVB group, while the mean cumulative clearance dose and adverse effects were significantly lower in the NBUVB group. CONCLUSION: We concluded that PUVA group patients achieved a faster clearance, but the adverse effects were significantly lower in the NBUVB group.

Micosis fungoides en niños / Mycosis fungoides in children

La micosis fungoides rara vez se describe en la niñez, aunque es el tipo más frecuente de linfoma cutáneo de células T en esta edad, en la que predomina el inmunofenotipo CD8+. Se manifiesta con parches hipopigmentados, por lo que se debe hacer diagnóstico diferencial con otras lesiones hipopigmentadas, como vitiligo, pitiriasis versicolor y lepra, entre otras. En niños es más común que se presente clínicamente en los estadios iniciales de mancha o placa, lo que hace que su pronóstico sea bueno. Las terapias más utilizadas para el tratamiento en niños son los corticosteroides tópicos de alta potencia y la fotoquimioterapia PUVA.

Is CCR7 a potential target for biologic therapy in psoriasis? Increased expression of CCR7 in psoriasis vulgaris.

BACKGROUND: Activated T cells present in psoriatic plaques play a key role in the pathogenesis of psoriasis. CCR7 on T cells plays a crucial role in native immune response and formation of secondary lymphoid organ. AIMS: To determine whether differential expression and functions of the CCR7 occur in psoriasis patients in China, we examined CCR7 on T cells from normal and psoriasis subjects. METHODS: Skin specimens and T cells from 33 patients and 22 healthy controls were analyzed by immunohistology, flow cytometry, and RT-PCR. RESULTS: Patients with psoriasis had a skewed distribution of T lymphocytes, with an increased level of CCR7+ T lymphocytes compared to healthy controls (P<0.01) By flow cytometry, it was found that CCR7 was selectively, frequently, and functionally expressed on CD4+ (20.5+/-6.8%)but not on CD8+ (9.5+/-3.4%) T cells from patients with psoriasis, whereas this phenomenon was not seen in normal subjects. Through RT-PCR it was also found that CCR7 was highly expressed on T cells in patients with psoriasis than in healthy controls in the level of gene. CONCLUSIONS: Patients with psoriasis had a skewed distribution of T lymphocytes, with an increased level of CCR7+ T lymphocytes compared to healthy controls. CD4+ CCR7+ T cells had abnormal expression, which might induce protraction and persistence of psoriasis.

An open labeled, comparative clinical study on efficacy and tolerability of oral minipulse of steroid (OMP) alone, OMP with PUVA and broad / narrow band UVB phototherapy in progressive vitiligo.

BACKGROUND: Several modalities of treatment have been tried in vitiligo with varied results; however, Indian data on comparative studies of two or more therapies are limited. AIMS: We compared different phototherapy methods with an oral steroid as an adjunct to determine the method with the best tolerability and efficacy. METHODS: Eighty-six patients with progressive vitiligo were randomly assigned to different study groups according to a continuous selection method over a period of one year. Group 1 was given OMP + PUVA, group 2 OMP + UVB (NB), group 3 OMP + UVB (BB) and group 4 was given OMP alone. Each patient was followed up for six months and then released from treatment. Clinical evaluation was made at the end of three and six months. RESULTS: In group 1 (OMP + PUVA), marked improvement was seen in 18.51% while moderate improvement was seen in 66.66% of the patients. Marked improvement was seen in 37.03% in group 2 (OMP + NB-UVB) while 44.44% had moderate improvement. In group 3 (OMP + BB UVB), 8.33% showed marked improvement while moderate improvement was seen in 25% of the patients. Marked and moderate improvement was seen in 5 and 10% of group 4 (OMP) patients, respectively. CONCLUSIONS: Our study compared four treatment modalities in vitiligo patients, out of which oral minipulse of steroids (OMP) only had an adjunct value and was not very effective by itself. Narrow band UVB has a definite edge over broad band UVB and should be preferred when both options are available. NB-UVB and PUVA showed comparable efficacy.

DermaMan: scoring dermatology in your palm.

Personal Digital Assistants (PDAs) have become a part of everyday life. DermaMan is a freely available, Java-based, dermatology-specific calculator for handheld devices. It includes modules to compute PASI, MASI, SCORAD, and for calculations related to topical PUVA and Botox (R) administration.

Keratinocyte-melanocyte graft technique followed by PUVA therapy for stable vitiligo.

BACKGROUND: Various surgical procedures for correcting stable vitiligo exist but these have their own limitations. Autologous, non-cultured, non-trypsinized, melanocyte plus keratinocyte grafting is a new and simple method of vitiligo surgery. OBJECTIVE: The study aimed to evaluate efficacy of a new grafting technique in vitiligo patches. METHODS: Eighteen vitiligo patches underwent this procedure. The upper layer of epidermis was removed by superficial dermabrasion using a dermabrader micromotor until the epidermis appeared wet and shiny. Then, antibiotic ointment was applied and dermabrasion was continued up to the whitish area of the upper dermis. The paste-like material (ointment with entangled epidermal particles) was collected and spread over the dermabraded recipient site. RESULTS: Pigmentation usually started at 4-6 weeks. Complete uniform pigmentation took 16-20 weeks. CONCLUSION: For smaller vitiligo patches this method gives cosmetically acceptable results. It is easy to perform and does not require specific laboratory setup.

Psoralene in repigmentation of tuberculoid leprosy.

Photoactivated psoralens were studied in sixty cases of tuberculoid leprosy for the repigmentation of hypopigmented macules. It was observed that in 42 (70%) cases there was significant repigmentation within 6 months of therapy; in 5 (8.33%) cases there was mild to moderate repigmentation and in 13 (22.64%) cases, there was no change. No significant untoward effects of drug were encountered, except in four patients (6.66%), who developed, marked erythema and eczematous reaction.