Faculty Bibliography

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare disorder of keratinization involving the intraepidermal eccrine duct (acrosyringium). We detail two examples of this unique clinicopathological entity--one with a more typical clinical presentation and one with a solitary lesion and late adult onset. In addition, we discuss the distinctive histologic and immunohistochemical findings and review the literature

Chronic actinic dermatitis is a photodistributed, eczematous dermatitis that preferentially affects elderly men and persists for months to years. Its occurrence in individuals infected with human immunodeficiency virus (HIV) has been described in five patients. We report four additional cases of this uncommon, chronic photodermatosis associated with HIV infection. In two of the patients, photosensitivity was a presenting disorder leading to the diagnosis of HIV infection. All patients were men of skin type VI with a mean age of 50 years, all had decreased minimal erythema doses to ultraviolet B, three of the four patients had decreased minimal erythema doses to ultraviolet A and all had CD4 cell counts of < 200 x 10(6)/L

BACKGROUND: Pruritus in patients positive for HIV may be debilitating. OBJECTIVE: Our purpose was to evaluate the efficacy of UVB therapy in the treatment of pruritus in patients positive for HIV. METHODS: Twenty-one male HIV-positive patients with intractable pruritus (14 with eosinophilic folliculitis and 7 with primary pruritus) were treated three times weekly with UVB phototherapy. Pruritus was quantified with use of a subjective score of 0 (none) to 10 (severe). RESULTS: Mean CD4 counts at the initiation of therapy were 91.0 +/- 31.9 cells/microliter. Pruritus scores before and after treatment were 8.6 +/- 0.4 and 2.2 +/- 0.5, respectively (p < 0.001). The mean number of treatments to achieve maximal improvement was 20.7 +/- 2.3, with a cumulative UVB dose of 3399.1 +/- 597.4 mJ/cm2. No significant difference was found between the group with eosinophilic folliculitis and the group with primary pruritus. CONCLUSION: UVB phototherapy can produce significant relief of pruritus and improvement in the quality of life in patients positive for HIV

Chronic actinic dermatitis (CAD) describes a persistent photosensitivity disorder in the absence of continued exposure to photosensitizers; it is characterized by a T-cell infiltrate within the epidermis and dermis. The purpose of this study was to characterize the T-cell infiltrate better immunohistochemically. Serial cryostat sections of fresh-frozen punch biopsy specimens of skin were analyzed in 11 patients with CAD and 3 patients with erythrodermic cutaneous T-cell lymphoma (CTCL). Monoclonal antibodies against the pan T-cell, pan B-cell, and T-cell subsets and the T cell-receptor (TCR) antigens were used. CD8-positive (T-suppressor-cytotoxic) cells were predominant in the epidermis of CAD, while CD4-positive (T-helper) cells were predominant in the epidermis and dermis of CTCL. CDw29-positive (T-memory) cells were predominant in all cases. The number of BF1 (beta-chain constant region of the TCR)-positive cells approximated the number of CD3-positive cells in all CAD cases but was significantly lower than the number of CD3-positive cells in two of three cases of CTCL. There was no clustering or preferential staining with any of the beta-chain variable-region antibodies in any of the specimens. These results indicate that CAD has a characteristic immunophenotype distinct from that of most cases of CTCL and that discordance between BF1 and CD3 expressions did not occur in the CAD cases

BACKGROUND: In patients with psoriasis and human immunodeficiency virus type 1 (HIV-1) infection, therapeutic options may be limited by their potential immunosuppressive effects. UVB radiation can activate HIV-1 gene expression in transgenic mice and in vitro. It is not known whether this viral activation leads to a clinically significant effect or if these findings can be extrapolated to humans. OBJECTIVE: This study was performed to evaluate the safety of UVB light treatment in HIV-infected persons. METHODS: We prospectively studied the effect of UVB phototherapy on five HIV-infected patients with psoriasis and one with pruritus. A complete blood cell count with differential count, CD4+ and CD8+ T-lymphocyte counts, serum beta 2-microglobulin and HIV-1 p24 antigen were obtained before UVB phototherapy and after 21 and 42 treatments. After every five treatments patients were evaluated for opportunistic infections, and psoriatic involvement was quantified with the Psoriasis Area and Severity Index (PASI). RESULTS: Cumulative UVB doses ranged from 3326 to 43,364 mJ/cm2. There were no statistically significant changes in laboratory findings after 21 and 42 treatments. Of three patients without detectable serum levels of HIV-1 p24 antigen before phototherapy, only one became positive after 42 treatments. None of the six subjects had an opportunistic infection or malignancy during phototherapy. The PASI improved in all five patients with psoriasis, and the other patient noticed decreased pruritus. CONCLUSION: Our results suggest that UVB phototherapy is efficacious in HIV-1-infected patients with UVB-responsive dermatoses and is not associated with short-term changes in immune function

The vesiculobullous porphyrias are a group of blistering diseases with systemic as well as cutaneous manifestations ranging from mild to disabling, secondary to endogenous photosensitizing porphyrins. The characteristic patterns of porphyrin accumulation in erythrocytes, plasma, urine, and feces are invaluable for differentiating the vesiculobullous porphyrias from other blistering diseases and from one another. Most importantly, these porphyrin profiles are essential for distinguishing the acute vesiculobullous porphyrias, HCP and VP, from PCT, which is not associated with acute attacks. This distinction may prevent the potentially fatal administration of porphyrinogenic drugs to patients with HCP or VP. Histopathology is not helpful in differentiating the vesiculobullous porphyrias, as all typically show subepidermal bullae. Avoidance of sun exposure and precipitating factors are the initial steps in both the prevention and treatment of these entities. In addition, more specific therapy may be indicated, as discussed in the preceding pages