Henry W. Lim

Eosinophilic pustular folliculitis in the acquired immunodeficiency syndrome. Treatment with ultraviolet B phototherapy.

EOSINOPHILIC pustular folliculitis is a rare pruritic dermatosis1 that has only recently been reported in patients from the United States.2 3 4 5 6 The eruption is characterized by sterile pruritic...

Generation of Chemotactic Activity in Serum from Patients with Erythropoietic Protoporphyria and Porphyria Cutanea Tarda

THE porphyrias are a group of diseases characterized clinically by photosensitivity and biochemically by elevated levels of porphyrins in erythrocytes, plasma, urine, or feces.1 , 2 Within minutes ...

Effect of UVA and blue light on porphyrin biosynthesis in epidermal cells.

Abstract— To study porphyrin biosynthesis in normal human keratinocytes and A431 cells derived from human epidermoid carcinoma, cultured cells were incubated with delta‐aminolevulinic acid (ALA), the precursor of porphyrin synthesis, and accumulation of porphyrins was measured spectrofluorometrically. Both human keratinocytes and A431 cells accumulated porphyrins in a time‐dependent and a dose‐dependent fashion. Protoporphyrin was the predominant porphyrin accumulated by both cell types. Porphyrin accumulation was enhanced by Ca Mg ethylene‐diaminetetraacetic acid, a ferrochelatase inhibitor, and the enhancement was reversed by the addition of iron, suggesting the utilization of iron by ferrochelatase. The effect of light on porphyrin accumulation was evaluated by exposing the ALA‐loaded A431 cells to ultraviolet‐A (UVA) and blue light radiation, followed by continued incubation with ALA for 2–48 h. There was an enhancement of porphyrin accumulation 2–48 h after the radiation as compared with nonirradiated controls. Consistent with this finding, ferrochelatase activity decreased in these cells at 24 h and 48 h. These data demonstrate that human keratinocytes and A431 cells are capable of porphyrin biosynthesis, and that exposure of porphyrin‐containing A431 cells to light, which includes the Soret band spectrum, decreases the ferrochelatase activity, which is responsible, at least in part, for the further increase in porphyrin level.

UVB phototherapy is an effective treatment for pruritus in patients infected with HIV

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 associated with human immunodeficiency virus infection

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 times; 106/L.

Photosensitivity, abnormal porphyrin profile, and sideroblastic anemia***

Cutaneous photosensitivity in a 43-year-old man with idiopathic sideroblastic anemia associated with an abnormal porphyrin profile is reported. This condition was associated with elevated free erythrocyte porphyrin, plasma protoporphyrin, urine porphyrins (predominantly coproporphyrin), stool porphyrins (predominantly protoporphyrin), decreased ferrochelatase activity, and deletion of portions of the long arms of chromosomes 18 and 20. Five other patients with sideroblastic anemia and abnormal porphyrin profiles have been described; all but one of these patients had photosensitivity. The porphyrin profile of this patient is similar to that of three other previously described patients.

Hepatoerythropoietic porphyria: A variant of childhood-onset porphyria cutanea tarda: Porphyrin profiles and enzymatic studies of two cases in a family

Hepatoerythropoietic porphyria is a rare variant of porphyria cutanea tarda, manifested clinically as photosensitivity starting in early childhood. Biochemically, there are elevated levels of protoporphyrin in erythrocytes and acetate-substituted porphyrins in the plasma, urine, and feces. Uroporphyrinogen decarboxylase activities in these patients are markedly suppressed. Thus far, only nine patients have been reported. We hereby describe the clinical manifestations, histologic changes, porphyrin profiles, and erythrocyte uroporphyrinogen decarboxylase determinations of two additional patients, 9-year-old and 7-year-old siblings, that are consistent with those of nine previously reported patients with hepatoerythropoietic porphyria.

Chronic actinic dermatitis: Results of patch and photopatch tests with Compositae, fragrances, and pesticides

reported from the United Kingdom, 36% to 85% had positive patch or photopatch tests to Compositae oleoresin extracts, and 21% to 66% had positive responses to fragrances.1-3 It has been suggested that repeated exposure to these agents contributes to the pathogenesis of chronic actinic dermatitis. Because Compositae oleoresin extracts are not part of the North American Contact Dermatitis Group photopatch test series, the prevalence of positive reactions to these agents in U.S. patients is not known. The purpose of this study was to determine the prevalence of positive patch and photopatch test responses in patients with chronic actinic dermatitis, by means of a newly expanded series of photoallergens that includes Compositae and pesticide allergens.

Complement-derived chemotactic activity is generated in human serum containing uroporphyrin after irradiation with 405 nm light.

Patients with porphyrias have varying degrees of photosensitivity, associated with elevated levels of porphyrins in plasma, erythrocyte, urine and/or feces. To investigate the role of complement in the pathogenesis of cutaneous lesions, varying amounts of uroporphyrin were added to normal human serum (0.1-10 microgram/ml), and the mixtures were then exposed to 405 nm irradiation. Such treatments result in the diminution of total hemolytic complement activity and hemolytic titers of C1, C4, C2, C3, and C5; furthermore, cleavage products of C3 and C5 were detected. Chemotactic activity for human polymorphonuclear leukocytes was generated that was inhibitable by incubation with anti-C5, but not with anti-C3 antisera. No chemotactic activity was generated in Mg++-EGTA treated serum nor in C4-deficient guinea pig serum. These data indicate that irradiation with 405 nm light of normal human serum containing uroporphyrin results in activation of the complement system via the classical pathway, and the generation of complement (C5)-derived chemotactic activity for human polymorphonuclear leukocytes.

Chronic actinic dermatitis. An immunohistochemical study of its T-cell antigenic profile, with comparison to cutaneous T-cell lymphoma.

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.