Melasma.

Melasma is a common disorder of macular hyperpigmentation which involves mostly in sun exposed areas of the face and neck. Those most affected are women. Multiple factors have been postulated to involve in the etiology and pathogenesis of melasma including pregnancy, oral contraceptives, genetics, sun exposure, cosmetics and race. We have conducted a clinical trial utilizing all trans-retinoic acid (tretinoin, Retin-A) cream 0.1% q pm and hydroquinone lotion 3% (Melanex) applied every morning in Korean women with melasma. Our study patients demonstrated all three clinical patterns common to melasma: centrofacial, malar and mandibular. Wood's light examination was performed on all patients and identified two of the four types of melasma described. Most patients showed epidermal melasma and a few manifested a mixed type. No patients exhibited solely dermal or inapparent type in melasma. With open studies of tretinoin cream and hydroquinone lotion followed by sun screen, we have found significant improvement within 5 months with a few side effects. Histopathologic examination of melasma in the pre-trial biopsies revealed increased pigmentation of the epidermis, dermis or both. In addition, significant alterations of the dermis with solar damage was noted in all melasma patients sampled. Biopsies taken after five months of treatment revealed significant decreases in epidermal pigmentation and improvement of solar damage in the dermis. We reconfirmed that a synergistic mechanism between tretinoin and hydroquinone is responsible for the improvement seen in the female Korean melasma patients from our study.

Major suppression of pro-alpha1(I) type I collagen gene expression in the dermis after keloid excision and immediate intrawound injection of triamcinolone acetonide.

BACKGROUND: A keloid is a benign tumor that contains excess collagen, primarily type I collagen. A common therapy is intralesional injection of a glucocorticosteroid, such as triamcinolone acetonide (TA). Surgical excision is also common; often a glucocorticosteroid is injected weeks after excision when wound repair has already begun. OBJECTIVE: Our purpose was to determine the efficacy of TA in reducing the pro-alpha1(I) type I collagen mRNA in the dermis, when TA is injected into the wound bed immediately after surgical excision of the keloid. METHODS: Six patients with previously untreated keloids were studied. Three were treated with 10 mg/ml of TA immediately after excision of the keloid (experimental group); the other three patients were not treated with TA until 2 weeks after excision (control). Punch biopsy specimens were obtained from the TA-treated sites 2 weeks after removal of the keloid and from the wounds of the control group of patients before they were treated with TA. Sections were prepared for in situ hybridization analysis of the pro-alpha1(I) collagen mRNA, as well as for histochemical analysis of collagen fibers. RESULTS: All keloids showed greatly elevated levels of pro-alpha1(I) type I collagen mRNA in the dermis. Postsurgical wounds injected with TA after removal of the keloid expressed decreased pro-alpha1(I) collagen transcripts, compared with skin not treated with TA. The collagen bundles were also thinner and less dense in the TA-treated skin. CONCLUSION: Downregulation of the type I collagen gene expression is elicited by immediate TA injection after keloid excision. This suggests that prevention of recurrent keloid growth is possible if surgical excision is accompanied by immediate TA injection into the wound bed and that healing of the wound is not apparently compromised by inhibition of type I collagen gene expression.

Systemic lupus erythematosus in patients diagnosed with treatment-resistant acne.

BACKGROUND: Systemic lupus erythematosus (SLE) may involve any number of organ systems and varies greatly in the severity and type of involvement. Cutaneous manifestations of SLE are equally numerous and varied throughout the course of the disease within an individual, as well as varying between patients. Cutaneous manifestations of SLE are frequently the presenting symptoms, typically noted in the classic malar "butterfly" rash; however, other cutaneous patterns are frequently observed. METHODS: We present here two patients who presented with what was thought to be acne refractory to treatment. RESULTS: These patients actually were found to have a facial eruption associated with SLE as confirmed by skin biopsy. CONCLUSIONS: The importance of investigating atypical or treatment-resistant eruptions, especially in patients experiencing other symptoms, is emphasized.

Solitary congenital self-healing reticulohistiocytosis.

Congenital self-healing reticulohistiocytosis (CSHR) was first described in 1973 by Hashimoto and Pritzker. Since then, both multiple and solitary forms have been described. We report a further case of solitary congenital self-healing reticulohistiocytosis. CSHR is a rare disorder which frequently presents a diagnostic dilemma in the newborn. Nodular forms of systemic Langerhans cell histiocytosis may present in a manner similar to CSHR. Because their differentiation on histopathological grounds is impossible, physical examination, laboratory investigations, and follow-up are necessary to ensure a lack of systemic involvement. Although previously thought to represent a rare variant of CSHR, solitary lesions of CSHR account for almost 25% of reported cases. A review of the literature relating to solitary CSHR, and a consideration of the differential diagnosis of a solitary congenital nodule, are also presented.

Elevated plasma histamine in chronic uremia. Effects of ketotifen on pruritus.

A role for histamine in the pathogenesis of uremic pruritus was investigated in maintenance hemodialysis patients. Venous plasma histamine levels, as determined by radioenzymatic assay, were significantly higher (p less than 0.05) in hemodialysis patients with pruritus (368 +/- 103 pg/ml [mean +/- SEM], n = 6) than in those without pruritus (146 +/- 22 pg/ml, n = 5) and in normal controls (142 +/- 16, n = 5). Arteriovenous fistula histamine levels (202 +/- 52 pg/ml, n = 6) were significantly lower (p less than 0.05) than simultaneously drawn venous samples. Markedly elevated histamine-degrading enzyme (histaminase) activities were found in both hemodialysis patients with (2.95 +/- 0.18 pg histamine degraded/minute) and without (2.44 +/- 0.28) pruritus, but was undetectable in normal controls. Histaminase activities did not significantly differ in simultaneously drawn venous and fistula samples. With hemodialysis, histaminase activities fell significantly (p less than 0.01), whereas plasma histamine did not change. We further examined the effects of ketotifen, a putative mast cell stabilizer, on severe uremic pruritus. Five of five patients had significant (p less than 0.01) reductions in pruritus, as judged on a six-point pruritus index, after 8 weeks of drug (x = 2.3), as compared to conventional therapy (x = 5.9). Despite these improvements, no significant differences were noted in pre- versus post-drug plasma histamine levels, histaminase activities, or the histamine content per gram of skin biopsy specimen. These data support prior hypotheses that mast cell activation contributes to the pruritus of uremia.

Cutaneous malignancies mimicking herpes zoster.

Cutaneous metastases occur in 2.5% to 5% of patients with malignant disease. The relative frequency of the primary site roughly parallels the frequency of the various malignancies in each sex. We present two cases of cutaneous malignancy occurring in a dermatomal distribution and masquerading as herpes zoster. The differential diagnosis of zosteriform eruptions is reviewed and the possible pathogenesis of metastatic disease in this cutaneous distribution is discussed. Skin biopsy is recommended in these cases to determine the etiology of the eruption.

Connective tissue nevus masquerading as a localized linear epidermal nevus.

A 22-year-old black female patient presented to the dermatology clinic with an eruption on her left arm. The eruption consisted of hyperpigmented grouped papules with a verrucous appearance. Because the lesions were soft on palpation, the eruption was diagnosed clinically as an atypical localized linear epidermal nevus. Examination of a biopsy specimen of the lesion, however, revealed marked fibroblastic proliferation and pronounced dermal fibrosis, along with epidermal changes of hyperkeratosis, focal hypergranulosis, and papillomatosis. These findings are consistent with connective tissue nevus with epidermal changes suggestive of epidermal nevus. Therefore a deep biopsy is strongly suggested to rule out the possibility of connective tissue nevus in suspected cases of atypical linear epidermal nevus.

Leukemic macrocheilitis associated with hairy-cell leukemia and the Melkersson-Rosenthal syndrome.

Specific cutaneous infiltrates in hairy-cell leukemia have distinctive histopathologic and histochemical findings. The true incidence of leukemia cutis in hairy-cell leukemia is uncertain, however, because reports often fail to document these distinctive features. A case of leukemic macrocheilitis associated with hairy-cell leukemia and the Melkersson-Rosenthal syndrome is presented. The Melkersson-Rosenthal syndrome is a clinically defined entity composed of Bell's palsy, macrocheilitis, and lingua plicata. Granulomatous cheilitis is often seen on histopathologic examination of the swollen lip tissue; in some cases, however, the findings may be nonspecific. Neoplastic macrocheilitis has not been reported in Melkersson-Rosenthal syndrome. Furthermore, leukemic macrocheilitis has not been reported in hairy-cell leukemia. A possible relationship between these entities is proposed.

A surgical approach for dermatosis papulosa nigra.

Dermatosis papulosa nigra (DPN) is a disorder commonly encountered in the adult black population. We describe 20 cases of clinicopathologically confirmed DPN cases treated with light abrasive curettage without local anesthesia. We found this procedure to be an effective therapy, which did not cause postoperative scarring or significant postoperative pigmentary change.

Periorbital granuloma annulare.

A 40-year-old woman had erythematous, papular lesions on both periorbital areas. Histologic examination disclosed the presence of palisading granulomatous lesions. To our knowledge, periorbital granuloma annulare is extremely rare. Our experience demonstrates the importance of obtaining biopsy specimens from periorbital papules and considering granuloma annulare in the differential diagnosis of such lesions.

Pseudoatrophoderma colli. A familial case.

A case of familial pseudoatrophoderma colli was found in a 22-year-old Indian woman whose sister and father had similar eruptions. Pseudoatrophoderma colli is an unusual papillary dermatosis in which the lesions appear atrophic. The condition responded very well to treatment with 5% lactic acid ointment. It is likely that pseudoatrophoderma colli is more common than has been reported. It should be considered in the differential diagnosis, along with acanthosis nigricans and confluent and reticulated papillomatosis of Gougerot and Carteaud when a pigmented and papillary dermatosis is present around the neck and upper part of the thorax.

Malignant proliferating angioendotheliomatosis.

A 48-year-old man had fever and generalized asymptomatic erythematous telangiectatic plaques and patches. Skin biopsy specimens confirmed the diagnosis of malignant angioendotheliomatosis. Local irradiation therapy induced a temporary remission. Chemotherapy was administered without altering the course of the disorder. Limited autopsy revealed visceral as well as cutaneous involvement.

Sarcoidosis associated with mycosis fungoides.

In November 1966, a 47-year-old black woman had a skin eruption and an abnormal chest roentgenogram that were found to be due to sarcoidosis. In September 1974, nodular lesions and subsequently generalized exfoliative dermatitis developed that were found to be due to mycosis fungoides. The concurrence of sarcoidosis and mycosis fungoides has been recorded only once in the literature. The depression of cell-mediated immunity found in this patient and other sarcoid patients suggests that impairment of the host's immunosurveillance system may predispose to the development of T-cell lymphomas.

Ichthyosiform sarcoidosis.

A 31-year-old black woman had acquired ichthyosis. Histologic examination of her skin revealed a sarcoidal reaction in the dermis. The diagnosis of sarcoidosis was supported by negative tuberculin (intermediate strength purified protein derivative) and Candida albicans extract intradermal skin tests; by a positive Kveim test; and by roentgenographic evidence of bilateral hilar adenopathy, paratracheal node enlargement, and diffuse pulmonary parenchymal changes. Sarcoidosis must be considered when acquired ichthyosis develops in a patient.