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Background Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long-term prognosis are unknown. Objective To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. Methods Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. Results All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis-like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. Limitations This was a retrospective study with incomplete data from referring physicians. Conclusions Although spontaneous complete regression is expected, patients with SHJCM need long-term follow-up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.

Background Spontaneous remission is recognized in mycosis fungoides (MF) and Sezary syndrome (SS). Objective We analyzed the outcome of 44 patients with previously treated CD25-positive (CD25+), recurrent/persistent MF/SS randomly assigned to receive placebo as part of a phase III trial. Methods This trial investigated the efficacy and safety of two doses of denileukin diftitox in patients with MF/SS who had received up to 3 prior therapies. The primary end point was overall response rate. Multivariate regression analyses were used to assess the relationship between baseline covariates and clinical outcomes. Results The overall response rate was 15.9% for placebo recipients (complete response: 2.3%; partial response: 13.6%), reflecting the baseline rate of disease remission that can be expected in a clinical trial. The median progression-free survival (PFS) in the placebo arm was moderately short at 4.4 months compared with the active-agent arm but important to consider in the context of recent single-arm phase II studies of other therapies for MF/SS that report PFS of approximately 6 months. Multivariate analyses identified no significant effects of any baseline factors on either overall response rate or PFS, although there was a trend toward poorer PFS with advanced age. Because sepsis occurred significantly more often in the placebo arm versus the active-treatment arm, the role of antibiotics in causing remission cannot be discounted (6.8% vs 0%; P Limitations This study had a relatively small sample size, yielding a wide 95% confidence interval. Conclusion The results may serve as a useful comparator for other active-treatment studies of MF/SS that lack a placebo-control arm.

Alopecia areata (AA) is an autoimmune disease that presents as nonscarring hair loss, although the exact pathogenesis of the disease remains to be clarified. Disease prevalence rates from 0.1% to 0.2% have been estimated for the United States. AA can affect any hair-bearing area. It often presents as well demarcated patches of nonscarring alopecia on skin of overtly normal appearance. Recently, newer clinical variants have been described. The presence of AA is associated with a higher frequency of other autoimmune diseases. Controversially, there may also be increased psychiatric morbidity in patients with AA. Although some AA features are known poor prognostic signs, the course of the disease is unpredictable and the response to treatment can be variable. Part one of this two-part series on AA describes the clinical presentation and the associated histopathologic picture. It also proposes a hypothesis for AA development based on the most recent knowledge of disease pathogenesis. Learning objectives After completing this learning activity, participants should be familiar with the most recent advances in AA pathogenesis, recognize the rare and recently described variants of AA, and be able to distinguish between different histopathologic stages of AA.

Acanthosis nigricans is associated with many diseases, including internal malignancies, genetic disorders, and endocrine abnormalities. Insulin resistance frequently accompanies endocrine-associated acanthosis nigricans. We describe a patient with acanthosis nigricans associated with hyperandrogenism and extreme insulin resistance that spontaneously resolved after a marked decrease in insulin receptor antibodies.

Large or giant congenital melanocytic nevi are rare. They are associated with a risk of melanoma, which is poorly quantified, and often represent a major cosmetic problem. Surgical intervention is the most common treatment, although dermabrasion or curettage in the first weeks of life is used in some centers. When these nevi occur in the scalp, the risk of melanoma has been reported to be lower. Here we describe a series of children in whom large congenital melanocytic nevi on the scalp appeared to spontaneously involute. Therefore, our findings indicate that these nevi also have a better prognosis cosmetically. It is suggested that the decision about surgical treatment should be deferred until the maximal improvement in appearance has occurred during the first 2 years of life.

Background Cutaneous Rosai-Dorfman disease (RDD) is a rare entity of unknown origin and is not well documented, especially in Asian populations. Objective The purpose of this study was to evaluate the clinical manifestation, diagnostic histopathology, clinical course, and response to treatment of cutaneous RDD in Taiwan. Materials and methods This study included 21 patients with cutaneous RDD who presented at our institution from 1995 to 2003. Pathologic examinations with both hematoxylin-eosin and immunohistochemical stains were reviewed, as were associated clinical features and therapeutic methods. Results None of the 21 patients with cutaneous RDD had nodal lesions. The clinical manifestation was variable, but most commonly involved a central noduloplaque with satellite papules. One patient manifested as an ulcerated nodule, something not reported previously. Multifocal involvement only occurred in 4 patients. Concurrent involvement of uvea or vocal cord occurred in two patients. The most prominent histologic feature was a florid and mixed inflammatory infiltration. The phagocytosis of inflammatory cells into the cytoplasm of histiocytes, a process called "emperipolesis," is a characteristic finding of nodular RDD but usually only focally presented in cutaneous ones. Positivity for S-100 protein helped to confirm the diagnosis. The most effective treatment was surgical excision of solitary lesions. High-dose thalidomide (300 mg/d), but not low-dose, was effective to control the extensive cutaneous diseases in two patients. A total of 3 patients experienced spontaneous remission 1 to 2 years after diagnosis. Conclusions Cutaneous RDD appeared more frequently in Asian populations than in reports from Western countries. The incidence of multifocal involvement in this series is much lower than in other literature. Although treatment of disseminated cutaneous RDD is difficult, high-dose thalidomide (300 mg/d), which was effective in two patients in this series, may be helpful.

Self-healing juvenile cutaneous mucinosis is an extremely uncommon disorder characterized by the acute eruption of multiple papules and subcutaneous nodules. In this report, we describe one of the youngest patients reported to date with self-healing juvenile cutaneous mucinosis and further define several of the histologic and clinical characteristics unique to the disorder. Awareness of this disease is important because, despite an ominous presentation, all reported cases have resolved spontaneously. Therefore, aggressive therapy should be avoided.

Papular mucinosis is a rare, idiopathic cutaneous mucinosis that is typically chronic and progressively severe. We present a case of a 59-year-old woman with a 6-month history of a dramatic papular eruption on her face and neck, with no associated internal organ involvement. A biopsy specimen demonstrated increased dermal mucin with associated plump fibroblasts, consistent with a diagnosis of papular mucinosis. Multiple therapeutic interventions were unsuccessful, and after 2 years the lesions resolved spontaneously without treatment. During the following 3 years, the patient has had no recurrence of disease.

Three open-label, uncontrolled studies have documented successful treatment of warts with cimetidine, whereas two placebo-controlled, double-blind studies and two open-label comparative trials have failed to demonstrate efficacy. This double-blind, placebo-controlled study was designed with stringent enrollment and outcome criteria to minimize the confounding issue of spontaneous remission. Efficacy was not statistically superior to that of placebo, but a trend toward efficacy was suggested for younger subjects.

The clinical and pathologic findings of Kikuchi's histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.

We report two unrelated boys aged 4 and 9 years who had the recently delineated phenomenon of easily pluckable hair. Hair tufts could be pulled out without effort and without pain. Alopecia without any sign of scalp inflammation or scarring was noted. In the older child spontaneous remission occurred within 3 months, whereas the condition remained active in the younger boy for a follow-up period of 1 year. Trichograms from clinically involved and uninvolved areas disclosed a striking predominance of anagen hairs (98% to 100%) and complete absence of telogen hairs. A loss of the normal round or oval configuration of many hair shafts could be well visualized by the shrinking tube technique. Horizontal and transverse sections of scalp obtained for biopsy revealed marked cleft formations between hair shafts and regressively altered inner root sheaths. We suggest the name loose anagen hair of childhood for this newly described disease.

A new case of congenital self-healing reticulohistiocytosis was studied with S-100 antibody and electron microscopy. Many tumor cells were 5-100-positive and contained Langerhans cell (LC) granules and concentrically laminated dense bodies. Octopus-like bodies and wormlike bodies were also found. The formation of LC granules at the cytomembrane was frequently found, not only between the cytomembrane and its own villi, but also between the cytomembranes of adjoining cells. These findings strongly support the cytomembrane origin of LC granules in general.

Although spontaneous remission of granuloma annulare is not uncommon, most cases are chronic and resistant to a variety of therapeutic modalities. In our study we confirm the beneficial effect of dapsone treatment in patients with localized or generalized granuloma annulare. The results indicate that dapsone is a useful drug to control or at least to improve skin lesions in the majority of patients.