M. Yadira Hurley

Cutaneous Rosai-Dorfman Disease: Comprehensive Review of Cases Reported in the Medical Literature since 1990 and Presentation of an Illustrative Case

Background: The latest comprehensive review of primary cutaneous Rosai-Dorfman disease was published as part of an exhaustive survey of sinus histiocytosis with massive lymphadenopathy in 1990. Since then, much progress has been made in the understanding of malignant lymphoma and benign disorders of lymphoid and histiocytic origin. Objective: We reviewed cases of primary cutaneous Rosai-Dorfman disease published since 1990 and discuss their clinical and pathologic features, comparing them with cases of systemic Rosai-Dorfman disease. Methods: We conducted a search of the National Library of Medicine PubMed database for cases of cutaneous Rosai-Dorfman disease reported in the English-language medical literature since February 1990. Results: We identified 72 patients with cutaneous Rosai-Dorfman (female to male ratio 1:0.5). The gross appearance and number or distribution of lesions were highly variable. Abnormal laboratory data included peripheral blood cytopenias (10 patients) and increased gammaglobulin fraction (10 patients). The response to treatment was variable. Conclusion: Purely cutaneous disease without the characteristic lymphadenopathy is rare but has been increasingly reported in the literature. Compared with patients with systemic Rosai-Dorfman disease, patients with primary cutaneous Rosai-Dorfman disease are older, women are more commonly affected, and whites are more likely than blacks to be afflicted.

A transient benign lymph node-based proliferation of T-cells simulating non-Hodgkin lymphoma in a patient with psoriasis treated with tumor necrosis factor alpha and CD11a antagonists.

BackgroundTherapeutic biologic agents are uncommonly associated with lymphoma.Case presentationWe report a patient with psoriasis treated with the biologic agents efalizumab (Raptiva®) and etanercept (Enbrel®), who developed painless lymphadenopathy with peripheral lymphocytosis during treatment, simulating a non-Hodgkin lymphoma clinically and pathologically. Lymphocytosis and lymphadenopathy spontaneously remitted following cessation of etanercept therapy and have not recurred.ConclusionDistinction between clinically benign lymphoid proliferations related to antipsoriasis therapy and malignant lymphoma avoids the unnecessary use of anti-lymphoma chemotherapy.

Histiocytic sarcoma with secondary involvement of the skin and expression of CD1a: evidence of indeterminate cell differentiation?

Background:  Histiocytic sarcoma is an exceedingly rare malignant neoplasm composed of cells with a monocyte/macrophage phenotype. In the current nosology of histiocytic neoplasms, histiocytic sarcoma is separate from indeterminate cell histiocytosis, a generally benign disorder characterized by proliferation of a CD1a+ and S‐100+ population of cells lacking Birbeck granules usually limited to the skin.

Features of basal cell carcinomas in basal cell nevus syndrome.

Basal cell nevus syndrome (BCNS) is an autosomal dominant genodermatosis that is characterized by early onset basal cell carcinomas (BCCs) that define the disease and often lead to diagnosis of the underlying syndrome. The objective of this study was to investigate the anatomic location, subtypes, and impact of BCCs on a group of 61 individuals affected with BCNS attending a research colloquium. Fifty individuals had at least one prior BCC with 22 participants having over 100 lesions. The median age of syndrome diagnosis was 11 years and median age of first BCC was 16 years. Males had more lesions on the upper back, upper extremities, and M‐zone of the face, while females had more lesions on the scalp, back, and lower extremities. Pigmented BCCs were concentrated on the neck, upper trunk, and extremities. Subjects with >100 lesions showed wider anatomic distribution. The number of BCCs did not correlate with any of the other major features of the syndrome. Eighty percent of affected individuals reported great concern related to BCCs, citing the limitations and morbidity of available treatments. Vigilant surveillance, which was found to be inconsistent for participants in this study, is warranted. Future work should include development of a consensus guideline on skin examinations and strategies to optimize therapy of BCCs in this syndrome.

Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease

Cutaneous and systemic plasmacytosis is a rare condition primarily affecting middle‐aged individuals of Japanese descent and has principally been reported in cases originating from Asia. Its relationship, if any, to Castleman disease, is controversial. The authors report a case of cutaneous and systemic plasmacytosis involving a 36‐year‐old man of Chinese ancestry born in North America. The biopsy of an involved lymph node revealed changes resembling Castleman disease. Significantly, this case to our knowledge represents the first case of cutaneous and systemic plasmacytosis presenting in a patient of Chinese ancestry born on the North American mainland.

Identification of Helicobacter pylori in skin biopsy of prurigo pigmentosa.

A 23-year-old Chinese man presented with a 3-year history of a pruritic eruption. On examination, pink urticarial papules associated with hyperpigmented reticulated patches were noted on his neck, back, and upper chest. Histopathology revealed vacuolar interface dermatitis and numerous gram-negative rods within a dilated hair follicle. The organisms were reactive with anti-Helicobacter pylori immunohistochemisty. The histologic findings and clinical presentation support the diagnosis of prurigo pigmentosa. Additional testing demonstrated a positive urease breath test and serum H. pylori IgG antibodies. The patient was referred to gastroenterology and treated with appropriate antibiotics. After treatment, esophagogastroduodenoscopy revealed chronic gastritis without evidence of H. pylori infection and his skin showed reticulated hyperpigmented patches without evidence of active inflammatory papules. Although previous reports have associated prurigo pigmentosa to H. Pylori gastritis, this is the first report of H. pylori organisms identified in a skin biopsy of prurigo pigmentosa.

Lentiginous melanoma in situ treatment with topical imiquimod: need for individualized regimens.

M elanoma in situ, lentiginous type (LM), is a precursor lesion for invasive malignant melanoma, lentiginous type (LMM). Already the most prevalent subtype of in situ melanoma, LM has been shown to be increasing in incidence. Currently, nonsurgical patients with LM have no treatment alternative but irradiation and so must endure the associated adverse effects of this treatment. In addition, recurrence following standard therapies is unacceptably high (8%-20%). For these reasons, a new effective therapy for LM that provides local control, prevents progression to LMM, and decreases morbidity and mortality is clinically desirable. Small studies have reported successful treatment of LM with imiquimod, 5%, cream. The present case series highlights 15 LM lesions in 14 patients treated with topical imiquimod. Histologic tissue specimens obtained before, during, and after treatment were evaluated to assist in directing patient management and in providing objective posttreatment histopathologic response.

Cutaneous rosai-dorfman disease and morphea: coincidence or association?

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease. A third of patients with this disease have extranodal involvement affecting the skin. Of these individuals, only around 3% will have purely cutaneous Rosai-Dorfman disease, which is limited to skin manifestations without systemic involvement. Cutaneous (localized) scleroderma or morphea, on the other hand, is a more common disease that most often affects women of all ages. Both conditions have unknown etiologies. Presented here is a case of a 60-year-old white woman with cutaneous Rosai-Dorfman disease and coexisting morphea. Representative biopsies from both areas were performed: one showing a dermal S-100+ histiocytic infiltrate with emperipolesis and the other showing a deep perivascular and interstitial plasma cell infiltrate with dermal sclerosis and loss of perieccrine fat. A laboratory and radiologic workup revealed no evidence of systemic involvement by either entity. The diagnosis of coexisting cutaneous Rosai-Dorfman disease and morphea was established. To our knowledge, this is the first report of these 2 entities found simultaneously in 1 patient.

Pigmented basal cell carcinoma: increased melanin or increased melanocytes?

Studies on the precise cause of increased melanization in pigmented basal cell carcinomas (BCC) are limited. We aimed to determine whether the cause of melanization is from increased number of melanocytes or increased melanin pigment, and if there is a difference in the number of melanocytes on different sun‐exposed locations.

Nevus Comedonicus: A Novel Approach to Treatment

Kofmann first described nevus comedonicus in 1895. The initial U.S. report was in 1914, from White, who referred to the disorder as nevus follicularis keratosus. Subsequently, the condition has been termed comedone nevus, nevus acneiformis unilateralis, and nevus zoniforme. The treatment of nevus comedonicus remains challenging. Numerous therapies have been reported in the literature, including surgical excision, dermabrasion, manual extraction of keratin, application of ammonium lactate lotion, application of topical retinoic acid or keratolytic agents, and oral isotretinoin. Ineffectiveness, potential adverse effects, or both restrict each of these treatment modalities. We report the first successful use of the 1,450-nm diode laser (Smoothbeam, Candela Corporation, Wayland, MA) in the treatment of nevus comedonicus.