George Kroumpouzos

Cutaneous Rosai-Dorfman Disease: Histopathological Presentation as Inflammatory Pseudotumor. A Literature Review

Purely cutaneous Rosai-Dorfman disease is exceptional. The disease is characterized histologically by large, proliferating histiocytes exhibiting inflammatory cells within their cytoplasm (emperipolesis). We present here a case of purely cutaneous generalized disease in which the routine histopathology was suggestive of an inflammatory pseudotumor. Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. The rarity of this case lies in the presence of conspicuous inflammatory pseudotumor-like histopathologic changes, masking an otherwise typical sinus histiocytosis cell infiltrate. This unusual presentation of the disease requires a high index of suspicion by clinicians and pathologists.

ANCA-positive necrotizing vasculitis and thrombotic vasculopathy induced by levamisole-adulterated cocaine: A distinctive clinicopathologic presentation

REFERENCES 1. Karrer S, Abels C, Wimmershoff MB, Landthaler M, Szeimies RM. Successful treatment of cutaneous sarcoidosis using topical photodynamic therapy. Arch Dermatol 2002;138:581-4. 2. Wilsmann-Theis D, Bieber T, Novak N. Photodynamic therapy as an alternative treatment for cutaneous sarcoidosis. Dermatology 2008;217:343-6. 3. Patterson C. Successful treatment of cutaneous sarcoid by photodynamic therapy with minimal discomfort using a fractionated dosing regime. Photodermatol Photoimmunol Photomed 2009;5:276-7. 4. Baughman RP, Lower EE. Newer therapies for cutaneous sarcoidosis: the role of thalidomide and other agents. Am J Clin Dermatol 2004;5:385-94.

Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature

We report a unique case of a congenital keratinocytic naevus associated with severe alopecia, onychodysplasia and palmoplantar involvement in a 13‐year‐old girl. The lesions, consisting of scaly, spinous and verrucous papules and plaques, mainly followed Blaschko’s lines and have remained unchanged since birth. The predominant histopathological picture was that of a column of parakeratosis overlying the eccrine ostia and hair follicles. This is the first case of a systematized keratinocytic naevus characterized by histopathology of eccrine and hair follicle porokeratosis and a widespread bilateral involvement. This may be a distinct entity to be included in the differential diagnosis of linear, hyperkeratotic dermatoses. We suggest its classification as systematized porokeratotic eccrine and hair follicle naevus.

Intrahepatic cholestasis of pregnancy: what's new

Itching has been reported to occur in 17% of pregnancies. Pruritus without primary skin lesions often results from intrahepatic cholestasis of pregnancy (ICP). The condition manifests itself in severe cases as jaundice (intrahepatic jaundice of pregnancy), but in mild cases itching may be the only symptom (pruritus gravidarum). The terms ‘obstetric cholestasis’, ‘recurrent jaundice of pregnancy’, ‘pruritus gravidarum’, ‘icterus gravidarum’ and ‘idiopathic jaundice of pregnancy’ all refer to the same clinical entity (ICP), characterized by pruritus with or without jaundice due to cholestasis. There is considerable geographical variation in the incidence of ICP. 1 The condition is very common in Chile (14%), especially among Araucanian Indian women (24%), and in Bolivia. ICP used to be quite common in Scandinavia. The incidence of ICP is overall lower in Europe (0.1–1.5%), the United States, Canada and Australia. 2 This geographical variation is now attributed to environmental and/or dietary factors. ICP recurs in 60–70% of subsequent pregnancies. There is a family history in 50% of ICP cases. ICP has been associated with twin and triplet pregnancies. 2

Risk of Basal Cell and Squamous Cell Carcinoma in Persons with Prior Cutaneous Melanoma

Background. Melanoma has been associated with an overall increase in actinic tumors, including actinic keratoses, as well as with noncutaneous malignancies. Objective. Determine the risk of developing basal cell and squamous cell skin cancer in patients with prior cutaneous melanoma (actinic keratoses not encountered). Methods. This retrospective study included 1396 white patients with prior cutaneous melanoma followed at the Roswell Park Cancer Institute in the period 1977–1978. The control group was the white population of the Detroit area in the same period (1977–1978). Results. A total of 25 patients (18 males, 7 females) developed 35 basal cell and/or squamous cell carcinomas: 18 developed basal cell carcinomas, 2 squamous cell carcinomas, and 5 both. The calculated odds ratio was 3.49 (males 3.67, females 2.86, 95% confidence interval 1.52–8.00). No correlations were found with age, type, anatomic site, and length of follow‐up of cutaneous melanoma. Conclusion. A history of cutaneous melanoma significantly increases the risk of basal cell and squamous cell skin cancer.

Atopic dermatitis in pregnancy: current status and challenges.

Atopic dermatitis (AD) is the most common pregnancy dermatosis. This evidence-based review article provides an evaluation of AD in gestation. Our literature search revealed 4 epidemiologic studies on AD in pregnancy, and a total of 55 articles that provide the basis for this review. The limitations of epidemiologic studies included herein are critically reviewed. The management of AD in gestation is reviewed with an emphasis on drug safety. Further studies are required to determine whether it is the intrinsic (“nonallergic” or “atopiform dermatitis”) and/or extrinsic (IgE-associated) AD that is affected by pregnancy, and to establish the postpartum prognosis of “new atopic dermatitis” (AD presenting for the first time in pregnancy). A revision of the diagnostic criteria will allow a more accurate confirmation of the prevalence of AD, and especially “new atopic dermatitis,” in pregnancy as well as differentiation of AD from specific dermatoses of pregnancy, such as prurigo and pruritic folliculitis. Addressing the above issues and unraveling the etiopathogenesis of AD in pregnancy will help clarify a suggested overlap with the above specific dermatoses. Target Audience: Obstetricians & Gynecologists and Family Physicians Learning Objectives: After the completing the CME activity, physicians should be better able to correctly diagnose and safely treat atopic dermatitis in pregnancy; differentiate atopic dermatitis from other pregnancy dermatoses; and appraise the controversies and challenges in the field.

Intradermal melanocytic nevus with prominent Schwannian differentiation

Features of peripheral nerve sheath differentiation such as neuroid cords, nerve corpuscles, fascicle-like structures, and, exceptionally, palisading have been reported in melanocytic nevi. We report an intradermal melanocytic nevus with prominent Verocay-like bodies. The upper portion of the neoplasm was composed of typical round intradermal nevus cells, many of which were pigmented. Within the deeper portion, there was a nonpigmented spindle cell proliferation with prominent Verocay bodies, simulating a neurilemmoma. Typical nevus nests merged with neurilemmoma-like areas. The entire lesion stained positively for S-100 and Mart-1 proteins and negatively for HMB-45 stain. Diffuse Mart-1 positivity excluded a collision of a melanocytic lesion with a neurilemmoma. The histopathologic features of this nevus further support a close relation between nevus cells and Schwann cells.

Pregnancy-Associated Hyperkeratosis of the Nipple: A Report of 25 Cases

IMPORTANCE Reported physiologic nipple changes in pregnancy do not include hyperkeratosis and are expected to resolve or improve post partum. Hyperkeratosis of the nipple and/or areola can develop in the context of inflammatory diseases (such as atopic dermatitis), in acanthosis nigricans, as an extension of epidermal nevus, after estrogen treatment, and/or in nevoid hyperkeratosis of the nipple and areola. We performed a clinicopathologic analysis of cases of pregnancy-associated nipple hyperkeratosis. OBSERVATIONS Twenty-five cases of pregnancy-associated nipple hyperkeratosis identified during a 5-year period (January 1, 2007, through December 31, 2012) are reported. The lesions were bilateral and involved predominantly the top of the nipple. Lesions were symptomatic in 17 patients (68%), causing tenderness or discomfort, pruritus, sensitivity to touch, and/or discomfort with breastfeeding. Nine patients (36%) experienced symptomatic aggravation only during pregnancy or breastfeeding. The lesions persisted post partum in 22 patients (88%). Histopathologic features were conspicuous orthokeratotic hyperkeratosis, with papillomatosis and acanthosis being mild or absent. CONCLUSIONS AND RELEVANCE Pregnancy-associated hyperkeratosis of the nipple can be symptomatic and persist post partum. It may represent a physiologic change of pregnancy. The characteristic clinicopathologic features of this disorder allow differentiation from nevoid hyperkeratosis of the nipple and areola. We suggest that this distinctive clinicopathologic entity be called pregnancy-associated hyperkeratosis of the nipple.

Demonstration of Polarizable Crystals in Fresh Comedonal Extracts: Sebum Crystallizes

Previous studies using paraffin-embedded sections showed the presence of varying degrees of lipidic calculus (sebolith) formation in the pilosebaceous duct in acne comedonal lesions. The objective of this study was to examine the content of fresh acne comedonal extracts and pustules in polarizable crystalline material. Furthermore, to investigate if the amount of crystalline material correlates with the morphology, evolutionary stage, age and location of the comedone we performed polariscopic examination of 20 fresh acne comedonal extracts and 6 acne pustules. As controls, we used extracts from solar comedones, milia and epidermal inclusion cysts, follicular extracts from acne rosacea lesions, pustules of bacterial folliculitis and extracts from normal follicles from acne-prone individuals. The vast majority of acne comedones contained considerable amounts of polarizable crystalline material. Crystallization was more prominent in closed comedones, long-standing macrocomedones and conglobate comedones. Crystal formation was seen less commonly (p < 0.01) in solar comedones, milia and epidermal inclusion cysts. As shown in this study, crystallization of sebum is a common element of comedogenesis and may possibly contribute to comedo preservation.

Combined classic and iatrogenic Kaposi's sarcoma: Corticosteroid withdrawal can result in remission

A 72~year~old white man of Italian ancestry had a 7~month history of asymptomatic plaques on his legs and feet. The plaques had appeared 1 month after he started taking prednisone, 25 mg daily, for symp~ toms of temporal arteritis. The patient had several medical problems and had been recently hospitalized on account of melena. He had been treated with topical medications for seborrheic dermatitis. He had no known drug allergies. Physical examination revealed several well~demarcated plaques on both legs, the dorsa and toes of both feet, and the left sole (figures 1 and 2). The lesions, which measured 1 to 3 em across, were purple to dusky red and non~ blanchable. Some of the plaques had minimal scale. No lesions were seen in the oral mucosa. Microscopic examination of the scaly lesions using a potassium hydroxide preparation revealed no fungi. Histopathologic examination showed extensive red blood cell extravasation and a proliferation of superficial dermal spindle cells forming dilated, slitlike, jagged vascular channels separating collagen (figure 3). These findings are diagnostic of Kaposis sarcoma. The lesions improved markedly after two courses of radiation therapy, each consisting of 15 Gy over a 1 O~day period, and tapering of prednisone from 25 mg to 5 mg daily. The lesions resolved completely after a third course of radiation therapy. After 2 years, the lesions are still in remission.