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Tick bites can cause a number of local inflammatory reactions, which are often difficult to differentiate from those induced by other arthropod bites or stings. These include erythematous nodular or pustular lesions, erosive plaques, annular lesions of erythema chronicum migrans, and both scarring and nonscarring inflammatory alopecia. We report a case of nonscarring alopecia in a 21-year-old male who reported a recent history of tick bite to the scalp. The biopsy demonstrated a dense pseudolymphomatous inflammatory infiltrate with numerous eosinophils associated with hair follicle miniaturization and an elevated catagen-telogen count. Signs of external rubbing, including lichen simplex chronicus and the "hamburger sign", were also visualized and are indicative of the associated pruritus. To the authors' knowledge, this is the fifth report of nonscarring tick bite alopecia in the literature and the first in an adult patient. This text will review the classic clinical presentation, histologic findings, and proposed mechanism of tick bite alopecia.

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis (PLCA), which presents as discrete, firm, closely-set 1-3mm, dome-shapedbrown papules commonly involving the anterior aspect of shins and extensor surfaces of forearms. We present a case of an otherwise healthy man in his 30s with solitary facial involvement of lichen amyloidosis, which is very uncommon.

Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis (PLCA), which presents as discrete, firm, closely-set 1-3mm, dome-shapedbrown papules commonly involving the anterior aspect of shins and extensor surfaces of forearms. We present a case of an otherwise healthy man in his 30s with solitary facial involvement of lichen amyloidosis, which is very uncommon.

Meyerson's phenomenon is a well-documented inflammatory reaction described in a variety of cutaneous lesions, including the original description in nevocellular nevi (1). Such an inflammatory reaction was subsequently described in melanocytic and a sundry of nonmelanocytic lesions alike, including vascular malformations (2-11). We present three cases of infants with nevus sebaceus on the scalp, which were obscured by an eczematous, eosinophilic reaction reminiscent of that first described by Meyerson. Two of the patients had concomitant atopic dermatitis, but one had no association. Recognition of this secondary feature is important in establishing the correct diagnosis.

immunoglobulins from the mother to the fetus through the placenta. Affected neonates present with an urticarial erythematous vesicular rash with occasional blisters. Their disease is milder and usually resolves spontaneously within days to weeks, sometimes before the maternal antibodies are removed from the neonate’s blood. The primary goal of treatment is to relieve pruritus and prevent new blister formation. Mild cases of PG respond well to topical steroids and antihistamines. Systemic steroids are the treatment of choice. Prednisone or prednisolone is given at a dose of 0.5 to 1 mg/kg/day and is adjusted depending on response. It is usually tapered preterm, but may need to be increased postterm because of postpartum flares. Other treatment options that have been used in refractory cases include cyclosporine, cyclophosphamide, azathioprine, dapsone, intravenous immunoglobulin, rituximab, plasmapheresis, goserelin, pyridoxine, sulfapyridine, and tetracyclines with nicotinamide for postpartum flares. For this series the recommended choices are: 1, c; 2, e; 3, a; 4, b.

Melanoma or melanoma metastases can rarely mimic blue nevi clinically and/or histologically, presenting a diagnostic pitfall for both the clinician and the dermatopathologist. We report a case of an invasive lentigo maligna melanoma with subsequent development of multiple, cutaneous blue nevus-like localized metastases followed by a distant metastasis, heralding widespread systemic metastases.

Henoch-Schonlein purpura is a common small-vessel vasculitis that presents in children as nonthrombocytopenic, nontender, and palpable purpura, arthritis/arthralgia, and abdominal pain. Central nervous system involvement is a reported, but likely, under-recognized complication. We present an 8-year-old boy with Henoch-Schonlein purpura and posterior reversible encephalopathy syndrome, after proposed mechanism and treatment considerations.

Graft-versus-host disease (GVHD) is an underappreciated complication of autologous hematopoietic stem cell transplantation (AHSCT) that can affect the skin, gastrointestinal tract, and liver. The development of this rare condition is probably due to an impairment of immunologic tolerance that can occur spontaneously through T-cell dysregulation, possibly from intensive conditioning chemotherapy regimens, or intentionally through administration of cyclosporine in the hopes of promoting an antitumor response. We present the case of a 2-year-old boy with metastatic neuroblastoma who spontaneously developed autologous GVHD after AHSCT. Severe pruritus and an inability to taper his oral steroids without a disease flare marked his disease. Eventually partial relief was achieved with initiation of cyclosporine and a strict soak and smear protocol using topical triamcinolone 0.1% ointment.

Background. Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. Objective. The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. Methods. A 45-year-old woman presented with a nevus sebaceusthat contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. Results. Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. Conclusion. This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus.

Background. Angiolymphoid hyperplasia with eosinophilia is a benign vascular proliferation that typically presents on the head and neck. Multiple treatment modalities have been proposed for angiolymphoid hyperplasia with eosinophilia, each with limited success or undesirable side effects. At this time, standard surgical excision is considered the treatment of choice but carries recurrence rates of 33% to 50%. Objective. The objective was to present a case of angiolymphoid hyperplasia with eosinophilia successfully extirpated using Mohs micrographic surgery. Methods. A 52-year-old woman presented with an ill-defined solitary plaque of angiolymphoid hyperplasia with eosinophilia within her left conchal bowl that enlarged despite conservative therapy with intralesional and topical corticosteroids. Mohs micrographic surgery using the fresh tissue technique and standard hematoxylin and eosin staining was performed. The characteristic histologic features of angiolymphoid hyperplasia with eosinophilia were readily identifiable on frozen sections and complete extirpation required two stages of micrographically controlled resection. Results. Resection of angiolymphoid hyperplasia with eosinophilia of the conchal bowl with complete resolution of symptoms and no evidence of clinical recurrence 8 months after surgery. Conclusion. Given the high recurrence rates reported for standard excision, Mohs micrographic surgery with complete margin examination should be considered as a treatment option for angiolymphoid hyperplasia with eosinophilia, particularly for lesions with ill-defined margins or in locations where tissue sparing is desirable.

In 1999, we noted the development of inflammation and/or abscesses at the site of radial access in a group of patients. Over a 3-year period, we noted this inflammation in 33 patients out of 2,038 (1.6%) who had catheterization via the radial approach. The radial abscesses occurred in 30 patients out of 1,063 (2.8%) in whom we could confirm the use of a hydrophilic-coated sheath, but in no patient for whom we can document that an uncoated sheath was used. No infectious agent could be implicated, and the time course for the development of the abscess, typically 2 to 3 weeks, seemed long for a bacterial infection. Later patients had biopsies, and granulomatous reactions were seen in most. Additionally, a few of the biopsies showed an amorphous extravascular substance consistent with the catheter coating. All patients had good long-term outcomes. Cathet Cardiovasc Intervent 2003;59:207–213. © 2003 Wiley-Liss, Inc.

We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.

Cohen et al have proposed a comprehensive diagnostic and treatment plan for patients diagnosed with MTS or carrying an MTS-prone sebaceous neoplasm and their family members. This includes a review of the patient’s annual medical records and a comprehensive physical examination, regular monitoring of the serum carcinoembryonic antigen level, upper gastrointestinal endoscopy and colonoscopy, chest radiography, urine cytology, computed tomography scans of the abdomen and pelvis, mammography, cervical smear, and endometrial biopsy. Immunohistochemical staining for MSH2, MSH6, and MLH1 protein expression was found to be useful for identifying the underlying mismatch repair defects in the sebaceous neoplasm and for additional molecular analysis of the disease. For this series, the recommended choices are: 1, a; 2, c; 3, c; 4, d.

Recent ultrastructural data indicate that tumor-induced angiogenesis involves polarized outgrowth of endothelial cells from established vessels into tumor parenchyma and interstitium. Efforts to define the cytoarchitecture of the angiogenic response and to ascertain molecular determinants of polarized endothelial migration have been impeded by lack of sensitive and specific immunologic markers for endothelium and vessel wall components. In this study, we utilized monoclonal antibody to platelet-endothelial cell adhesion molecule-1 (PECAM-1), a cell-cell adhesion molecule belonging to the immunoglobulin superfamily, to determine extent and patterns of angiogenesis in metastatic melanomas before (N = 7) and after (N = 3) induction of tumor-infiltrating lymphocyte responses provoked by administration of experimental melanoma vaccine. PECAM-1 proved to be a more reliable marker for angiogenesis than antibodies to von Willebrand Factor. Although both normal and tumor vessels exhibited prominent staining for PECAM-1, different patterns of endothelial reactivity were observed. Formation of new vessels was associated with i) PECAM-1 redistribution from a constitutive circumferential membrane pattern to a pattern restricted to cell-cell junctions; ii) formation of endothelial cords formed by cell-cell interactions; and iii) eventual development of open lumens. Vessels within inflamed (vaccine-treated) and non-inflamed melanomas did not differ with regard to patterns of PECAM-1 expression. Forming vessels of inflamed melanomas failed to express the cytokine-inducible activation marker, E-selectin. Although normal microvessels and reactive vessels of healing wounds demonstrated prominent staining for α6; laminin receptor, vessels within melanomas showed apparently diminished expression. These data establish PECAM-1 as a sensitive and specific marker for experimental angiogenesis, and further support the possibility that cell-cell adhesion mediated by PECAM-1 may contribute to directed endothelial migration typical of tumor-induced formation of new vessels.

Scleromyxedema is a rare variant of lichen myxedematosus. In addition to cutaneous manifestations, scleromyxedema often presents with systemic manifestations, including dysphagia, proximal muscle weakness, central nervous system disturbances, encephalopathy, and restrictive lung disease. It is almost always associated with paraproteinemia, usually IgG with gamma light chains. We review the literature on scleromyxedema associated with neurologic symptoms and present a case of a 49-year-old woman with encephalopathy attributable to scleromyxedema.

Erythema multiforme is histologically characterized by liquefactive degeneration along the dermal-epidermal junction, necrotic keratinocytes and a lymphocytic infiltrate. We report a 10-year-old boy with recurrent erythema multiforme major of undetermined etiology with unusual histologic findings. A skin biopsy taken at day 2 of his eruption revealed histologic features otherwise characteristic of erythema multiforme, but mediated instead by a CD68-positive infiltrate, resembling cutaneous Kikuchi's disease. To the best of our knowledge this is the first reported case of 'histiocytic' erythema multiforme.

Tumid lupus erythematosus is a rare variant of chronic cutaneous lupus erythematosus that is characterized clinically by smooth, nonscarring, pink to violaceous papules or plaques without evidence of surface change. Histopathologic features include superficial and deep lymphocytic infiltration in a perivascular and periadnexal distribution, with dermal interstitial mucin deposition and focal or absent dermoepidermal junction involvement. These clinical and histopathologic features can be challenging to differentiate from other cutaneous diseases. This is particularly true because patients with tumid lupus erythematosus usually do not have other manifestations of systemic lupus erythematosus or cutaneous lupus erythematosus. We present two cases of tumid lupus erythematosus, one associated with concomitant systemic lupus erythematosus and the other occurring concurrently with discoid lupus erythematosus. Furthermore, we demonstrate the rare occurrence of a patient with tumid LE occurring below the waist at a photoprotected site.

Interstitial granulomatous drug reactions are an uncommon entity presenting as asymptomatic, annular, erythematous to violaceous plaques. The incidence of such reactions has been increasing with the use of biologic agents. We report, to the best of our knowledge, the first such reaction to the interleukin (IL)-1 inhibitor anakinra. Our patient presented with pink dermal plaques and nodules in the periaxillary region which resolved with discontinuation of anakinra and recurred upon restarting anakinra. Biopsy revealed a diffuse dermal infiltrate of lymphocytes and histiocytes with interspersed neutrophils and eosinophils. Fragmentation and degeneration of collagen and elastic fibers was also present. Withdrawal of anakinra led to complete resolution of the lesions. Interstitial granulomatous drug reactions are increasing in frequency and we add anakinra to the list of causative agents.

Up to one fourth of patients with rheumatoid arthritis (RA) may have extraarticular findings such as subcutaneous nodules. These are discrete subcutaneous granulomatous nodules located on extensor surfaces, especially of the elbows. Over the past 10 to 15 years, there have been reports of accelerated cutaneous nodulosis in patients receiving methotrexate therapy. Recently, antitumor necrosis factor alpha (anti-TNFalpha) biologic therapy has become commonplace in the management of RA, especially in methotrexate-resistant or toxic patients. There have been recent reports of accelerated nodulosis in patients with RA on etanercept. We describe what we believe is the first case of accelerated cutaneous nodulosis resulting from infliximab anti-TNFalpha therapy in a patient with RA. One year after the initiation of infliximab, with RA in remission, our patient noted the rapid development of rheumatoid nodules of both hands. A biopsy was characteristic of a rheumatoid nodule, revealing palisading granulomas and fibrinoid necrosis.

posi's sarcoma in human immunodeficiency virus-positive patients. Arch Dermatol 1992;128:1365-70. 4. Diasio RB, Zihong L. Dihydropyrimidine dehydrogenase activity and fluorouracil chemotherapy. J Cfin Onco11994; 12:2239-42. 5. Leo S, Tatulli C, Taveri R, et al. Dermatologic toxicity from chemotherapy containing 5-fluorouracil. J Chemother 1994;6:423-6. 6. Vukelja S J, Bonner MW, McCollough ML, et al. Unusual serpentine hyperpigrnentation associated with 5-fluorouracil. J Am Acad Dermatol t991;25:905-8. 7. Vukelja SJ, James WJ, Weiss RB. Severe dermatologic toxicity from 5-fluorouracil in the presence of seborrheic dermatitis. Int J Dermatol 1989;28:353-4. 8. Goette DK, Odom RB, Owens R. Allergic contact dermatitis from topical fluoronracil. Arch Dermatol 1994; 113:196-8. 9. Sloan JB, Soltani K. Iontophoresis in dermatology. J Am Acad Dermatol 1986;15:671-84. 10. Glass JM, Stephen RL, Jacobson SC. The quality and distribution of radiolabeled dexamethasone defivered to tissue by iontophoresis. Int J Dermatol 1980;19:519-25. 11. Prussick R, Thibault A, Turner ML. Recall of cutaneous toxicity from fluorouracil. Arch Dermatol 1993;129:64-5.

A 55-year-old white female with a complex medical history including mixed connective tissue disease and peripheral vascular disease developed a group of red-purple papules on her proximal medial thigh that was followed, five months later, by the development of a large violaceous patch. She reported a history of radiation to this site (for melanoma) during her childhood. She was admitted to the hospital with a presumptive diagnosis of cellulitis, but failed to respond to antibiotics. A biopsy was performed and demonstrated a well-differentiated angiosarcoma arising in conjunction with reactive angioendotheliomatosis. Excision of the lesion was performed, and fifteen months of follow-up have shown no recurrence or metastasis.

Background: Primary cutaneous LELC is a cutaneous neoplasm with histopathologic features identical to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. It is extremely rare, with only approximately 30 cases reported in the literature. Methods: We report a case of primary cutaneous LELC arising on the forehead of a 72 year-old male in which a proportion of the neoplastic cells demonstrated distinctive spindle cell morphology. Results: Microscopic examination showed a dense lymphoplasmacytic infiltrate admixed with large spindle-shaped cells with vesicular nuclei, prominent nucleoli, and frequent mitotic figures. These cells were negative for an extensive panel of immunohistochemical markers and positive only for broad-spectrum cytokeratins and epithelial membrane antigen. There was no connection between the tumor and the epidermis and no epidermal dysplasia. In situ hybridization for Epstein-Barr virus was negative. Conclusions: The spindle cell differentiation in this case is unusual and suggests that in some cases the differential diagnosis of cutaneous spindle cell neoplasms might include primary cutaneous LELC.

Neutrophilic eccrine hidradenitis (NEH) is an acute, self-limited, inflammatory neutrophilic dermatosis. It is most commonly described in patients with acute myelogenous leukemia (AML) following chemotherapy. NEH generally presents as erythematous papules and plaques on the head, trunk, and extremities. Histologically, NEH is characterized by a neutrophilic infiltrate surrounding eccrine ducts and coils with occasional necrosis. We describe a case of NEH with an unusual presentation of annular plaques. A search of the literature revealed only one other case report of NEH presenting as an annular eruption.

Background: The CD30-positive lymphoproliferative disorders lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (C-ALCL), and systemic anaplastic large cell lymphoma (S-ALCL) are lesions that overlap clinically, histopathologically, and immunophenotypically. Their biologic behaviors, however, vary considerably. In particular, lesions of LyP regress spontaneously while those of S-ALCL persist and often progress. Apoptosis has been suggested as the mechanism by which the lesions of LyP regress, but the underlying signaling pathways remain unclear. In this study, we used newly developed activation state-specific antibodies to demonstrate apoptosis signaling through the death receptor-mediated pathway regulated by FADD and caspase 3. Methods: Dual immunohistochemistry for CD30 and activated forms of FADD and caspase 3 was performed on cutaneous biopsy specimens from 27 patients with CD30-positive lymphoproliferative disorders involving the skin. The patients included 18 with primary cutaneous CD30-positive LPDs (15 with LyP and 3 with C-ALCL) and 9 with S-ALCL. Results: The proportion of CD30-positive cells expressing activated FADD was significantly different between primary cutaneous CD30-positive lymphoproliferative disorders and S-ALCL (36.4% vs. 14.5%, P = 0.0083). Expression of cleaved caspase 3 was also significantly different between primary cutaneous lesions and S-ALCL (9.2% vs. 1.9%, P = 0.048). Conclusions: Although a larger number of cases should be studied to validate these results, these data provide evidence that differences in signaling through the death-receptor apoptosis pathway mediated by FADD may be responsible for the varying biologic behaviors of CD30-positive lymphoproliferative disorders involving the skin.

Background and Objective: Technological improvements have helped digital cameras become widely available and inexpensive. Dermatopathologic diagnosis is often aided by clinical information. The purpose of this study was to determine whether submitting a digital image along with a skin biopsy provides additional helpful information to the dermatopathologist. Methods: Digital pictures were taken of the lesion being biopsied and were submitted along with routine paper work to the dermatopathologists. The dermatopathologists interpreted the skin biopsy as usual without viewing the digital image, and then later viewed the submitted picture. The dermatopathologists evaluated the help provided by the image. Results: A clinical digital image was helpful in confirming the initial diagnosis and narrowing down the differential diagnosis ( p < 0.001). Including pictures did not change the diagnosis, result in an increase in the differential diagnosis, or result in fewer or additional step sections being performed. Digital imaging of inflammatory disorders was felt to be good help in 43 % of the biopsies and narrowed down the differential diagnosis in 31%. Conclusion: In certain cases the inclusion of a digital image may help a dermatopathologist. Digital imaging was found to be most helpful in evaluation of inflammatory disorders.

Christie T. Ammirati, MD*, Michael D. Ioffreda, MD, George J. Hruza, MD Department of Dermatology, Pennsylvania State University, Milton S. Hershey Medical Center, 500 University Drive, PO Box 850, Hershey, PA 17033, USA Department of Dermatology and Otolaryngology, St. Louis University School of Medicine, 1402 South Grand Boulevard, St. Louis, MO 63104, USA Laser and Dermatologic Surgery Center, 14377 Woodlake Drive, Suite 111, St. Louis, MO 63017, USA

Basal cell carcinoma (BCC) has many histologic variants. These variants may show differentiation toward benign and malignant tumors of cutaneous appendages, especially hair follicles. Herein we describe a lesion that was clinically thought to be a BCC, but a superficial biopsy showed histologic features suggestive of an apocrine hidrocystoma. Because of some cytologic atypia, complete excision was recommended. The excision specimen revealed a cystic BCC. The importance of examining the entire neoplasm before reaching a final pathologic diagnosis is emphasized.

Eccrine syringofibroadenoma (ESFA) is a benign neoplasm arising from the intraepidermal portion of eccrine ducts. It is characterized by a distinctive histologic pattern of epithelial cells arranged in anastomosing cords surrounded by a fibrovascular stroma. Approximately 50 cases of ESFA have been reported, and in recent years the lesion has been described occurring in association with other skin conditions. We report 2 cases of ESFA arising in abdominal skin adjacent to enterostomy sites.

Generalized atrichia with papules is a rare disorder characterized by loss of hair shortly after birth and development of cutaneous cysts. Mutations in the hairless gene (HR) cause this phenotype in both mouse and human. Here we present a case of atrichia with papules in a patient with a normal HAIRLESS gene but with mutations in both alleles of the VITAMIN D RECEPTOR. The patient exhibited vitamin D resistant rickets, which was confirmed by an absent response of her fibroblasts to 1,25-dihydroxyvitamin D3 in vitro. Similar to individuals with HAIRLESS mutations, her skin showed an absence of normal hair follicles and the presence of follicular remnants and cysts. The cyst epithelium contained keratin-15- and keratin-17-positive cells suggesting derivation from the hair follicle bulge and the presence of epithelial stem cells. Although hair loss has been reported in association with hereditary vitamin D resistant rickets, we now characterize this alopecia as clinically and pathologically indistinguishable from generalized atrichia with papules, which was previously thought to be caused only by mutations in HAIRLESS. These findings suggest that VDR and HR, which are both zinc finger proteins, may be in the same genetic pathway that controls postnatal cycling of the hair follicle.

Importance Nilotinib, a recently approved multitargeted tyrosine kinase inhibitor targeting the BCR-Abl translocation involved in chronic myelogenous leukemia, reportedly produces alopecia according to the package insert, but clinical and histologic descriptions of the alopecia are lacking. Observations A 33-year-old woman with chronic myelogenous leukemia developed widespread alopecia involving scalp and body hair within weeks after starting nilotinib therapy. Biopsies revealed perifollicular lymphocytic inflammation and evidence of follicular injury but normal hair density, consistent with a nonscarring alopecia. Conclusions and Relevance Nilotinib therapy may induce perifollicular inflammation and widespread persistent alopecia. We present the first clinical and histologic description of this potential adverse effect. Further investigation into the underlying mechanism of this adverse effect may produce insights into the hair growth cycle as well as potential therapeutic targets.