Ciliary Gland Adenocarcinoma of the Eyelid Arising in Hidrocystoma: Case Report and Review of Previously Reported Cases.

ABSTRACT: Moll gland is a modified tubular-shaped apocrine sweat gland, which is located on the margin of the eyelid. Moll glands are also known as ciliary glands. The function of these glands was not well known for a long time. However, based on the different studies, it was proved that Moll glands are active from birth and have local immunologic function producing immunoglobulin A. We present a case of Moll adenocarcinoma, a type of apocrine carcinoma, arising from the right upper eyelid of a 57-year-old man. Although Moll gland cysts are frequently seen, this type of adnexal malignancy is extremely uncommon. These tumors can have variable presentation and behavior from less invasive forms to highly malignant metastatic recurrent lesions.

Linear lichen planus pigmentosus of the face with histological findings of lichen planopilaris - an uncommon variant of lichen planus.

Lichen planus pigmentosus and lichen planopilaris are two clinically and histologically distinct forms of lichen planus. Lichen planus pigmentosus presents with sudden onset hyperpigmented macules and patches, predominantly in darker skin phototypes. On the other hand, lichen planopilaris is a scarring follicular variant of lichen planus that presents with progressive, permanent patches of alopecia. It is not uncommon for different variants of lichen planus to clinically coexist with each other. However, to our knowledge, there has been no previous reporting of linear lichen planus pigmentosus of the face with histological features of lichen planopilaris. We herein present a hybrid case of these two entities.

Acrokeratoelastoidosis: is there an association between asthma and sporadic cases in children?

Acrokeratoelastoidosis (AKE) is a rare, benign papular keratoderma that presents as keratotic papules on the lateral margins of the palms and soles. It is most commonly inherited in an autosomal dominant fashion, although sporadic cases are also described. We present a sporadic case of AKE in an 11-year-old girl with a past medical history significant for asthma. On literature review, we found three other cases presenting in children with a past medical history of asthma. We suggest a possible association between asthma and sporadic cases of AKE in children. Current understanding of the pathophysiology of AKE and its associated risk factors is limited and no effective treatment exists. Awareness of a possible association with asthma and atopy, careful history recording in young patients presenting with sporadic cases of AKE, and further research may help to delineate the likelihood of an association between AKE and asthma or atopy. Developing a better understanding of the associated factors that may contribute to the disease process may help guide more effective, targeted treatments in the future.

Linezolid as a treatment option for cutaneous non-tuberculous mycobacterial infections.

Cutaneous non-tuberculous mycobacterial (NTM) infections have rapidly increased in incidence in recent years. Currently there is no standard treatment and the variable and nonspecific ways in which cutaneous NTM infection presents makes it a therapeutic and diagnostic challenge. We describe a 67-year-old immunocompetent woman with cutaneous NTM infection after she recently underwent a root canal procedure. Although the species was not identified and she was unable to tolerate multiple antibiotics, she ultimately responded well to three months of treatment with linezolid. Given that cutaneous NTM infection can present in immunocompetent patients and that the incidence is rising, it is important for clinicians to maintain a high index of clinical suspicion, especially in patients with a recent history of surgery, trauma, or cosmetic procedures. Linezolid has coverage against non-tuberculous mycobacteria and is an effective therapeutic option for cutaneous NTM cases in which identification to the species level is not possible or when adverse effects limit therapeutic options.

Fibro-osseous pseudotumor on the hyponychium of the great toe.

Fibro-osseous pseudotumor of the toe is a benign soft tissue tumor that is predominant in the young adult population. Although the etiology is unknown, a history of trauma has been reported to precede tumor development. The differential diagnosis includes myositis ossificans, extraskeletal osteosarcoma, and pyogenic granuloma. Once removed, the tumor typically has no recurrence. Surgical excision is the treatment of choice. We present a patient with fibro-osseous pseudotumor of the great toe, which had no recurrence following excision.

Apocrine adenocarcinoma of the eyelid: case report and literature review on management.

Apocrine adenocarcinoma of the eyelid is a rare sweat gland cancer. It is predominant in older adults and has increased prevalence in males. Management is based on recommendations from reported cases and their outcomes. Surgical excision is considered effective in apocrine adenocarcinoma of the eyelid. We report the case of a 58-year-old woman with apocrine adenocarcinoma located on her left upper eyelid. Excisional biopsy demonstrated focal apocrine secretion in a basaloid nest proliferation. The patient had no recurrence at four months. Our case provides insight into the workup and management of eyelid apocrine adenocarcinoma. Furthermore, we discuss key management recommendations according to previous authors' experiences with eyelid apocrine adenocarcinoma.

A case of folliculosebaceous cystic hamartoma: a rare and clinically indistinct lesion.

We report a case of a slowly growing papule on the nasal bridge of an elderly woman. Histopathological findings revealed a very unusual type of folliculosebaceous cystic hamartoma with a retiform and primitive epithelial proliferation associated with the stromal component. Folliculosebaceous cystic hamartoma is an uncommon lesion that involves a cystically dilated follicle embedded in a stroma of distinct mesenchymal tissue. Because folliculosebaceous cystic hamartoma is a rare and clinically indistinct lesion, clinical photographs of the lesion are not widely available. A recent search on PubMed revealed less than 50 published articles on folliculosebaceous cystic hamartoma; among these manuscripts there was a scarcity of clinical images of the lesion. This disparity is to be expected as the lesion typically has a very indistinct presentation. We thus describe an inconspicuously appearing case of folliculosebaceous cystic hamartoma to bring attention to the indistinct clinical morphology of the lesion and to report a histopathologically uncommon variant.

Atrophic dermatofibroma in an elderly male - a rarely described variant of a common lesion.

Dermatofibroma frequently presents as a red-brown nodule on the extremities of the middle aged. Atrophic dermatofibroma is a rare variant that has been most commonly described as an atrophic depressed, erythematous lesion in females. The correct diagnosis of atrophic dermatofibroma is often hindered by its infrequent presentation. It has a female preponderance with an occurrence ratio of 10:1. We describe a case of an atrophic dermatofibroma on the back of an elderly man. Skin biopsy demonstrated a spindle cell proliferation in a storiform pattern, loss of elastic fibers, and substantial atrophy of both the underlying dermis and subcutaneous tissue. An aggregation of elastic fibers was found in the periphery of the tumor. These histologic features supported the diagnosis of atrophic dermatofibroma. The dermal and adipocyte atrophy was likely responsible for the retracted appearance of the lesion.

An independent validation of a gene expression signature to differentiate malignant melanoma from benign melanocytic nevi.

BACKGROUND: Recently, a 23-gene signature was developed to produce a melanoma diagnostic score capable of differentiating malignant and benign melanocytic lesions. The primary objective of this study was to independently assess the ability of the gene signature to differentiate melanoma from benign nevi in clinically relevant lesions. METHODS: A set of 1400 melanocytic lesions was selected from samples prospectively submitted for gene expression testing at a clinical laboratory. Each sample was tested and subjected to an independent histopathologic evaluation by 3 experienced dermatopathologists. A primary diagnosis (benign or malignant) was assigned to each sample, and diagnostic concordance among the 3 dermatopathologists was required for inclusion in analyses. The sensitivity and specificity of the score in differentiating benign and malignant melanocytic lesions were calculated to assess the association between the score and the pathologic diagnosis. RESULTS: The gene expression signature differentiated benign nevi from malignant melanoma with a sensitivity of 91.5% and a specificity of 92.5%. CONCLUSIONS: These results reflect the performance of the gene signature in a diverse array of samples encountered in routine clinical practice. Cancer 2017;123:617-628. © 2016 American Cancer Society.

A rare case of cutaneous acanthamoebiasis in a renal transplant patient.

A 35-year-old woman receiving immunosuppressionfor renal transplantation presented with a onemonthhistory of tender skin nodules on herbilateral upper extremities. A skin biopsy revealedgranulomatous inflammation in the deep dermisand the subcutaneous fat with foci of necrosis.Within the foci of necrosis were large histiocytoidstructures with prominent nuclei. Periodic acid-Schiffstain revealed a round organism with a thick capsule,consistent with amoebal trophozoites. Testing withthe Center for Disease Control revealed the organismto be Acanthamoeba. Despite antimicrobial therapy,the patient continued to develop subcutaneousnodules that extended to the lower extremities andtrunk and ultimately extended to the bone, causingacanthamoebal osteomyelitis. Throughout thehospital course, the patient remained neurologicallyintact without evidence of central nervousinvolvement. A diagnosis of isolated disseminatedcutaneous acanthamoebiasis secondary to iatrogenicimmunosuppression was made. Historically, mostcases of granulomatous amoebic encephalitisand cutaneous acanthamoebiasis have occurredin patients with HIV/AIDS. However, with the useof newer and more effective immunosuppressiveregimens, both are occurring more frequently inthe setting of iatrogenic immunosuppression. Therare and isolated cutaneous nature of this patient'spresentation makes this case unique.

Disseminated Mycobacterium avium Complex With Cutaneous Lesions.

BACKGROUND: Although most commonly encountered in patients with human immunodeficiency virus infection, disseminated Mycobacterium avium complex (MAC) is becoming more common in patients receiving immunosuppressive medications. Disseminated MAC with skin lesions may occur, and several presentations have been reported, including panniculitis, cutaneous granulomas, pustules, ulcerations, and erythematous skin lesions. OBJECTIVES: The objective of this report is to describe an unusual presentation of MAC that is unlikely to be encountered frequently in the outpatient dermatology setting, especially in a patient without human immunodeficiency virus infection. METHODS: The authors present a case of disseminated MAC infection with cutaneous manifestations in an iatrogenically immunocompromised patient. CONCLUSIONS: Diagnosis of MAC infection is challenging given the varied clinical presentations and the difficulty in culturing MAC. In addition, the acid-fast stain is nonspecific. Clinicians should remember to consider MAC infection in patients with acid-fast-positive skin lesions, as the selection of appropriate antibiotic therapy is species specific.

The routine use of iron stain for biopsies of dermatoses of the legs.

BACKGROUND: Our goal was to study the utility of the routine use of the Gomori iron stain in the evaluation of leg biopsies of inflammatory lesions. METHODS: One hundred consecutive skin biopsies from the legs were evaluated with hematoxylin and eosin and Gomori iron stains. Iron positivity was semi-quantitatively graded from trace to 4+. RESULTS: Forty-two (42) cases were positive with the Gomori iron stain as follows: stasis dermatitis (14), Schamberg's disease (7), folliculitis (2), psoriasis (2), trauma (2), arthropod bite (2), allergic contact dermatitis (2), lichen simplex chronicus (1), senile purpura (1), vascular ectasia (1), lobular capillary hemangioma (1), scar (1), pretibial pigmented patches (1), tinea (1), lentigo maligna (1), traumatic fat necrosis (1), lichen planus (1) and fixed drug eruption (1). Twelve of 14 cases of stasis dermatitis had 2+ or greater iron staining; 8 cases were 3+ or 4+. All other diagnoses had trace or 1+ staining for iron except for one scar (2+). One of 19 samples (5.3%) of 'normal' leg skin controls showed iron stain positivity, and was graded as trace. CONCLUSIONS: After correcting for iron staining in 5.3% of normal leg skin controls, we observed Gomori iron positivity in 36.7% of 100 consecutive leg biopsies. We suggest that routine application of an iron stain on biopsies of dermatoses from the legs is useful. Stasis dermatitis and Schamberg's disease are the most frequent iron positive diagnoses, and the diagnosis is aided by the pattern of hemosiderin deposition. Negative iron stain is also useful in suggesting the presence of melanin pigment in macrophages in lichenoid and other dermatoses. To confirm a truly negative iron stain, study of the section at ×400 rather than ×100 is recommended.

Choriocarcinoma-like squamous cell carcinoma: a new variant expressing human chorionic gonadotropin.

Cutaneous squamous cell carcinoma (SCC) has been classified into a large number of subtypes, which have been grouped according to malignant potential. We describe a new morphological variant containing areas of intermingled cytotrophoblast-like and syncytiotrophoblast-like cells and designate it "choriocarcinoma-like SCC." Furthermore, the neoplasm exhibits expression of human chorionic gonadotropin predominantly in the syncytiotrophoblast-like foci, mimicking the germ cell tumor. Human chorionic gonadotropin expression has been described in SCC from other organs, but not by cutaneous SCC, to our knowledge. This new variant could be misinterpreted as metastatic choriocarcinoma, especially in small punch biopsies. The frequency of this neoplasm and its malignant potential are undetermined.

Blastic plasmacytoid dendritic cell neoplasm: evolving insights in an aggressive hematopoietic malignancy with a predilection of skin involvement.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) derived from precursors of plasmacytoid dendritic cells is a very rare, unique, and highly aggressive immature hematopoietic malignancy, more frequently occurring among healthy elderly adults. BPDCN can be characterized by a striking predilection for cutaneous involvement, which is often detected incidentally by dermatologists and is difficult to clinically distinguish it from other primary skin lesions and histologically from leukemia/lymphoma cutis. Thus, histological diagnosis of cutaneous biopsies is crucial to correctly classify this entity. Most patients eventually progress to acute myeloid leukemia and are generally not curable. Here, we present 2 cases of classic BPDCN and discuss the origin of tumor and literature-based characteristic clinical and morphological features, evolving immunomarkers, and molecular genetic aspects of this neoplasm.

Radiation-associated atypical vascular lesions: vascular lesions with endothelial cell atypia presenting in the radiation port of breast cancer patients.

Atypical vascular lesions are an uncommon adverse sequela to the radiotherapy of tumors. Many characteristics are shared between atypical vascular lesions caused by radiation port and well-differentiated radiation-induced angiosarcomas. The authors retrospectively reviewed the medical literature using PubMed, searching the terms acquired, atypical, benign, lymphangioma, lymphangioendothelioma, lymphangiomatous, lesion, papules, progressive, and vascular. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. Atypical vascular lesions associated with the radiation port present as clinically innocuous flesh-colored to erythematous papules or plaques. The condition presents within the radiation field, approximately 3 years after initial treatment. While the exact me chanism remains to be elucidated, growing evidence supports an association between radiation-associated atypical vascular lesions and radiation-induced angiosarcomas. Atypical vascular lesions within a radiation port are suggested to be in a state of morphologic continuum, which may progress into the more aggressive, malignant angiosarcoma. The authors recommend consideration for biopsy of new skin lesions within or adjacent to radiation. While it is clear that atypical vascular lesions caused by radiation are not equivalent to angiosarcoma, growing evidence supports that radiation-associated atypical vascular lesions may progress to angiosarcoma in some patients; therefore, the authors recommend excision of the lesion with margins depending on clinical judgment and the lesion encountered.

Intratumoral heterogeneity of chromosome 9 loss and CDKN2A (p16) protein expression in a morphologically challenging spitzoid melanoma.

In young adults and children, spitzoid melanoma carries striking resemblance to spitz nevus clinically and histopathologically. Many have attempted to differentiate between these 2 entities by searching for immunohistochemical and molecular diagnostic differences. Here, the authors report a spitzoid melanoma in a young adult, which was morphologically similar to a spitzoid nevus and carried a loss of chromosome 9 detected by an array-based comparative genomic hybridization. However, the expression of cyclin-dependent kinase inhibitor 2A (p16) protein, encoded on chromosome 9p21, detected by the immunohistochemical study was heterogeneous among tumor cells. This case demonstrates the presence of clonal heterogeneity of losses of chromosome 9 and p16 protein expression within a single spitzoid melanoma, challenging a robust application of p16 expression detected only by immunohistochemical staining in determining the diagnosis of spitzoid melanoma.

Molluscum contagiosum of the areola and nipple: case report and literature review.

Molluscum contagiosum is a common cutaneous infection caused by a double-stranded DNA poxvirus. Skin lesions classically present as small, flesh-colored papules with central umbilication. Lesions are frequently seen on the face, trunk, and extremities of children, or on the genitals of young adults as a sexually transmitted infection. Molluscum contagiosum on the nipple or areola has only been previously described in 4 women. We describe a woman with molluscum contagiosum on the left areola and review the clinical characteristics and histological findings of patients who developed molluscum contagiosum of the nipple or areola.

Atypical Pediatric Presentation of Pilomatricoma.

Pilomatricomas are uncommon, benign tumors of the hair follicle, which are often misdiagnosed upon initial inspection. Here we describe the case of a 4-year-old boy who presented with a persistent draining tumor on the left side of his neck for approximately two years. The tumor was originally misdiagnosed as scrofuloderma but, eventually, our patient's pilomatricoma was identified with biopsy and successfully treated with elliptical excision. We discuss the importance of considering pilomatricoma in the differential diagnosis.

Growth of a Nipple Adenoma After Estrogen Replacement Therapy.

A nipple adenoma is an epithelial tumor of the lactiferous ducts, typically affecting women aged 50-60 years old. This case report discusses a 52-year-old woman who developed a papillary adenoma of the right nipple after initiating oral estrogen replacement therapy (ERT) for perimenopausal symptoms. A 4 mm punch biopsy and subsequent immunohistochemistry stain revealed the proliferation of ductal structures consistent with a papillary adenoma and tumor cells expressing estrogen receptors (ER) and progesterone receptors (PR). Despite their benign nature, nipple adenomas may exhibit alterations in immunophenotype, including ER and PR expression, which could lead to potential tumor growth in women undergoing these treatments. This case describes the first reported growth of a nipple adenoma in the context of estrogen replacement therapy, highlighting a potential risk of hormone therapy in promoting hyperproliferation of benign tumors such as nipple adenomas. When utilizing ERT, it is important to weigh the potential advantages and risks, as its application in the management of vasomotor symptoms during menopause may increase the risk of both breast cancer and benign proliferative breast diseases. These considerations underscore the need for individualized therapy when approaching perimenopausal and postmenopausal care.