A Case of a Rare Histologic Variant: Cavernous Hemangioma-Like Kaposi Sarcoma.

ABSTRACT: Kaposi sarcoma (KS) is an endothelial tumor associated with human herpesvirus 8. Cutaneous lesions can present with pink or purple patches, plaques, and nodules which can be ulcerated. The main subtypes of KS generally have similar histologic appearances, with spindle cells and expression of human herpesvirus 8 being characteristic features. However, various histologic variants have been reported. We present the case of a 55-year-old man with cutaneous KS with cavernous hemangioma-like histological features. Cavernous hemangioma-like KS is a rare morphologic type of KS, with only a handful of cases reported in the literature.

Metastatic vulvovaginal Crohn disease in the setting of well-controlled intestinal disease.

The cutaneous manifestations of Crohn disease (CD) are varied and include pyoderma gangrenosum, erythema nodosum, and metastatic CD (MCD). The latter is defined as the occurrence of granulomatous lesions at a skin site distant from the gastrointestinal tract. Metastatic CD involving the vulva and perineum is rare and thus often is difficult to diagnose. It may precede, coincide with, or develop after the initial diagnosis of intestinal disease. A variety of clinical presentations have been described, including widespread nonspecific pain and swelling, erythematous papules and plaques, and nonhealing ulcers. The diagnosis often is delayed because of a low index of suspicion brought about by the rarity of the disease and its diverse and confusing manifestations. A skin biopsy usually confirms the diagnosis by revealing noncaseating granulomas in the dermis. Multiple oral and parenteral therapies are available, with surgical intervention reserved for resistant cases. We present a case of vulvovaginal MCD in the setting of well-controlled intestinal disease.

Basal cell carcinoma and malignant melanoma cutaneous collision tumor.

Malignant tumors composed of both epithelial and melanocytic populations are exceedingly rare. Because the etiology of these malignant tumors is not yet defined, several authors have argued for the continued cataloging of these lesions. We present a 67-year-old man with a suspicious-appearing lesion on the right forearm; initial biopsy was consistent with a pigmented basal cell carcinoma. On re-excision, a collision of malignant melanoma and basal cell was identified.

Disseminated Syringomas of the Upper Extremities in a Young Woman.

Syringomas are benign, eccrine sweat gland tumors frequently found on the eyelids and neck in post-pubescent women and may present in healthy individuals or be associated with various medical comorbidities. We present a case of an otherwise healthy 19-year-old female with an abrupt onset of disseminated syringomas on the bilateral forearms and dorsal hands. Eruptive acral syringomas have not been previously reported in adolescents, and this diagnosis should be considered in patients presenting with a papular eruption on the hands and forearms.

Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoproliferative disorder: Report of a case and review of the literature.

Primary cutaneous small/medium-sized T-cell lymphoma (PCSM-TCL), which was included in the World Health Organization - European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas as a provisional entity in 2008, has recently been reclassified as primary cutaneous small/medium-sized T-cell lymphoproliferative disorder (PCSM-TCLPD) because of its indolent behavior and uncertain malignant potential. Treatment with local therapies is usually curative, although there have been reports of aggressive, systemic disease. This spectrum of disease behavior evokes the consideration that this entity may actually be multiple diseases with a shared clinicopathologic features rather than a singular disease process with a variety of behaviors. PCSM-TCLPD retained its designation as a provisional entity under the updated WHO-EORTC guidelines; however, additional cases of PCSM-TCLPD are needed to shed more light on this rare disorder.

Perforating granulomatous dermatitis reaction to exogenous tattoo pigment: a case report and review of the literature.

: The majority of cutaneous hypersensitivity reactions to exogenous tattoo pigments can be histologically classified as lichenoid or granulomatous. The etiology is still uncertain but is generally accepted to be a delayed-type hypersensitivity reaction to either the pigment itself or its carrier solution. In this report, we review the literature concerning adverse reactions to tattoos. In addition, we describe the second case of a localized granulomatous dermatitis to the red dye within a tattoo that histologically resembled granuloma annulare. This is the first reported example of a perforating granuloma annulare-like reaction.

Traumatic neuromas of the penis: a clinical, histopathological and immunohistochemical study of 17 cases.

We present 17 penile traumatic neuromas. The mean patient age at presentation was 38 years (range 23-59 years). The most common site involved was the penile shaft. The lesions ranged from 1 to 7 mm in greatest dimension. The clinical diagnosis in all cases included condyloma acuminatum. In all cases, a history of trauma because of prior biopsy and/or circumcision was found. Histologically, all lesions showed similar features consisting of an increased number of dermal nerve bundles embedded within a fibrous stroma. Often, single or multiple Meissner corpuscle-like structures were noted in the papillary dermis. Our study suggests that circumcision or other forms of trauma to the skin of the penis likely plays an important role in the pathogenesis and clinical presentation of this peculiar neural neoplasm. We call attention to this entity because it is often clinically misdiagnosed as condyloma acuminatum.

A Solitary Neck Nodule as Late Evidence of Recurrent Lobular Breast Carcinoma.

Recurrent lobular breast carcinoma manifesting as a cutaneous neck nodule in a woman, 14 years after successful chemotherapy, illustrates the importance of following at-risk patients with a high level of clinical suspicion. This case emphasizes the value of combining clinical findings with appropriate histopathologic and immunohistochemical analysis when evaluating a cutaneous lesion in such a patient.

Sarcoidosis with subcutaneous lesions.

A 30-year-old man presented for evaluation of multiple, hypopigmented macules and erythematous papules on his upper and lower lips and upper extremities. He subsequently developed firm, non-tender, subcutaneous, mobile nodules on the left forearm, left calf, and right thigh. Clinical and histopathologic features were consistent with cutaneous and subcutaneous sarcoidosis. Systemic evaluation showed no evidence of extracutaneous involvement. Subcutaneous sarcoidosis is a rare form of nodular cutaneous sarcoidosis. Typical skin findings are painless, firm, mobile nodules without overlying epidermal involvement and with a predilection for the trunk and extremities. Histopathologic alterations include epilthelioid cell tubercles in the subcutaneous fat. As this is a diagnosis of exclusion, other etiologies of granulomatous inflammation must be ruled out with tests and special stains. The mainstay of treatment is with oral glucocorticoids.

Epidermodysplasia verruciformis.

A 29-year-old woman presented for evaluation of an ulcerated nodule on her right temple in the presence of numerous additional scaly papules, plaques, and seborrheic keratosis-like lesions that were distributed on her forehead, frontal scalp, and temples. Further examination showed pityriasis rosea-like lesions on her neck, back, and arms and verruca plana-like lesions on the dorsa of the hands and forearms. A biopsy specimen of the ulcerated nodule showed a nodular basal-cell carcinoma. Two additional biopsy specimens from her forehead showed one invasive and one in-situ squamous-cell carcinoma. Both biopsy specimens demonstrated histopathologic evidence of epidermodysplasia verruciformis.

Hereditary papulotranslucent acrokeratoderma.

A 79-year-old woman presented with a history of peeling of the palms and soles that began in young adulthood, with exacerbation after exposure to water. Her mother, 2 sisters, and a female maternal cousin have similar symptoms. Physical examination showed scale and hyperlinearity of the palms. Brief exposure to water initiated the development of 1-to 2-mm, translucent, white papules that were distributed diffusely on the palmar surface, with a concentration at the palmar margins and pressure points. Histopathologic examination showed an acanthotic epidermis with a central depression that was filled with compact orthokeratosis. The physical examination and histopathologic findings are consistent with a diagnosis of hereditary papulotranslucent acrokeratoderma.

X-Linked ocular albinism; Nettleship-Falls ocular albinism.

A 39-year-old man with foveal hypoplasia, nystagmus, and decreased visual acuity was found to have multiple, cutaneous, hypopigmented macules. Macromelanosomes were demonstrated in normal skin on histopathologic examination. The patient's constellation of findings along with a strong X-linked inheritance pattern in family members led to the diagnosis of X-linked ocular albinism, which is an uncommon condition that is characterized by congenital nystagmus, iris translucency, hypopigmentation of the ocular fundus, strabismus, foveal hypoplasia, photophobia, and impaired vision.

Nephrogenic fibrosing dermopathy.

A 75-year-old woman with end-stage renal disease on hemodialysis presented with a 2-month history of progressive skin thickening of the lower extremities. A punch biopsy specimen showed plump fibroblasts entrapping collagen bundles and positive staining for CD34 and procollagen. These changes were consistent with a diagnosis of nephrogenic fibrosing dermopathy (NFD). Nephrogenic fibrosing dermopathy is a rare, sclerosing disorder in patients with renal failure, which may be mistaken for scleromyxedema. The etiology of NFD is unclear but may be associated with systemic involvement, antecedent surgical procedures, gadolinium, or an underlying hypercoagulable state. The treatment is limited, and only a few reported cases have shown remission after stopping dialysis in transient renal failure.

Multiple apocrine hidrocystomas.

A 83-year-old woman presented with a 7-year history of translucent papules that were scattered diffusely over her nose, peri-orbital region, and cheeks. These lesions were exacerbated by heat and exercise. Histopathologic examination of a biopsy specimen from the cheek showed a thin-walled cyst lined by a flattened bi-layered epithelium that exhibited decapitation secretion in the upper part of the epidermis. A diagnosis of multiple apocrine hidrocystomas was made based on the clinical and histopathologic findings, and the patient is currently considering treatment options. These include electrodesiccation, excision, trichloracetic acid, carbon-dioxide laser, and 1450-nm diode laser.

Erythrokeratoderma variabilis.

A 51-year-old woman presented with well-demarcated, erythematous patches and hyperkeratotic plaques that were arranged symmetrically on the upper extremities. Her skin lesions first appeared at age 17, and there was a family history of similar skin lesions in nine of her eleven siblings. Physical and emotional stress increased the number and intensity of her skin lesions as did chocolate. The distribution of lesions on her skin had changed over time; however, the lesions consistently appeared symmetrically and favored the extensor surfaces of her extremities. The physical examination, histopathologic findings, and family history were consistent with a diagnosis of erythrokeratoderma variabilis (EKV), a rare genodermatosis caused by various mutations in connexin genes. Connexin genes code for proteins that form intercellular channels called gap junctions that allow for transport and signaling between neighboring cells in the epidermis. Mutations in connexin-31 and connexin-30.3 are known to cause the EKV phenotype, which presents as erythematous patches and hyperkeratotic plaques. Studies to examine the effectiveness of treatment of EKV have not been performed; however, several case reports suggest the efficacy of oral retinoids. After 4 months of acitretin 25 mg per day, our patient experienced near complete clearance of her skin lesions.

Papular mucinosis (discrete papular lichen myxedematosus).

A 56-year-old woman presented with small, skin-colored papules on the trunk and thighs. Histopathologic findings were consistent with papular mucinosis. Laboratory evaluation did not show an associated paraproteinemia. Treatment with topical glucocorticoids was unsuccessful. Papular mucinosis, also known as localized lichen myxedematosus, has been categorized into 4 subtypes. The discrete papular form, as seen in our patient, is characterized by a few to multiple, 2-5-mm, skin-colored, firm, waxy, dome-shaped papules on the trunk and proximal aspects of the extremities. By definition there is no associated paraproteinemia, but this form may be associated with human immunodeficiency virus infection. Focal or diffuse mucinous deposits are seen on histopathologic examination. The usual clinical course is slow cutaneous progression without spontaneous resolution. Treatment is empiric and is usually unsuccessful.

Anaplastic large-cell T-cell lymphoma.

A 64-year-old woman presented with 2 years of pruritic and ulcerated nodules and tumors on the trunk and arms. Histopathologic examination showed a diffuse infiltrate that consisted of predominantly small lymphocytes and scattered large atypical multinucleated cells positive for CD30. These findings were consistent with a diagnosis of anaplastic large-cell T-cell lymphoma, which is a CD30+ cutaneous lymphoma. This case highlights the importance of considering both histopathologic and clinical criteria in diagnosing a patient with a CD30+ cutaneous lymphoma.

Amalgam tattoo.

A 53-year-old woman with a history of melanoma status-post excision two years prior presented with a 4-month history of 4, dark-brown macules on the inferior surface of her tongue. A biopsy specimen showed a squamous mucosa with chronic submucosal inflammation and brown pigment. The clinical and histopathologic findings were consistent with a diagnosis of amalgam tattoo. Amalgam tattoos are common, oral pigmented lesions that clinically present as isolated, blue, grey, or black macules on the gingivae, the buccal and alveolar mucosae, the palate, and/or the tongue. They are due to deposition of a mixture of silver, tin, mercury, copper, and zinc, which are components of an amalgam filling, into the oral soft tissues. Amalgam tattoos can either be treated surgically or with a Q-switched ruby laser. In the case of our patient with the history of melanoma, her oral lesions proved not to be the more dire diagnosis of malignant melanoma.