Percentage of {Gamma}{Delta} T Cells in Panniculitis by Paraffin Immunohistochemical Analysis.

Cutaneous T-cell lymphomas with panniculitis-like histologic features have different clinical courses depending on whether they are composed of alphabeta T cells or gammadelta T cells, necessitating their distinction for proper prognostication. However, unlike alphabeta T cells, gammadelta T cells cannot be reliably detected in formalin-fixed, paraffin-embedded sections. We demonstrated that a commercially available antibody can detect gammadelta T cells and examined 2 cases of flow cytometry-proven gammadelta T-cell lymphomas and 15 control cases of nonneoplastic panniculitis. In both lymphomas, the atypical lymphocytes were gammadelta T cells, whereas the reactive lymphocytes were alphabeta T cells. In contrast, nonneoplastic panniculitis had predominantly alphabeta T cells with many fewer and individually scattered gammadelta T cells. The detection of gammadelta T cells in paraffin sections provides a powerful new tool to characterize T cells in lymphomas and inflammation

Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas.

Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αβ T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αβ/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases

Cystic acantholytic dyskeratosis of the vulva: An unusual presentation of a follicular adnexal neoplasm

Acantholytic dyskeratosis (AD) is a histologic pattern seen in Darier's disease or dyskeratosis follicularis, warty dyskeratoma, and transient AD. This pattern is characterized by suprabasilar clefting, acantholysis, and formation of corps ronds and grains. We present a case of AD that is unique based on its genital location and cystic architecture. A 53-year-old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration. On microscopic examination, there were fragments of cystic epithelium with areas of hypergranulosis, acantholysis, corps ronds, and corps grains formation. These features are felt by the authors to be a unique presentation of a follicular adnexal neoplasm

Two cases of systemic mantle cell lymphoma involving the skin

Mantle cell lymphoma (MCL) is a form of non-Hodgkin lymphoma that rarely affects skin. Cutaneous involvement is non-specific but usually indicates widespread disease. Herein we present two cases of MCL with secondary skin involvement. One case presented as an acneiform eruption on the face and had aberrant expression of bcl-2 and bcl-6 with weak CD5 expression. The second presented with multiple tumors on the abdomen and thighs. In both cases expression of Cyclin-D1 by the tumor cells was seen. Both patients died shortly after the diagnosis was established

Hyperkeratotic variant of porokeratosis in a patient with Hepatitis C virus infection and a concomitant immunosuppressed state

Porokeratoses are acquired and hereditary disorders of keratinization that share a distinctive lesion characterized by raised keratotic borders corresponding histologically to an angled column of parakeratotic cells, called a cornoid lamella. Although a precise mechanistic explanation is lacking, ultraviolet radiation and immunosuppressed states are considered causally-associated with most cases of acquired porokeratosis. Hepatitis C virus (HCV) infection has been proposed as a link between the immunosuppressed states and development of acquired porokeratosis. Among the various recognized clinical entities that constitute this group, rare cases of hyperkeratotic variants have been described that may pose a diagnostic challenge. Herein we describe a remarkable case of the hyperkeratotic variant of porokeratosis that occurred in a patient with known HIV and HCV infections and a coexisting therapy-related immunosuppressed state. We also provide a review of the relevant literature

Microcystic adnexal carcinoma arising within a nevus sebaceus.

Nevus sebaceus (NS) is a congenital skin lesion arising on the face and scalp that has been linked to the development of various carcinomas. We describe a case of microcystic adnexal carcinoma (MAC) arising in an NS on the scalp of a 62-year-old man. Excisional skin biopsy and hematoxylin and eosin stains were performed to examine the specimen. Serial sections revealed papillomatosis typical of NS, with focal changes consistent with syringocystadenoma papilliferum. Adjacent to the syringocystadenoma papilliferum was an area containing small epithelial islands that extended focally into the subcutaneous layer. The cystic islands were embedded in a desmoplastic stroma with poor circumscription, consistent with MAC. This case presents a rare finding of MAC within an NS

Multiple nodules on the sole of the foot

Ledderhose disease, or plantar fibromatosis, is a benign hyperproliferative disorder of the plantar aponeurosis. It presents as one or more round, firm slow-growing plaques or nodules on the plantar surface of the foot, typically on the medial side. The etiology is unknown, though it has been associated with trauma, liver disease, diabetes mellitus, epilepsy and alcoholism. Histopathological examination of plantar fibromatosis reveals dense fibrocellular tissue with parallel and nodular arrays of fibrocytes and fibrillar collagen with a distinctive cork-screw morphology. The differential diagnosis includes various fibroblastic and myofibroblastic proliferations

Methotrexate-associated lymphoproliferative disorder in a patient with rheumatoid arthritis presenting in the skin.

A 91-year-old woman who had been taking methotrexate for approximately 5 years for rheumatoid arthritis developed papules and nodules on her face that enlarged during 6 months. A series of biopsy specimens demonstrated a lymphoplasmacytic infiltrate with increasingly atypical histopathologic features that resembled diffuse large B-cell lymphoma. Epstein-Barr virus was not identified. Withdrawal of methotrexate resulted in complete resolution of all lesions within 8 weeks. This case illustrates the rare occurrence of methotrexate-associated lymphoproliferative disorder with primary presentation in the skin and documents clinical and histopathologic progression from early changes to fully developed lesions

Cutaneous metastatic chordoma with concomitant tuberous sclerosis.

BACKGROUND: Chordomas are rare malignant tumors of notochord origin and are locally aggressive with a metastatic potential. Of noted interest, the skin rarely is seen as a metastatic site. METHODS AND OBSERVATIONS: We describe a 20-month-old infant whose primary presentation of a clivus-based chordoma was multiple asymptomatic cutaneous nodules. A skin biopsy showed large vacuolated cells embedded in a myxoid stroma. Immunohistochemical staining was positive for S-100, keratin, and epithelial membrane antigen markers consistent with malignant chordoma. Magnetic resonance imaging revealed a clivus-based chordoma with scattered metastases within the neuraxis and multiple visceral sites. Further examination also revealed a diagnosis of tuberous sclerosis. LIMITATIONS: An observed association between chordoma and tuberous sclerosis cannot be established firmly on the basis of a case report. CONCLUSIONS: This case illustrates a diagnostic challenge because of the unusual presentation of an already rare tumor. By reporting our case, we hope to assist in the recognition of this rare dermal condition and highlight the speculated coexistence of tuberous sclerosis with chordoma tumors

Lupus erythematosus induced by medications, ultraviolet radiation, and other exogenous agents: a review, with special focus on the development of subacute cutaneous lupus erythematosus in a genetically predisposed individual.

Exogenous agents implicated in or suspected of precipitating subacute cutaneous lupus erythematosus (SCLE) and lupus erythematosus (LE) are reviewed. An illustrative case of environmentally induced SCLE is presented. A previously healthy 30-year male homozygous for the tumor necrosis factor-alpha (TNF-alpha) 308. A promoter allele developed SCLE after spending several hours removing fertilizer- and pesticide-containing hay from an agricultural barn in the springtime. The cutaneous eruption soon resolved, only to reappear 3 weeks later on the day the patient re-entered the barn. An environmental agent present in the barn, coupled with springtime ultraviolet light, likely triggered the disease in this immunogenetically susceptible individual