Nephrogenic Fibrosing Dermopathy: A Review of the Massachusetts General Hospital Experience

Mycosis fungoides with central nervous system (CNS) involvement is a rare event. Most cases evolve from progressive tumor stage skin disease or CD30 large cell transformation. Cases with CD 30 transformation can show CNS involvement without advanced skin disease. We report a 63 year‐old woman with 6 year history of mycosis fungoides with patch and plaque stage disease who developed a single 2.5 cm tumor on the left chest without lymphadenopathy. Histology revealed a dense infiltrate of small atypical lymphocytes with cerebriform nuclei. Immunohistochemistry showed CD 4+, CD 43+ and CD 30‐ staining. Within 2 weeks she developed ataxia and right‐sided weakness. Magnetic resonance imaging (MRI) revealed left frontal and brainstem masses. Stereotactic biopsy showed a dense infiltrate of atypical lymphocytes with large convoluted nuclei. The immunohistochemistry showed a population of CD 4+, CD 43+, Alk‐1‐ cells with CD 30+ transformation. Gene rearrangement studies for the gamma T cell receptor on the skin patch and tumor as well as brain tumor failed to reveal a monoclonal population in any of the specimens. No other areas of involvement were found on complete staging. This case is an example of tumor stage MF with CD 30 transformation and CNS involvement.     

Primary Cutaneous Carcinosarcoma (PCCS0 Aka Metaplastic Carcinoma)

A 25‐year‐old man presented with a large, undermined, violaceous ulcer with hemorrhagic crust on his nose for 1 year. It began as a "pimple". He had similar lesions on his scalp, abdomen and groin. His sister has Crohn's and his mother has rheumatoid arthritis. Screening blood tests and CT scans revealed no underlying disease. The clinical impression was pyoderma gangrenosum. Biopsies from the nose and abdomen revealed dense superficial and mid‐dermal perivascular lymphocytic infiltrate composed of enlarged, atypical lymphocytes with focal epidermotropism and epidermal necrosis. Immunohistochemistry revealed the infiltrate was primarily CD3 positive T‐cells without deficiency of CD4, CD7 or CD8. CD30, TIA and Granzyme were not detected. A T‐cell receptor gamma gene rearrangement was detected. Flow cytometry was negative. This is a case of pyoderma gangrenosum‐like cutaneous T‐cell lymphoma, a rare form of cutaneous lymphoma that clinically resembles PG, but histologically and immunohistochemically is cutaneous T‐cell lymphoma. Although rare, PG‐like ulcers should be added to the list of atypical manifestations of CTCL. This case highlights the need to evaluate all atypical lymphocytic infiltrates thoroughly with immunohistochemistry and gene rearrangement. The diagnosis must be made with caution as proliferative clones of lymphocytes can be found in true PG.     

Aggressive Peripheral T‐cell Lymphoma Presenting as Cutaneous Nodules with a Dense Infiltrate of Eosinophils

A 65 year‐old, well‐appearing and asymptomatic man presented with a small non‐healing ulcer on the right palm present for 6 weeks. He reported a 15 year history of hand and foot "eczema" treated with topical steroids. Six months earlier his right 4th toe had been amputated for "infection", but additional information was not immediately forthcoming. Punch biopsy from the palm demonstrated superficial ulceration, dermal eosinophils and possible cytoplasmic inclusions within keratinocytes, leading to suspicion of parapox infection. Over the ensuing two months his condition deteriorated markedly, and he developed multiple fungated and ulcerated cutaneous nodules. Incisional biopsy of the nodule on the right palm revealed a dense dermal inflammatory infiltrate with a predominance of eosinophils, and a perivascular infiltrate of medium to large lymphocytes with irregular nuclei and mitoses. These atypical lymphocytes were positive for CD3, but were negative for CD4, CD8, CD30, CD56, TIA‐1 and EBER in‐situ hybridization. TCR‐gamma gene rearrangement revealed a clonal population. Retrospective analysis of the amputated toe demonstrated similar features. He declined conventional treatment, and has been reluctant to pursue follow‐up. Our case highlights the fulminant course and dense mixed inflammatory cell infiltrate with eosinophils often present in peripheral T‐cell lymphoma that may complicate diagnosis.     

NK/T‐Cell Lymphoma Presenting as Subcutaneous Nodules in a Previously Healthy Young Woman: an Unusual Granulomatous Component Found on Biopsy

We present the case of an HIV‐positive 38‐year‐old Caucasian male with a history of fevers, chills, and disseminated nonpruritic erythematous papules that began on his abdomen. An initial skin biopsy revealed a lymphohistiocytic infiltrate at the dermoepidermal junction, exocytosis of atypical lymphocytes, and minimal spongiosis. Immunohistochemistry showed increased CD8‐positive cells but only scattered CD30‐positive cells. PCR analysis demonstrated T‐cell receptor gamma gene rearrangement. RPR, blood and tissue cultures were all negative. The patient was thought to have a cytotoxic cutaneous T‐cell lymphoma. Over time, the lesions progressed to the palms and soles and the patient remained febrile. Repeat biopsy demonstrated a lichenoid interface dermatitis with a superficial and deep perivascular, interstitial, and periadnexal lymphohistiocytic infiltrate and the formation of epithelioid granulomas throughout the dermis. The epidermis showed blurring of the dermoepidermal junction, spongiosis, and exocytosis of lymphocytes and neutrophils. Few spirochetes were demonstrated by Steiner stain. Repeat RPR and FTA‐Abs serologies were positive. The patient was diagnosed with late secondary syphilis and was successfully treated with benzathine penicillin. This case demonstrates that atypical lymphoid infiltrates can simulate mycosis fungoides in an HIV‐positive patient with secondary syphilis and also reiterates that syphilis is a great mimicker of other entities.     

I Forgot to Shave My Hands: Spiny Keratoderma of the Palms and Soles

Lymphomatoid papulosis (LyP) is a cutaneous CD30+ lymphoproliferative disorder characterized by recurrent papulonecrotic or papulonodular lesions with histologic features of a malignant lymphoma and an excellent prognosis. Three morphologic types (A, B, and C) of LyP exist and typically display an immunophenotype of CD4+, CD8−, and CD30+. We present a case of a 63‐year‐old woman with a one‐year history of relapsing papulonecrotic skin eruptions. Biopsy of a lesion on the forearm showed a superficial and deep dermal wedge‐shaped infiltrate with large lymphoid cells (Type A LyP). A lesion on the abdomen showed a band‐like superficial dermal infiltrate composed of small lymphoid cells with extensive epidermotropism and cerebriform nuclei (Type B LyP). Lastly, a lesion on the buttock histologically showed a dense, diffuse dermal infiltrate composed of a mixture of small and large lymphoid cells (Type C LyP). In all three lesions, the atypical lymphocytes were immunoreactive to CD8 and CD30 and nonreactive to CD4. CD8 positive cases of LyP are rare in the literature and tend to occur in pediatric patients. Moreover, while approximately 10% of patients may present with lesions of both the type A and type B types, cases showing all three types of LyP are exceedingly rare.     

Tumor‐stage mycosis fungoides in palmoplantar localization with large‐cell transformation and partial CD30 expression shows complete response to brentuximab vedotin

Mycosis fungoides in palmoplantar localization (MFPP) is a rare variant of MF that is confined to the hands and feet. Patients commonly receive treatment over many years for suspected palmoplantar dermatitis before the diagnosis is made. Most MFPP patients remain at patch or plaque stage, and often respond to treatment with radiotherapy. Herein, we describe a 77‐year‐old man who suffered 6 years of hand and foot dermatitis that failed multiple treatments, most notably TNF‐α inhibitors and mycophenolate mofetil. He eventually developed a tumor on the hand, which was biopsied to reveal a dense dermal infiltrate of large lymphocytes (CD3+/CD4‐/CD8‐/TCR‐BetaF1+/partial CD30+). A subsequent biopsy of an eczematous patch from his hand revealed an epidermotropic and syringotropic infiltrate comprised of smaller lymphocytes with a concordant immunophenotype and matching clonal peak with TCR gene rearrangement. He was diagnosed with MFPP and started on radiotherapy with a modest response; therefore, a decision was made to start brentuximab vedotin, which resulted in a complete response. MFPP is an exceedingly rare variant of MF that can show large‐cell transformation and progress in stage. We highlight a possible association between disease progression and immunosuppressants and the potential role for treatment with brentuximab.     

FS04.8The U.S. Cosmetic Ingredient Review: process and products

Methyl(chloro)isothiazolinone is an effective preservative and a major cause of cosmetic allergy in most European countries. On the face unusual clinical presentations are seborrhoeic dermatitis, lupus erythematosus, lymphocytic infiltrate, photodermatosis or atopic dermatitis. Three patients who suffered a chronic, recurrent, itchy and generalized cutaneous erytematous‐desquamative and eczematous eruption with a common pathologic event, an atypical lymphoid infiltrate, whose induction by a contact allergen can be discussed are presented. The first, a 59 years old man showed a biopsy with an atypical dermal‐epidermal T cell infiltrate of mycosis fungoides and monoclonal TCR‐rearrangement. The second, a 74 years old man showed an atypical lymphoid infiltration with polyclonal TCR‐rearrangement and a second biopsy showed an eczematous pattern. The third, a 50 year old man with pathology of plaque parapsoriasis with atypical lymphoid infiltrate. The three patients showed strong positive reaction to methyl(chloro)isothiazolinone (0.01%aq.) Trolab,,μ. Avoiding the allergen the cutaneous eruption disappeared and any recurrence has been observed yet. Pathologic criteria for mycosis fungoides remains controversial and could not be done only on the basis of cellular density or the percentage of atypical T‐cells. Autoinvolutive mycosis fungoides shows the unknowledge of its pathogenesis. How some allergens could affect on the cellular life needs further studies. We highly recommend to patch test all patients with atypical cutaneous T‐cell infiltrates.     

Interstitial Granulomatous Dermatitis with Plaques

An 8‐year‐old male presented with several 1–4 cm, indurated, brownish‐red nodules with superficial, punctate hemorrhagic crusts localized to the groin, which developed over several weeks. An initial biopsy was consistent with Langerhans cell histiocytosis. A work up, including a chest and abdominal CT scan demonstrated a large mediastinal mass which was diagnosed as precursor T cell lymphoblastic lymphoma. Treatment with a chemotherapeutic protocol resulted in shrinkage of both the mediastinal and cutaneous lesions. Late in the course of treatment the cutaneous lesions rapidly recurred and spread to various sites. A second skin biopsy showed an extensive diffuse infiltrate of histiocytic‐appearing cells extending from the papillary dermis into the superficial subcutis. The cells had markedly pleomorphic, vesicular nuclei and abundant amphophilic cytoplasm. Numerous mitotic figures were present. The neoplastic cells were S‐100 protein and CD1a positive by immunohistochemistry. The histopathologic diagnosis was Langerhans cell sarcoma. Gene rearrangement studies demonstrated clonal rearrangement of the T cell receptor gamma gene in specimens from both the mediastinal mass and one of the recurrent cutaneous lesions. The sizes of the PCR products were identical demonstrating a clonal relationship between the two neoplasms.     

Mycosis fungoides involving an acrochordon: a case report

We present the case of a 77‐year‐old male undergoing treatment for mycosis fungoides (MF) who presented for removal of an acrochordon on his mid back. Histopathologic examination of the acrochordon revealed a dense, band‐like lymphocytic inflammatory infiltrate in the dermis with epidermotropism of single lymphocytes and small nests of lymphocytes into the lower epidermis. Immunohistochemical staining characterized the dermal and epidermal lymphocytic population as CD3‐positive T lymphocytes with a predominance of CD4‐positive over CD8‐positive lymphocytes. These findings were consistent with the patient's known MF and molecular identification of a clonal T‐cell receptor gene rearrangement further supported the diagnosis. Our unusual case reports MF involving an acrochordon and provides evidence to support the importance of submitting acrochordons for histopathologic examination.     

Fatal Subcutaneous Panniculitis‐Like T‐Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead‐Ringer for Lupus Erythematosus

We report a 48‐year‐old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4−CD8+/−CD56+. Monoclonal rearrangements of T‐cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis‐like T‐cell Lymphoma (SPTCL) with systemic involvement.     

Marginal Zone Lymphoma with a Dual Cutaneous and Leukemic Presentation and an Aberrant Myeloid‐Monocytic Phenotype

A 69‐year‐old man with heart failure was hospitalized with worsening dyspnea. At the time of admission he also complained of a "rash" of three weeks duration over the left neck. Multiple erythematous plaques were noted. There was no lymphadenopathy or hepatosplenomegaly. The white blood cell count was elevated at 37,700/uL, with 70% composed of atypical monocytoid lymphocytes with abundant pale‐staining cytoplasm. Skin biopsy showed a superficial and deep nodular dermal lymphoid infiltrate. Bone marrow aspirate and biopsy showed nodules of similar cells. Flow cytometry identified a monoclonal B cell population with light chain restriction, with positivity for CD19, CD20, HLA‐DR, CD11c, FMC7, CD71, and the myeloid‐monocytic marker CD13. There was no expression of CD5 or CD10. B cell receptor gene rearrangement was identified. A diagnosis of extranodal marginal zone lymphoma was made. He was treated for congestive heart failure and discharged. The patient has had an indolent course without chemotherapy, with waxing and waning of the skin lesions. This case is unusual, as leukemic involvement is rare in marginal zone lymphoma and is typically only seen in patients with severe widespread disease with an aggressive clinical course. This is also the first reported case with aberrant expression of myeloid‐monocytic markers.     

Atypical fibroxanthoma with lymphomatoid reaction

Background: Atypical fibroxanthoma (AFX) represents an uncommon skin tumor typically occurring on sun‐damaged skin of the elderly. Histopathologic variants include spindled, clear cell, osteoid, osteoclastic, chondroid, pigmented, granular cell and myxoid lesions. To date, an atypical lymphoid infiltrate, including CD30‐positive large cells mimicking lymphomatoid papulosis, has not been described in association with AFX. Methods: The clinical and histopathological characteristics of two AFX cases inciting an atypical lymphoid infiltrate, along with immunohistochemical profiles and T‐cell receptor gamma (TCRγ) gene rearrangement results, were reviewed. Results: Lesions in both cases occurred as solitary nodules in elderly patients. Microscopically, both lesions showed a cellular proliferation composed of pleomorphic spindle cells, associated with a prominent intralesional atypical lymphoid infiltrate. The spindle cells expressed CD10 but lacked the expression of S‐100, cytokeratins and muscle markers, thereby confirming the diagnosis of AFX. CD30 highlighted a significant subset of large mononuclear cells in the lymphoid infiltrate of one case. TCRγ gene rearrangement analyses were negative for both cases. Conclusion: An atypical lymphoid infiltrate, including the one resembling lymphomatoid papulosis, associated with AFX has not been previously described. It is important to recognize the reactive nature of the infiltrate to avoid a misdiagnosis of lymphoma. Zheng R, Ma L, Bichakjian CK, Lowe L, Fullen DR. Atypical fibroxanthoma with lymphomatoid reaction.     

Mycosis fungoides progression and chronic solvent exposure

The effect of repeated exposure to specific chemicals on the initiation or progression of mycosis fungoides (MF) remains unsettled. A patient with low-grade patch stage MF progressively developed MF plaques restricted to his arms, and a tumour on his right thigh. These areas were subject to repeated exposure to solvents. His thigh was indeed in close contact with his trousers pocket where he used to store a wiping rag drenched into white spirit and cellulosic thinner. Immunophenotyping these lesions revealed a dense LCA+, CD2+, CD3+, CD4+, CD5+, CD7+, CD45+, CD45RO+ T-cell infiltrate admixed with many factor XIIIa+ dendrocytes. T-cell receptor rearrangement analysis identified a monoclonal T-cell infiltrate. An internal work-up remained negative. Stopping further solvent exposure failed to improve his condition. Oral corticotherapy combined with low-dose interferon-alpha2a halted disease progression. This observation suggests that long-term solvent exposure may trigger MF and hasten its progression from the patch stage to the plaque and tumour stages.     

CD8+ mycosis fungoides clinically masquerading as alopecia areata

A 33‐year‐old female with a 7‐year history of CD8‐positive hypopigmented mycosis fungoides (MF) involving the trunk and extremities presented with a large well‐defined alopecic patch on her frontal scalp. Clinically, this area resembled alopecia areata (AA) and was without hypopigmentation or erythema. A scalp biopsy revealed a non‐scarring inflammatory alopecia and a superficial band‐like atypical lymphoid infiltrate with prominent epidermotropism. Atypical, predominately CD8‐positive lymphocytes were seen surrounding and infiltrating the bulb portion of several hair follicles. Treatments for her MF lesions have included topical bexarotene, topical corticosteroids and phototherapy. Her alopecia has been treated with high potency topical corticosteroids and multiple intralesional triamcinolone injections with very minimal hair regrowth to date. Alopecia due to cutaneous lymphoma is an uncommon phenomenon but can occur in erythrodermic MF or Sezary syndrome. AA‐like changes have most often been reported in conventional patch/plaque stage MF and folliculotropic MF. In these cases, the atypical lymphoid infiltrate is comprised predominately of CD4‐positive lymphocytes. This is a rare report of a CD8‐positive MF causing AA‐like changes. This case highlights the importance of a scalp biopsy in patients with a history of cutaneous lymphoma presenting with alopecia in order to evaluate the nature of their hair loss.     

An Unusual Hematologic Malignancy Derived from Plasmacytoid Dendritic Cells

Recently reported cases of CD4+ CD56+ hematologic malignancies with a strong predilection for the skin correspond to the neoplastic counterpart of plasmacytoid dendritic cells. This rare, aggressive malignancy lacks pan‐myeloid and pan‐lymphoid markers and often presents with cutaneous lesions, splenomegaly, and involvement of lymph nodes and bone marrow. It has a poor prognosis, and many patients progress to acute myeloid leukemia. The proposed cellular origin is a CD56+ precursor cell related to plasmacytoid monocytes, which strongly expresses CD123 (IL‐3Ra). We describe a 70 year‐old man who presented with gray‐brown truncal nodules and plaques, lymphadenopathy, and splenomegaly. His bone marrow demonstrated malignant CD4+ CD56+ mononuclear cells. Histologic sections of skin lesions showed an atypical infiltrate extending into the deep reticular dermis. Immunohistochemical staining of these cells for CD4 was diffusely positive. Moreover, the infiltrate strongly expressed CD56 and CD123 but showed only patchy or negative staining for other T and B cell markers. The combination of the patient's clinical presentation and biopsy results best fits the diagnosis of this newly characterized, distinct clinicopathologic entity described in recent literature as agranular CD4+ CD56+ hematodermic neoplasm, plasmacytoid dendritic cell acute leukemia, and tumor‐forming accumulations of plasmacytoid monocytes associated with myeloid disorders.     

Epstein Barr Virus (EBV)‐Associated Diffuse Large B‐Cell Lymphoma (BCL) with Isolated Skin Involvement Associated with Combined Immunodeficiency and Multiple Opportunistic Infections

A 72‐year‐old man presented with nausea, vomiting, cough, dyspnea, weight loss and an ulcerated nodule on the right calf of four month's duration. CT revealed multiple bilateral pulmonary nodules. Skin biopsy demonstrated an angiodestructive infiltrate with neutrophils, CD4+ T‐lymphocytes, CD20+ large, atypical lymphocytes and histiocytes in the reticular dermis and subcutis. The atypical lymphocytes were positive for EBV by ISH. PCR demonstrated a clonal immunoglobulin gene rearrangement. Lung biopsy showed histoplasmosis without evidence of a lymphoproliferative disorder or EBV infection. Further investigation revealed a low CD8 count, IgM deficiency and candidal esophagitis. Treatment consisted of itraconazole and rituximab. The cutaneous findings in this patient resemble lymphomatoid granulomatosis (LyG), an angiocentric and angiodestructive lymphoproliferative disorder involving extra‐nodal sites. While LyG can involve multiple organs, it primarily affects the lungs. The infiltrate consists of large, atypical EBV‐positive B‐cells admixed with reactive T‐cells. Although our patient's skin pathology resembles LyG, we favor the broader diagnosis of EBV‐associated diffuse large BCL since he had an isolated skin lesion without evidence of lung or other systemic involvement. We will compare the clinical and histologic findings in our patient to cases of LyG from our institution to help classify this entity among other cutaneous lymphoproliferative disorders.     

Primary Cutaneous Natural Killer Cell Blastic Lymphoma

An 81 yo male presented with several asymptomatic firm 1–5 cm red purple plaques on the trunk and lower extremities associated with a mild pancytopenia. Histological examination revealed a diffuse, monotonous dermal infiltrate of atypical medium sized cells with fine chromatin and scanty cytoplasm. Immunoperoxidase staining demonstrated positivity for CD 45, CD43, CD4, Bcl‐2, and TdT; but was negative for cytokeratin, melan‐A, CD30 and hematopoietic lineage specific markers. A subsequent bone marrow aspirate demonstrated a dense population of cells that were morphologically consistent with blasts. Immunophenotyping by flow cytometry revealed lesional cells that expressed CD56, CD4, CD7, CD5, HLA‐DR and TdT. However, lineage specific markers for B‐cells (CD19, CD20, cCD79a, and CD10), T‐cells (sCD3 and cCD3), and myeloid cells (CD13, CD33, CD117, cCD13, CD14, CD41, CD61, myeloperoxidase, and alpha napthyl butyrate esterase) were not expressed. Molecular studies by PCR exhibited no evidence of T‐cell receptor or heavy chain gene rearrangements. Collectively, these findings are consistent with a primary cutaneous blastic natural killer cell lymphoma. Blastic natural killer cell lymphomas are characterized by a high incidence of cutaneous involvement and an aggressive clinical course. Our patient responded dramatically to one cycle of CHOP chemotherapy with resolution of his cutaneous tumors.     

CD30+ large cell transformation of mycosis fungoides after psoralen plus ultraviolet A photochemotherapy

Psoralen plus ultraviolet A (PUVA) photochemotherapy is widely used for the therapy of mycosis fungoides (MF). Clinical progression of MF is often associated with an increase in the size of tumour cells known as transformation. We report two patients with CD30+ large cell transformation that appeared after low-dose PUVA therapy for MF. Clinical data, histopathology, immunohistopathology and T-cell receptor gene rearrangement were studied. Nodules consisted of atypical large cells that expressed CD30. Monoclonal rearrangement of T-cell receptors was observed in one case. Low-dose PUVA therapy may be associated with CD30+ large cell transformation in patients with MF.     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

Cutaneous Sparganosis: A Case Report and Literature Review

A sixty‐nine‐year‐old male patient, without a significant prior medical history, presented with a rapidly enlarging, 2 cm non‐tender nodule on the right lower eyelid. The lesion persisted in spite of an incision and drainage. Following an excision, the histology revealed a diffuse infiltrate of atypical mononuclear cells within the dermis. The cells were large, monomorphic, with irregular to convoluted nuclei, prominent nucleoli, amphophilic cytoplasm. They were very mitotically active. Immunohistochemical stains for CD45, CD3, and CD30 were strongly positive. Pancytokeratin, CD20, and ALK (Anaplastic Lymphoma Kinase fusion protein) were negative. The histopathologic diagnosis was CD30 (Ki‐1) positive anaplastic large cell lymphoma. The margin was positive for involvement by lymphoma. The lesion was re‐excised with no evidence of residual involvement by lymphoma. A primary cutaneous anaplastic large cell lymphoma was favored over systemic involvement based on the clinical presentation of a single eyelid nodule and ALK negativity. He was referred to a hematology‐oncologist for further management. Anaplastic large cell lymphoma is a distinctive type of malignant lymphoma with a relatively favorable prognosis. It frequently involves the skin, however, to the best of our knowledge, presentation of this lymphoma type as a single lesion on the eyelid is extremely rare.