patchy alopecia following chemotherapy
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inflammation, such as an elevated erythrocyte sed-imentation rate, elevated levels of C-reactive protein,thrombocytosis, and mild normocytic or microcyticanemia.
The treatment of DCS is varied and consists of bothmedical and surgical options. The disease is oftenrecalcitrant to medical therapies. Agents reportedto control the inflammatory process in some patientswith DCS include: topical and oral isotretinoin,intralesional corticosteroids, antibiotic soaps(chlorohexidine and benzoyl peroxide), dapsone,infliximab, adalimumab, zinc supplements, oralantibiotics, and oral corticosteroids. Combinationtherapy, for example with oral antiinflammatoryantibiotics (eg, tetracyclines) and intralesional corti-costeroids is often more successful than monother-apy, and may be combined with other systemic andtopical agents. Procedural treatments, such as inci-sion and drainage with marsupialization of intercom-municating sinuses, ablative and epilating lasertreatments, grafting, and total scalp excision followedby skin grafting and epilating radiation therapy areapproaches that may be employed when medicaltherapy fails; however, aggressive procedural modal-ities typically lead to permanent alopecia and somedegree of scarring. The prognosis for completerecovery from DCS is poor.
For this series, the recommended choices are: 1, c;2, d; 3, d; 4, a; 5, e; and 6, a.
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Patchy alopecia following chemotherapy
Joslyn S. Kirby, MD,a Mahsa Tehrani, MS,b andMichael D. Ioffreda, MDa
Hershey, Pennsylvania,a and Washington, DCb
A 57-year-old white female presented with small,scattered patches of alopecia. She had a history of
metastatic ductal breast cancer, and 2 months beforeher visit had finished three cycles of adriamycin andcyclophosphamide followed by three cycles of doce-taxol and cyclophosphamide. Her current medica-tions included anastrazole and omeprazole. Shedenied any areas of alopecia before treatment, andshe lost all of her hair during chemotherapy. Follow-ing chemotherapy, she noticed that some areas of herscalp remained bald. She denied pruritus, pain, andflaking. She had not attempted to treat the condition.She denied any personal or family history of autoim-mune disorders. Her review of systems was negative.A physical examination revealed many scattered, 1- to2-cm, white, atrophic patches of alopecia (Fig 2).A biopsy specimen was taken and anticytokeratin(CAM 5.2) staining was performed (Figs 3 and 4).
7. What is the most likely diagnosis?
a. Pseudopelade of Brocqb. Alopecia neoplasticac. Trichotillosisd. Basal cell carcinoma, infiltrative typee. Morphea8. Which of these conditions can clinically mimicthis disease?
a. Alopecia areatab. Discoid lupus erythematosusc. Lichen planopilarisd. Alopecia mucinosae. All of the above9. What is the most common malignancy associ-ated with this disease?
a. Lung carcinomab. Ovarian carcinomac. Gastric adenocarcinomad. Breast adenocarcinomae. Prostate adenocarcinoma10. Which of the following may cause destructionof the follicle?
a. Infiltration of the follicular sheath by neo-plastic cellsb. Fibroplasia of the dermisc. Effects by cytokines, such as interleukin-4,
fibroblast growth factor, and transforminggrowth factor-beta
d. Mass effect by the malignant cellse. All of the above
11. Which of the following statements is true?
a. Cutaneous metastasis, including alopecianeoplastica, does not impact prognosis.b. Hair loss is always permanent.c. Cutaneous metastases most often precede
diagnosis of malignancy.
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d. Treatment of the hair loss is primarily treat-ment of the underlying malignancy.
e. Cutaneous metastases occur in about 12% ofcancer patients.
DiscussionCutaneous metastases from an underlying malig-
nancy are uncommon, and alopecia neoplastica(AN) is a rare form of cutaneous metastasis. AN ismost commonly defined as alopecia caused by thecutaneous infiltration of malignant cells from a dis-tant malignancy. A metaanalysis estimated the prev-alence of cutaneous metastasis to be about 5%, whileindividual estimates range from 0.7% to 9%.
AN can have a variable clinical appearance. It canpresent as single or multiple alopecic patches,plaques, or nodules, and has been reported to mimicalopecia areata, discoid lupus, and pseudopelade ofBrocq. The differential diagnosis also includes tineacapitis, lichen planopilaris, and alopecia mucinosa.AN is most commonly associated with breast cancer.
It has also been associated with cervical cancer,gastric and colonic adenocarcinoma, melanoma,lymphoma, extramammary Paget disease, and otherconditions.
A scalp biopsy specimen often reveals relativelysparse neoplastic cells in a highly collagenous stromaand partial to complete disappearance of the pilose-baceousunits. Thepathophysiologyof thedestructionof the pilosebaceous unit is not understood. It may becaused by fibroplasia induced by the neoplastic cells.Histologic evaluation of our patient revealed thepartial to complete absence of hair follicles, follicularscars, absence of sebaceous glands, and the presenceof strands of single-file (‘‘Indian-filing’’) basophiliccuboidal cells (Fig 3). There was infiltration of thesecells into the follicular tracts, and the cells demon-strated signet ring forms and intracytoplasmic vacu-oles. In addition, the abnormal cells stained positivelywith CAM 5.2 (Fig 4), which is an immunostain for lowmolecular weight keratins. These findings are consis-tent with AN caused by metastatic breast cancer. CAM5.2 stain reacts with secretory epithelia, neuroendo-crine tumors, and some sarcomas, such as renal cellcarcinoma, extramammary and mammary Paget dis-ease, Merkel cell carcinoma, and epithelioid sarcoma,but not normal stratified squamous epithelium ormelanoma.
The pathophysiology of AN is not definitivelyunderstood. Theories include destruction by directinfiltration by of the hair sheath by the neoplasticcells, fibroplasias caused by or in response to theneoplastic cells, and injury by cytokines, such asinterleukin-4, transforming growth factor-beta, andfibroblast growth factor.
Treatment is aimed at the underlying malignancy.Archer and Smith reported a patient with AN causedby breast cancer that regrew some hair duringtreatment with tamoxifen. Cutaneous metastasesand AN often portend a poor prognosis and occurin patients with a known malignancy. There are rareinstances of AN leading to the diagnosis of the
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underlying malignancy. However, scalp metastasestypically develop years after the primary malignancyis diagnosed. The case discussed here continues tobe closely followed by an oncologist. Her metastaticdisease is stable.
For this series, the recommended choices are: 7, b;8, e; 9, d; 10, e; and 11, d.
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Linear scalp plaques
Mario L. Pinoli, MS,a Nathan W. Hanson, MD,b
Michael J. Wells, MD,b and Cloyce L. Stetson, MDb
El Pasoa and Lubbock,b Texas
An 88-year-old white man presented to our depart-ment for the evaluation of multiple scalp lesions ofrelatively recent onset. There was no pertinent familyhistory. He denied trauma to the area, ever havingbeendiagnosedwithan infectionof theareacausedbyherpes simplex virus (HSV) or varicella zoster virus(VZV), and ever having received radiation therapy.The physical examination revealed multiple indu-rated, red, and slightly violaceous plaques and nod-ules on the scalp in a strikingly linear pattern. Thecollectionof tumors covered a 9 cm 3 3 cmareaon theright forehead, frontal scalp, and parietal scalp (Fig 5).There was no clinical lymphadenopathy. Review by adermatopathologist yielded a diagnosis of malignantepithelioid neoplasm, and additional special stains
were performed. Immunohistochemical stainingshowed strong staining with CD31 and vimentin andnegative staining with CD68, S-100 protein, HMB45,CK7, CK20, CD43, pancytokeratin, CK5/6, CD20,CD34, chromogranin, synaptophysin, mucicarmine,and carcinoembryonic antigen stains.
12. Based on the patient’s presentation and patho-logic results, what is the next step?
a. Karyotype biopsy specimenb. Referral to the departments of surgery andradiation oncologyc. Positron emission tomography scan of the
chestd. Computed tomography scan of the abdomene. Bone marrow biopsy
13. What is the most likely diagnosis?
a. Metastatic melanomab. Spindle cell neoplasm with neuroendocrinedifferentiationc. Clear cell sarcomad. Angiosarcomae. Malignant peripheral nerve sheath tumor
14. Which of the following has NOT been anassociated contributing factor to this diagnosis?
a. Secondary VZV infectionb. Long-standing lymphedemac. Actinic keratosesd. Irradiatione. Trauma15. Which of the following cell types do NOT stainpositively with CD31?
a. Melanocytesb. Plateletsc. Embryonic stem cellsd. Endothelial cellse. NeutrophilsThe diagnosis was angiosarcoma. The patient wasreferred to the departments of surgery and radiationoncology. The treatment team decided he was a poorsurgical candidate because of his coexisting medical