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Dermatology Clinic
CASE
Red Bumps on Arms and Legs
DINA ZAMIL, BS; TARA L. BRAUN, MD; CHRISTOPHER RIZK, MD
A 6-year-old girl presents with a 1-year history of a waxing and waning rash on her limbs and face. The rash starts as red scaly bumps that fade over several weeks leaving smooth white spots. Every few months, the patient gets several new red bumps. The rash is not itchy or painful. Her parents have tried topical steroid creams that did not help clear the rash. Examination reveals scattered erythematous scaly papules on both legs and several hypopigmented macules on her legs, arms, and face.
Pityriasis lichenoides (PL) describes a spectrum of acute and chronic dermatoses that includes pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC).1,2 The chronic form, PLC, is characterized by scaly red to brown papules that resolve spontaneously and recur frequently.1,2
In 1894, the chronic and acute forms of PL were first described by Jadassohn and Neisser, respectively, in separate case reports.3-5 The term pityriasis lichenoides chronica was first used in 1899 by Juliusberg.3,6 The acute form of PL was distinguished from PLC in 1916 by Mucha7 and was named pityriasis lichenoides et varioliformis acuta in 1925 by Habermann.3,7
The prevalence of PL in the general population is estimated to be 0.05%.1 It is slightly more common in men and occurs more frequently in late childhood or early adulthood, but can occur at any age.3 Pityriasis lichenoides has been reported across many ethnic groups and geographic locations with no racial predilection.1,3,8 The PLC subtype occurs more frequently than PLEVA and tends to affect children.1,3,8 One study found that Black patients were more likely to develop PLC and White patients were more likely to develop PLEVA.9
Although the exact etiology and pathogenesis of PL are not understood fully, 3 theories predominate.8 The first theory is that PL is an inflammatory reaction to an antigenic agent. Infections and drugs have been linked to cases of PL.3,8 An infectious etiology is supported by several characteristics of PL such as familial outbreaks, young age of onset, and acute eruptions. Infectious PL cases have been linked to Toxoplasma gondii, Epstein-Barr virus, and HIV.3 The second theory is that PL is a lymphoproliferative disorder. This theory is supported by reported cases of PLC progressing to mycosis fungoides.8 The third major etiologic theory states that PL is an immune-complex–mediated hypersensitivity vasculitis.3,8 Studies have found immunoglobin deposits in biopsies of PL lesions and circulating immune complexes in some patients with PL.8
PLC typically presents with gradually developing lesions on the proximal extremities and trunk. The lesions are small, erythematous or brown, and have centrally attached fine parakeratotic scales.2,8 When removed, these scales may leave a shiny brown or pink macule.8 The lesions generally do not scar but leave areas of hyper- or hypopigmentation. Patients with PLC may experience periods of remission between outbreaks.1,2
DIAGNOSIS Pityriasis Lichenoides Chronica
Dermatology Clinic
The distribution of lesions can predict the disease course; peripheral PLC lesions can take years to fade, whereas a generalized rash tends to resolve more quickly.2 Compared with PLEVA, PLC has a more indolent course and lacks epidermal necrosis. The lesions also can present concurrently with PLEVA lesions or after PLEVA lesions subside.3,8 Fever and pruritus rarely occur in PL.8 The histopathology of PL shows epidermal parakeratosis, dermal edema, necrotic keratinocytes, lichenoid in ammation, and perivascular lymphohistiocytic in ammatory in ltrate. In PLC, the lymphocytic perivascular in ltrate tends to be sparse, super cial, and dominated by CD4+ T cells.8 Rarer histologic ndings in PLC include hemorrhage, spongiosis, neutrophilic in ltrate, epidermal vesicle formation, dyskeratosis, and basilar vacuolar changes.2,8
The condition is diagnosed by clinical presentation correlated with histologic ndings.3,8 A full workup may be needed to exclude other diagnoses. Depending on clinical presentation, complete blood cell count and serologic tests for T gondii, herpes simplex virus, HIV, Epstein-Barr virus, and cytomegalovirus may be useful.8
One condition that can be confused with PLC and PLEVA is lymphomatoid papulosis.3 This disease, however, will show di erent histologic ndings than PL and, unlike PL, it is characterized by overexpression of CD30+ atypical T cells.3,8 The di erential diagnosis should also include lichen planus, guttate psoriasis, pityriasis rosea, secondary syphilis, and viral or drug exanthems.3,8 These conditions usually can be differentiated from PL via clinical and histologic ndings.3,8 Laboratory tests such as venereal disease research laboratory test, immunopathologic evaluation, antistreptolysin O titers, and throat cultures can assist in di erential diagnosis.3
Although some cases of PL spontaneously resolve over several weeks, other cases persist for years. The condition is relatively benign, but treatment for PLC may be pursued because of cosmetic concerns and for symptomatic relief.1,8 Oral antibiotics such as azithromycin, erythromycin, and tetracycline are considered rst-line treatments. Antibiotic doses must be tapered gradually to prevent recurrence. Topical corticosteroids may be used in conjunction with antibiotics to o er symptomatic relief of pruritus and in ammation.8 Phototherapy with narrowband UV-B has shown success.1,8 Severe PL cases may warrant use of methotrexate, cyclosporine, dapsone, or acitretin.8
The patient in this case had a punch biopsy of a lesion on the lower extremity that con rmed a diagnosis of PLC. She is being treated with a 3-month course of erythromycin, which she is tolerating well. ■
References
1. Jung F, Sibbald C, Bohdanowicz M, Ingram JR, Piguet V. Systematic review of the ef cacies and adverse effects of treatments for pityriasis lichenoides. Br J Dermatol. 2020;183(6):1026-1032. 2. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classi cation, and treatment. Am J Clin Dermatol. 2007;8(1):29-36. 3. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;55(4):557-572; quiz 573-576. 4. Jadassohn J. Ube rein eingenartiges psoriasiformes und lichenoides exanthem. Verh Dtsch Dermatol Ges. 1894;4:524-529. 5. Neisser A. Zur Frage der lichenoiden eruptionen. Verh Dtsch Dermatol Ges. 1894;4:495-499. 6. Juliusberg F. Uber die pityriasis lichenoides chronica (psoriasiform exanthem). Arch Dermatol Syphilol. 1899;50:359-374. 7. Mucha V. Uber einen der parakeratosis variegata (Unna) bzw. Pityriasis lichenoides chronica (Neisser-Juliusberg) nahestehenden eigentumlichen Fall. Arch Dermatol Syph. 1916;123:586-592. 8. Moy A, Sun J, Ma S, Seminario-Vidal L. Lymphomatoid papulosis and other lymphoma-like diseases. Dermatol Clin. 2019;37(4):471-482. 9. Zang JB, Coates SJ, Huang J, Vonderheid EC, Cohen BA. Pityriasis lichenoides: long-term follow-up study. Pediatr Dermatol. 2018;35(2): 213-219.
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Triple-negative breast cancer comprises 10% to 15% of breast cancers, mostly in women who are premenopausal, Black, and BRCA1 positive.
Invasive ductal carcinomas are the most common type of triple-negative cancers.
A34-year-old Hispanic woman with no signi cant medical or surgical history presents to the surgical oncology clinic with a 5-year history of feeling a lump in her left breast and skin changes that have worsened over the past 6 months.
The patient states that she detected the lump in her left breast 5 years ago. At the time, she had a mammogram that indicated dense breast tissue but was otherwise negative for suspicious ndings. She was advised to monitor the lump in her left breast and to seek medical evaluation immediately if there were any changes. Six months ago, she noticed areolar skin thickening and nipple retraction in her left breast, which prompted her to seek further evaluation.
The patient started menarche at age 13, was on oral contraceptives for approximately 2 years, and is a nonsmoker. A 12-point review of systems is negative except for the presenting symptoms.
Physical Examination
The patient is well-nourished, well-groomed, alert and oriented ×3, and appears to be in no acute distress. She is 167.64 cm (66 in) tall and weighs 73.2 kg with a BMI of 24.2. Her vital signs are unremarkable: heart rate, 70 beats per minute; blood pressure, 118/72 mmHg; temperature, 98.2 °F; respirations, 16 breaths per minute; and oxygen saturation as measured by pulse oximetry, 99% on room air.
Her breasts are examined in sitting and supine positions. The left breast is fuller and sits slightly
FIGURE 1. Left. Cranial caudal diagnostic mammogram image of the left breast indicating a 2.0-cm irregular mass located 9.0 cm away from the nipple at the 1-o’clock region that is associated with pleomorphic calcifications. Right. Lateral medial diagnostic mammogram image of the left breast indicating an irregular mass associated with pleomorphic calcifications.
FIGURE 2. Left. Long-view axis via ultrasound indicating a conglomerate of masses in the left breast at the 12-o’clock position. The masses measure approximately 5.0 cm × 3.3 cm × 1.8 cm and are located 6.0 cm away from the nipple of the left breast. Right. Blood flow to the mass in the left breast.
FIGURE 3. Left. Long-view axis via ultrasound indicating suspicious node involving the left axilla at level 1. The node measures 2.1 cm × 1.5 cm × 0.9 cm. Right. Transverse view axis via ultrasound indicating a suspicious node involving the left axilla at level 1. higher than the right. Left nipple retraction with significant periareolar skin thickening is noted. A large, 8-cm, mobile palpable mass occupies the entire upper outer quadrant and central left breast. A palpable left axillary lymph node, measuring approximately 1.5 cm, is present. There is no discrete mass palpable in the right breast, and no palpable right axillary nodes are found. Physical examination is negative for cervical, supraclavicular, and infraclavicular lymphadenopathy bilaterally. Her cardiopulmonary, musculoskeletal, and neurologic examinations are unremarkable.
Diagnostic Workup
A bilateral diagnostic mammogram is ordered instead of a screening mammogram because the patient has abnormal clinical findings. This imaging indicates a 2.0-cm irregular mass 9.0 cm away from the nipple at the 1-o’clock region associated with fine pleomorphic calcifications in the left breast (Figure 1). The total suspicious area of volume on mammography measures approximately 6.0 cm × 5.0 cm × 4.4 cm and is associated with skin thickening. A 1.0-cm oval mass with circumscribed margins and associated course calcifications is found in the subareolar region of the right breast.
A bilateral breast ultrasound indicates a conglomerate of masses measuring approximately 5.0 cm × 3.3 cm × 1.8 cm located 6.0 cm away from the nipple of the left breast (Figure 2). Several other smaller masses and areas of ductal dilatation extending from these masses to the base of the nipple are found. In total, the measurable disease on ultrasound was approximately 7.0 cm. Associated left nipple flattening and skin thickening measuring 0.5 cm are noted. On the right side, ultrasound reveals a mass measuring 1.0 cm × 0.9 cm × 0.9 cm most consistent with a fibroadenoma. A second, smaller mass, measuring 0.8 cm × 0.7 cm × 0.4 cm, also likely represents a fibroadenoma. These findings correlate to the oval circumscribed mass in the right breast on mammogram.
Four suspicious-appearing nodes involving the left axillary levels 1 and 2 are found, the largest of which measures 2.1 cm × 1.5 cm × 0.9 cm (Figure 3). No axillary level 3, internal mammary, or supraclavicular adenopathy are found.
Pathology results from an ultrasound-guided core biopsy of the left breast at the 12-o’clock position with marker clip placement indicate invasive ductal carcinoma that is histologic grade 3, estrogen receptor-negative, progesterone receptor-negative, ERBB2 (formerly HER2)-negative, and Ki67 54%, with several foci of lymph vascular invasion. An ultrasound-guided, fine-needle aspiration with clip placement of the 2.1-cm left axillary lymph node indicates metastatic adenocarcinoma consistent with primary breast carcinoma.
A bone scan of the whole body and CT of the chest, abdomen, and pelvis with contrast is performed before chemotherapy to rule out distant disease. These studies show no evidence of metastases.
Although the patient does not have a family history of breast cancer, genetic testing was indicated per National Comprehensive Cancer Network guidelines because she is younger than 60 years with triplenegative disease. Genetic testing results are negative.
Oncofertility consultation is offered because the patient is of childbearing age and chemotherapy may induce ovarian failure. The patient declined this consultation.
To address psychosocial support, the patient meets with a social worker and is referred to support groups during her treatment. She also has a nurse navigator to educate her on the various teams involved in her care and what to expect at each appointment. The nurse navigator remains available as a resource for the patient during and after treatment.
Treatment Course
Treatment options such as chemotherapy, radiation, and surgical intervention are discussed with the patient. Based on her clinical stage of cT3cN1cM0 and triplenegative profile, chemotherapy is initiated before surgical intervention. Port placement is recommended for the patient before treatment with doxorubicin and cyclophosphamide for 4 cycles every 3 weeks followed by paclitaxel for 4 cycles, with 3 weekly treatments per cycle. The following laboratory evaluations are performed before each cycle of chemotherapy treatment: complete blood cell count with differential, blood urea nitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase, and total bilirubin.
A cold cap is ordered for the patient to decrease risk for hair loss. Cold caps are recommended to wear approximately 1 hour before the infusion, during the infusion, and at least 3 hours after the infusion for each treatment cycle. Ondansetron 8 mg, prochlorperazine 10 mg, and dexamethasone 4 mg as needed are prescribed to manage nausea during treatment.
Approximately 4 weeks after completion of chemotherapy, the patient undergoes a left modified radical mastectomy to remove the entire left breast and level 1 and 2 axillary nodes in the same surgery as 2 separate specimens. Magnetic seed localized excision is performed as part of the complete axillary dissection to ensure removal of the biopsyproven and clipped left axillary lymph node.
The pathology results from the left breast mastectomy show rare atypical cells consistent with focal residual carcinoma cells in the background of extensive treatment effects. No lymphovascular invasion is identified. Usual ductal hyperplasia, fibroadenomatous change, stromal fibrosis, and focal microcalcifications are noted. The margins are clear, with distance from closest margin greater than 10 mm anteriorly and superiorly (Figure 4).
Pathology of the left axillary lymph node with 1 magnetic seed indicates metastatic carcinoma with a metastatic focus of 3 mm without extranodal extension. Level 1 left axillary dissection contents indicate metastatic carcinoma with treatment effects in 2 of 21 lymph nodes, with the largest metastatic focus measuring 1.8 mm without extranodal extension. Left axillary dissection of level 2 nodes indicates metastatic carcinoma in 1 of 3 lymph nodes, with metastatic focus measuring 0.7 mm without extranodal extension.
Postmastectomy radiation is recommended for this patient to decrease the risk for disease recurrence in the setting of
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FIGURE 4. Pathology specimen obtained after a total left breast mastectomy showing clear margins, with distance from closest margin greater than 10 mm on the superior and anterior sides. All clips are located within the specimen.
M49737_PP-US-REL-CT-2100114_Myfembree_BriefSummary_BW_CommonA_size_Mv04_ClinicalAdvisor_LEFT_PG5 in the United States in 2021.1 Triple-negative breast cancer Triple-Negative Invasive Ductal Carcinoma 49737 n/a dnGiant Creative Strategy continued from page 29 has an incidence of 13.2 per 100,000 women and accounts 09/07/21 for approximately 10% to 15% of all breast cancers.1,2 This PMSxxxx PMSxxxx PMSxxxx PMSxxxx 9:45 am advanced node-positive disease. The patient starts physi- subtype is more common in patients who are premenopausal, cal therapy on postoperative day 14 to improve her range Black, and have BRCA1 gene mutations.2,3 Invasive ductal of motion, which decreased because of surgery, and allow carcinoma is the most common phenotype of triple-negative her to initiate radiation therapy within 4 to 6 weeks. The breast cancer, accounting for 14.6% of patients with triplepatient is scheduled for simulation to prepare for radiation negative disease.2 treatment of 50 gray in 25 fractions to the chest wall and Treatment decisions in this case were based on NCCN regional lymphatics followed by 10 gray boosts to the chest and MD Anderson Cancer Center guidelines.4,5 The first wall. The patient is asked to stop taking vitamin C during step was to determine whether the patient had invasive vs radiation treatment because that can decrease the effectiveness noninvasive cancer. In this case, because the patient had of radiation therapy. It is recommended that the patient start cancer cells present in lymph nodes and invasive disease on capecitabine for 6 cycles during radiation therapy to further left breast biopsy, the invasive protocol was followed. Based reduce her recurrence risk. on the treatment guidelines for stage III, triple-negative disease, it was recommended that the patient consider neoDiscussion adjuvant chemotherapy followed by total mastectomy with Triple-negative breast cancer is an aggressive type of breast axillary lymph node surgery. This differs from the protocol cancer that is negative for estrogen, progesterone, and ERBB2 for earlier stage, hormone-positive tumors, for which neoadreceptors, making targeted therapy difficult. An estimated juvant chemotherapy is not considered. Because the patient 281,550 new cases of female breast cancer and an estimated had stage III disease with 4 or more involved lymph nodes, 43,600 deaths because of female breast cancer are expected her treatment team also recommended postmastectomy
