Dermal and pannicular manifestations of internal malignancy.

The concept that noncutaneous malignancies may induce paraneoplastic inflammatory reactions and neoplastic or non-neoplastic proliferations in the skin is well known. Previous work on this subject primarily provides lists and descriptions of dermatologic entities that are exclusively or occasionally associated with specific or varied internal cancers or precancerous states. This review seeks to provide a different perspective to this subject by emphasizing components of the skin (the dermis and subcutis) as focal points of paraneoplastic phenomena, with the intent of broadening thinking and differential diagnoses when the findings described are encountered in dermatology clinics and dermatopathology laboratories.

Increased metastasis and mortality from cutaneous squamous cell carcinoma in patients with chronic lymphocytic leukemia.

In patients with chronic lymphocytic leukemia, squamous cell carcinoma behaves aggressively. Our purpose was to compare squamous cell carcinoma metastasis and mortality between patients with chronic lymphocytic leukemia and control subjects. Medical records were assessed retrospectively for 28 patients with chronic lymphocytic leukemia who underwent surgical excision of cutaneous squamous cell carcinoma and for 56 matched control subjects. The rate of metastasis and mortality from cutaneous squamous cell carcinoma were determined on a per-patient basis. Three of 28 patients with chronic lymphocytic leukemia had metastasis and died of metastatic disease. No metastases or deaths occurred among the 56 control subjects. Compared with control subjects, chronic lymphocytic leukemia patients with cutaneous squamous cell carcinoma were more likely to have metastasis (P = .0031) and die of metastasis (P = .0033). No significant association was detected between metastasis and history of chemotherapy administration for chronic lymphocytic leukemia. Among patients with chronic lymphocytic leukemia, surveillance for skin cancer and a decreased threshold for biopsy of suspect lesions are warranted.

Surgical revision.

Even the best surgeons must confront scars and complications that are aesthetically unacceptable and need revision. With careful assessment of the scar, various scar revision techniques can be applied to create an aesthetically pleasing result.

High recurrence rates of Basal cell carcinoma after mohs surgery in patients with chronic lymphocytic leukemia.

OBJECTIVES: To estimate and compare the recurrence rates of basal cell carcinoma (BCC) after Mohs surgery in patients with chronic lymphocytic leukemia (CLL) and controls and to evaluate differences among histologic subtypes of BCC. DESIGN: Retrospective assessment of clinical histories, postoperative notes, and surgical photographs. SETTING: Tertiary-care institution (Mayo Clinic, Rochester, Minn). PATIENTS: Twenty-four patients with CLL who underwent Mohs surgery for 33 BCCs and 66 controls matched for sex, age, and surgical year who underwent Mohs surgery for BCC of the head and neck from May 1988 through September 1998. RESULTS: Among the 24 patients with CLL who underwent Mohs surgery for 33 BCCs, there were 4 recurrences. The cumulative incidence of recurrence on a per-tumor basis was 3% at 1 year, 12% at 3 years, and 22% at 5 years. Basal cell carcinoma was 14 times more likely to recur in patients with CLL than in controls (P =.02). Overall, there were no significant differences between patients with CLL and controls in preoperative tumor size (median, 1.6 cm vs 1.4 cm; P =.18) and proportion of aggressive histologic subtypes of BCC (58% vs 41%; P =.12). CONCLUSIONS: Recurrence rates of BCC are significantly higher after Mohs surgery in patients with CLL. Overall, patients with CLL do not appear to have significantly larger BCCs or more aggressive histologic subtypes of BCC. In patients with CLL, close surveillance is warranted for recurrence of BCC and a decreased threshold is indicated for subsequent biopsies.

Association of porphyria cutanea tarda with hereditary hemochromatosis.

BACKGROUND: An increased frequency of hereditary hemochromatosis gene mutations occurs in patients with porphyria cutanea tarda. Polymerase chain reaction analysis of peripheral blood for hemochromatosis gene (HFE) mutations is available for clinical use. Early detection and treatment of hereditary hemochromatosis limit disease progression and improve life expectancy. OBJECTIVE: We present 8 patients with porphyria cutanea tarda subsequently found to have hereditary hemochromatosis or mutations in the HFE gene. METHODS: Retrospective review of patients in whom both porphyria cutanea tarda and hereditary hemochromatosis or HFE gene mutations were diagnosed between 1976 and 2000. RESULTS: Eight patients with porphyria cutanea tarda (6 males, 2 females; age range, 4-60 years; mean age at diagnosis of porphyria cutanea tarda, 42 years) were subsequently found to have hepatic iron overload or HFE gene mutations. Two patients had liver biopsy findings compatible with homozygous hereditary hemochromatosis. In the other 6 patients, HFE gene analysis revealed 3 homozygous C282Y, 1 compound heterozygous C282Y/H63D, and 2 heterozygous C282Y mutations. Seven patients (88%) had no specific signs or symptoms of hereditary hemochromatosis at diagnosis. In 5 patients (63%), the diagnosis of hereditary hemochromatosis or HFE gene mutation was initially suspected by the dermatologist. CONCLUSION: Porphyria cutanea tarda can be an important cutaneous marker for patients with mutations of the HFE gene. HFE gene analysis should be done in patients who present with porphyria cutanea tarda. The dermatologist may play a key role in the early diagnosis of subclinical hereditary hemochromatosis in patients who present with porphyria cutanea tarda.

Sentinel lymph node biopsy.

The introduction of sentinel lymph node biopsy (SLNB) has been an important development in the management of malignant melanoma. Lymph nodes have long been known to play a key role in melanoma metastasis. The importance of nodal staging accounted for the previous surgical practice of elective lymph node dissection (ELND) even with its controversial impact on final outcomes and associated morbidity. Although this morbidity has been reduced with the ability to identify the SLN, numerous questions have subsequently surfaced with respect to this procedure's utility and therapeutic efficacy. This chapter will focus on the indications for SLNB, as well as the current controversies surrounding this procedure.

Fusiform elliptical Burow's graft: a simple and practical esthetic approach for nasal tip reconstruction.

BACKGROUND: Reconstruction of cutaneous nasal tip and supratip defects presents a common challenge in which esthetic outcomes are dependent on appropriate flap or graft selection and execution. OBSERVATION: We describe the fusiform elliptical Burow's graft, a modified full-thickness skin graft, for repair of moderately sized nasal tip and supratip defects. It has the advantage of being a one-stage procedure with incisions and undermining similar to a primary closure. This technique allows the defect to become smaller in size and shallower in depth while taking the shape of a fusiform ellipse confined to a single cosmetic subunit. The color and texture match of the adjacent Burow's skin graft combined with its fusiform elliptical shape allow it to blend in more naturally with the nasal tip contour compared with the traditional circular-shaped Burow's graft, which is typically used on the nose. CONCLUSION: The fusiform elliptical Burow's graft is a simple, reliable, easily reproducible, and esthetically pleasing technique for repair of defects on the nasal tip and supratip.

Acne fulminans.

Acne fulminans is a syndrome of fulminant, necrotizing acne associated with bone lesions, constitutional symptoms, and laboratory abnormalities. A case report of an adolescent male with acne fulminans following withdrawal of isotretinoin and prednisone is presented.

High recurrence rates of squamous cell carcinoma after Mohs' surgery in patients with chronic lymphocytic leukemia.

BACKGROUND: Cutaneous cancers exhibit a much higher incidence in patients with chronic lymphocytic leukemia than in nonleukemic patients. Squamous and basal cell carcinomas also exhibit greater subclinical tumor extension in patients with chronic lymphocytic leukemia. OBJECTIVE: The purpose of this study was to estimate and compare the recurrence rates of squamous cell carcinoma after Mohs' surgery in patients with chronic lymphocytic leukemia compared with those in controls and to evaluate differences among squamous cell carcinoma size and histologic grade. METHODS: We retrospectively assessed the clinical histories, postoperative notes, and surgical photographs of patients with chronic lymphocytic leukemia and controls matched (2:1) for age, sex, and surgical year. Both patients and controls underwent Mohs' surgery for squamous cell carcinoma of the head and neck at the Mayo Clinic between March 1988 and April 1999. RESULTS: Twenty-eight patients who underwent Mohs' surgery for 57 squamous cell carcinomas had 7 recurrences. The cumulative incidence of recurrence on a per-tumor basis was 4.3% at 1 year, 14.8% at 3 years, and 19.0% at 5 years. Squamous cell carcinoma was seven times more likely to recur in patients with chronic lymphocytic leukemia than in controls (p = .003). The distribution of tumor histologic grade was not significantly different between patients and controls (p = .39). Maximum preoperative tumor diameters were clinically similar between patients and controls (median 15 mm vs 14 mm; p = .04). CONCLUSION: The recurrence rates of squamous cell carcinoma were significantly higher in patients with chronic lymphocytic leukemia. Squamous cell carcinomas in patients with chronic lymphocytic leukemia did not exhibit a significant difference in histologic grade or clinical difference in preoperative tumor size. Close surveillance for squamous cell carcinoma recurrence is warranted in patients with chronic lymphocytic leukemia.

Cryptococcal infection in sarcoidosis.

A 48-year-old man with a history of sarcoidosis was transferred to the Mayo Clinic for evaluation and management of progressive neurologic decline. Two years before admission, he was admitted to a local hospital with mental status changes accompanied by ataxia and severe headache. A diagnosis of pulmonary and central nervous system sarcoidosis was made based on computed tomography of the head, lumbar puncture, and chest radiography. A mediastinoscopy with lymph node biopsy exhibited noncaseating granulomas and negative stains for microorganisms. Prednisone therapy was initiated at 80 mg/day. Clinical improvement was apparent for 13 months during steroid therapy until the slow taper reached a dosage of 20 mg/day. At that time, the patient was readmitted to the local hospital with severe confusion and skin lesions. When intravenous methylprednisolone therapy for presumed central nervous system sarcoidosis did not improve the patient's mental status, he was transferred to the Mayo Clinic. Physical examination of the thighs revealed large, well-marginated, indurated, irregularly bordered, violaceous plaques and rare, umbilicated, satellite papules with central hemorrhagic crusts (Fig. 1A). Superficially ulcerated plaques with a similar appearance to the thigh lesions were coalescing around the lower legs (Fig. 1B). A skin biopsy specimen of the thigh demonstrated abundant numbers of encapsulated organisms and minimal inflammatory response (Fig. 2). Skin, blood, and cerebrospinal fluid cultures confirmed the presence of Cryptococcus neoformans. Amphotericin and flucytosine combination therapy was initiated, and steroid dosages were gradually tapered. A test for human immunodeficiency virus was negative. The patient was dismissed from hospital after a complicated 2-month course resulting in improved mental status but progression of the lower extremity ulcerations as a result of polymicrobial infection.

Disseminated gonococcemia.

A 26-year-old woman presented with a high-grade fever and chills of 2 days' duration. She complained of associated joint pain, especially in the wrists and knees. One day before admission, tender skin lesions began to develop on the fingers, and subsequently spread to the more proximal extremities. The patient recalled having a sore throat and a nonproductive cough before the onset of the fever and eruption. The past medical history was significant for Gardnerella vaginitis and several urinary tract infections. The patient was taking oral contraceptive pills; her most recent menstruation was 3 weeks before admission. She reported having sexual intercourse with her boyfriend 2 weeks before admission. The patient's temperature was 40 degrees C. Dermatologic examination revealed a 6-mm, hemorrhagic pustule on an ill-defined pink base, overlying the volar aspect of the left second proximal interphalangeal joint (Fig. 1a). Scattered on the upper and lower extremities were occasional round, ill-defined pink macules with central pinpoint vesiculation (Fig. 1b). A skin biopsy of the digit revealed a dense neutrophilic infiltrate with leukocytoclasis and marked fibrin deposition in the superficial and deep dermal vessels (Fig. 2a). Gram stains demonstrated the presence of Gram-negative diplococci (Fig. 2b). Laboratory findings included leukocytosis (leukocyte count of 20 x 109/L, with 81% neutrophils). Analysis of an endocervical specimen by polymerase chain reaction was positive for Neisseria gonorrhoeae and negative for Chlamydia trachomatis. Throat and blood cultures grew N. gonorrhoeae. Specimen cultures obtained by skin biopsy yielded no growth. Results of serologic analysis for human immunodeficiency virus, hepatitis, syphilis, and pregnancy were negative. Beginning on admission, intravenous ceftriaxone, 2 g, was administered every 24 h for 6 days, followed by oral cefixime, 400 mg twice daily for 4 days. Oral azithromycin, 1 g, was administered to treat possible coinfection with C. trachomatis. By treatment day 4, the patient was afebrile, with the resolution of leukocytosis and symptomatic improvement of arthralgias.

A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma.

BACKGROUND: Merkel cell carcinoma is an aggressive cutaneous neoplasm with a high propensity to metastasize to lymph nodes. OBJECTIVE: The objective of this study was to determine the prognostic significance of sentinel lymph node status in patients with Merkel cell carcinoma. METHODS: A meta-analysis of case series of patients with Merkel cell carcinoma managed with sentinel lymph node biopsy was performed. RESULTS: Forty of 60 patients (67%) had a biopsy-negative sentinel lymph node; 97% of this group had no recurrence at 7.3 months median follow-up. Twenty patients (33%) had a biopsy-positive sentinel lymph node; 33% of this group experienced local, regional, or systemic recurrence at 12 months median follow-up. Risk of recurrence or metastasis was 19-fold greater in biopsy-positive patients (odds ratio, 18.9; p = 0.005). None of 15 biopsy-positive patients who underwent therapeutic lymph node dissection experienced a regional recurrence; 3 of 4 who did not receive therapeutic lymphadenectomy experienced regional recurrence. CONCLUSION: Sentinel lymph node positivity is strongly predictive of a high short-term risk of recurrence or metastasis in patients with Merkel cell carcinoma. Therapeutic lymph node dissection appears effective in preventing short-term regional nodal recurrence. Aggressive adjuvant treatment should be considered for patients with positive sentinel lymph nodes.

Lymphocytic infiltrates and subclinical epithelial tumor extension in patients with chronic leukemia and solid-organ transplantation.

BACKGROUND: Dense infiltrates in association with squamous cell carcinoma (SCC) or basal cell carcinoma (BCC) in patients with underlying chronic lymphocytic leukemia (CLL) may complicate pathologic interpretation of histologic margins. OBJECTIVE: The study was conducted to determine the frequency of identifying dense inflammatory infiltrates in frozen histologic sections during Mohs operation for BCC or SCC in patients with CLL and organ-transplant recipients, to characterize the infiltrate (reactive versus leukemic) in CLL, and to estimate the subclinical tumor extension in patients with CLL, transplant recipients, and control subjects undergoing Mohs procedure. METHODS: Frozen sections of head and neck BCC and SCC obtained during Mohs procedures in patients with CLL, organ transplant recipients, and a control group were reviewed retrospectively. Biopsy specimens of CLL with dense infiltrates were assessed with immunohistochemical stains. Subclinical tumor extension (postoperative defect size minus preoperative tumor size) was evaluated in each group. RESULTS: Dense infiltrates were found in tumors of 20 of 55 patients with CLL (36%), 1 of 8 transplant recipients (13%), and 1 of 105 controls (1%). In patients with CLL, 75% of the dense infiltrates were B-cell leukemic. Compared with controls, the mean subclinical tumor extension was larger in patients with CLL (P=0.029) and in transplant recipients (P=0.55). CONCLUSION: Dense leukemic infiltrates associated with BCC or SCC in CLL may complicate pathologic interpretation of Mohs surgical histologic margins and may be associated with larger postoperative defects relative to preoperative clinical tumor appearance. In patients with CLL, as in transplant recipients, SCC seems more likely to develop than BCC.