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A case of nevus sebaceus associated with an unusual epithelial proliferation demonstrating apocrine and sebaceous differentiation and not conforming to known cutaneous or skin adnexal neoplasms is reported.
A 74-year-old man presented with a tumor on his scalp that he had since birth. The authors observed a verrucous pink crusted plaque. The tumor was clinically diagnosed as nevus sebaceus and was removed. Histopathologic study showed, arising from nevus sebaceus, a lobular neoplasm with the classic features of trichilemmoma. Desmoplasia was present in the center of the trichilemmomatous area. This description is typical of a special, uncommon type of trichilemmoma that histologically mimics invasive carcinoma.
A 51-year-old white woman presented with thickening of the scalp located at the vertex and left lateral occiput without hair abnormalities or alopecia. Skin biopsies of the thickened scalp showed thickening of the subcutaneous tissue with proliferation of mature subcutaneous fat cells but no signs of inflammation or hair abnormalities. During 2.5 years of follow-up, scalp thickening progressed over the entire hair-bearing scalp and persisted without signs of further progression at 3.5 year follow-up. Lipedematous scalp is an extremely rare diagnosis. It is defined by a thickening of the subcutaneous layer of the scalp and can be distinguished from lipedematous alopecia, in which subcutaneous thickening is associated with diffuse alopecia and shortening of scalp hairs. A total of seven cases of lipedematous alopecia and two cases of lipedematous scalp have been reported. We report the third case of lipedematous scalp in a 51-year-old white woman associated with early symptoms of meningitis. Additional features described in the literature include pruritus, pain, and paresthesia of the scalp as well as associated medical problems such as hyperelasticity of skin and laxity of joints, renal failure, and diabetes mellitus. This sporadic disorder is predominantly located at the vertex and occiput. The etiology and pathogenesis of lipedematous scalp and alopecia remain unclear. The treatment is symptomatic.
Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells found most commonly in bone, lungs, mucocutaneous structures, and endocrine organs. Cutaneous disease occurs in approximately one quarter of all cases. Cutaneous findings include soft-tissue swelling, eczematous changes, a seborrheic dermatitis-like appearance, and ulceration. We report a rare case of LCH confined to the scalp with folliculocentric infiltrates. This 32-year-old male patient presented with follicularly based erythema, scale, and pustules unresponsive to topicals and oral antibiotics. The patient's lesions mimicked lichen planopilaris and folliculitis decalvans during the disease process. On hematoxylin and eosin stain, scalp biopsy showed a perivascular interstitial patchy lichenoid mononuclear cell infiltrate that focally abutted follicular infundibula. Prominent mononuclear cells having reniform nuclei were present, and immunoperoxidase stains for CD1a confirmed Langerhans cell differentiation. Serological and imaging workup failed to display systemic involvement.
Histiocytic proliferations can mimic melanocytic tumors and vice versa. The authors describe the clinical, histologic, and immunohistochemical findings of three predominantly mononuclear xanthogranulomas that were misdiagnosed as malignant melanoma by experienced pathologists. All lesions occurred in male patients ranging in age from 14 to 75 years. The tumors presented as dermal nodules, two of which were surrounded by an epidermal collarette and were ulcerated focally. The tumors were composed of a mixed population of large epithelioid and plump spindle cells with pink or pale cytoplasm arranged in nests and short fascicles. Occasional mononuclear cells had cytoplasmic vacuolar changes, but none had well-developed foamy cytoplasm. Rare, multinucleated giant cells were present, but they were not of the Touton type. Mitotic figures were found in all lesions. Immunohistochemically, most tumor cells (80%-90%) were strongly positive for CD68 and a minority of cells (10%-15%), located typically at the periphery of the tumor, was positive for factor XIIIa. Two tumors contained rare cells positive for S-100 protein (5% of tumor cells or less). All tumors were completely negative for tyrosinase (T311), gp100 (HMB-45), and Melan-A (A103). Giant and foam cell-poor variants of juvenile xanthogranuloma have been reported previously, mainly in young children. Their occurrence in adolescents and adults is underrecognized. Knowledge of this variant is important to avoid misdiagnosing a benign tumor as malignant melanoma.
Gigantic cutaneous horns, grossly similar to the horns seen in animals, are exceedingly rare in humans. After finding one case in practice, we searched our departmental files for similar cases and examined them grossly and microscopically. Four cases were identified. All occurred as solitary lesions in older women on the parietal-occipital region of the scalp. They had a growth history of up to 30 years; the women hid these horns in their hair. Grossly, the horns were yellow-grey, and there were shallow furrows running along the length of the horns. The length ranged from 17 to 25 cm, and the width was up to 2.5 cm. All four lesions showed similar histologic changes. Microscopically, the gigantic horns consisted of a mixture of squamous epithelial cells and tricholemmal keratinized debris. In one case the base of the horn was directly connected with a mass composed of benign tricholemmal cysts of the scalp. Mitoses were common, but atypical mitoses were not observed. The nuclei of the squamous cells were bland without pleomorphism, hyperchromasia, or atypia. Follow-up of all patients was uneventful: all patients were well and without signs of recurrence or metastasis 2-15 years after the surgical excision. Gigantic cutaneous horns are rare and benign. We think that they represent an extremely well-differentiated variant of proliferating tricholemmal tumor with an unusual and remarkable gross pattern.
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The pathophysiology and treatment of hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp, which constitute the follicular occlusion triad, are reviewed. The unusual occurrence of all three components in a single patient resistant to medical management is presented with a review of the literature. This patient's course was complicated by multiple synchronous squamous cell carcinomas developing in a localized area where his acne conglobata was most pronounced. Although one lesion was clinically obvious, the majority were less suspicious for cancer. Occult cancer should be considered in recalcitrant cases of acne conglobata where isolated areas fail to respond to medical management, particularly when 13-cis-retinoic acid has been used.
This report describes a severe case of perifolliculitis capitis with the rare complication of skull osteomyelitis, a combination not previously reported. The patient demonstrated the typical features of perifolliculitis capitis, namely, rubbery, hard, elevated nodules involving almost the entire scalp with multiple deep-seated abscesses. Bone was exposed in two areas and the diagnosis of skull osteomyelitis was confirmed by skull x-ray films and bone biopsy specimens, which grew Staphylococcus aureus. The patient was treated with enteral hyperalimentation, intravenous antibiotics, and multiple debridements of the scalp, including the involved periosteum and outer cortex of the exposed bone, followed by reconstruction with split-thickness skin grafts. A complete eradication of the disease with closure of all open wounds and clearance of osteomyelitis of the skull was achieved.
Chronic subgaleal abscesses have been extremely rare since the advent of antimicrobial therapy. The majority of reported cases have occurred as acute infections following traumatic scalp lacerations or needle electrode insertion for fetal monitoring. The rich blood supply of the head makes widespread infection from a scalp surgical wound a very unlikely occurrence. Most acute infections of the scalp result in complete resolution with adequate early treatment. However, extensive purulent fibrosis of the scalp remains a potentially serious surgical complication. We report 2 cases of chronic necrotizing abscess of the scalp associated with a postsurgical scalp ulcer. The inflammatory process caused extensive necrotizing fibrosis (up to 2.5 cm thick) of the entire undersurface of the scalp and involved both the galea aponeurotica and the periosteum. We discuss the unique pathological features of this entity along with recommendations for its operative management and suggestions for flap design.
This review represents the largest reported series involving reconstruction of complex scalp and calvarial defects with rectus abdominis free flaps. Sixteen patients presented with extensive (up to 300 cm2) scalp and calvarial defects requiring free tissue transfer for closure. All of the 11 patients who underwent a rectus abdominis free flap had a technically successful microvascular transfer. The defects encountered involved a wide spectrum of complexities including extensive multilaminar defects with exposed brain and dura, irradiated fields, and infection. In our institutions, the rectus abdominis muscle has evolved as a uniquely superior donor choice for restoring extensive scalp defects for several reasons: (1) accessibility, which eliminates intraoperative patient repositioning and allows for a simultaneous two-team approach; (2) minimal donor-site morbidity; (3) vascular reliability; and (4) the ability to supply abundant, easily contoured tissue.
Marjolin's ulcer is an uncommon condition in which malignant transformation occurs in a chronic inflammatory focus. It was originally described in association with a thermal injury, however, its occurrence with protean chronic inflammatory foci has been described. We report the occurrence of and aggressive squamous carcinoma 3 decades after a high voltage electric mark injury to the scalp. This is the first such case described in the literature. The case supportively highlights the role of chronic inflammation and sepsis in epidermal scar cancer development, and it underlines the importance of appropriate wound management initially and expeditious treatment of sepsis and resistant skin ulceration because the malignant behavior of these tumors is aggressive and unpredictable.
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Primary cutis vertices gyrata is a rare condition of the scalp characterized by the formation of furrows and folds resembling the convolutions of the brain. A review of the English literature did not reveal any surgically treated cases. The authors report three cases treated by simple excision of the deepest furrows with a satisfactory result.
Aplasia cutis is one of the features of the Adams-Oliver syndrome, beside limb anomalies. Aplasia cutis, congenital absence of skin, is a lesion that usually presents over the vertex of the skull. Management of aplasia cutis depends on the size of the skin defect and the child's physical condition. Scalp defects larger than approximately 1 cm should be treated surgically. In patients with aplasia cutis, surgery should preferably be performed using rotation scalp flaps, and additional split skin grafts. The history of two babies with the Adams-Oliver syndrome is presented. In the Adams-Oliver syndrome, large rotation scalp flaps are not reliable due to the abnormal vascularity of the skin. Skin grafting is the safest way, preventing hemorrhage and infection, in the operative treatment of aplasia cutis in these babies.
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