Adrenergic urticaria: review of the literature and proposed mechanism.

Adrenergic urticaria is a rare type of stress-induced physical urticaria characterized by transient outbreaks of red papules surrounded by halos of hypopigmented, vasoconstricted skin. First described in 1985, there are 10 reported cases of adrenergic urticaria in the English-language medical literature. Episodes are caused by various triggers, including emotional upset, coffee, and chocolate, during which serum catecholamines and IgE are elevated, whereas histamine and serotonin levels remain within normal limits. The precise mechanisms leading to the pathogenesis of adrenergic urticaria have yet to be elucidated. Diagnosis can be made by intradermal injection of epinephrine or norepinephrine, which reproduces the characteristic rash, or by clinical observation. Trigger avoidance and oral propranolol are currently the best known treatments for adrenergic urticaria. Nonspecific therapies, including tranquilizers and antihistamines, may also ease symptoms. This article explores the pathophysiology of adrenergic urticaria and proposes a mechanism by which propranolol treats the condition.

Atypical vascular lesion of the breast.

Atypical vascular lesions (AVLs) are vascular proliferations that develop after surgery and radiation for breast carcinoma and may represent precursors to angiosarcoma. AVLs are not well-known entities and currently lack official prognostic factors and guidelines for surgical treatment. We report the case of a patient who developed an AVL, vascular type, 4 years after lumpectomy and radiation therapy for ductal carcinoma in situ of the breast. The patient underwent wide local excision with 1-cm margins with subsequent pathologic examination confirming complete excision of the residual atypical vascular proliferation. This case highlights the importance of close cutaneous surveillance in patients with a history of surgery and radiation for breast carcinoma, and a low threshold for biopsy. More studies are needed to further delineate the risk of AVLs progressing to angiosarcoma and to identify histologic features or immunophenotypic markers, which may be predictive of this risk. Furthermore, formal treatment recommendations for these enigmatic entities would be helpful.

Keloid formation occurring in the distribution of a congenital vascular malformation.

A 35-year-old African American man presented with complaints of malodorous drainage from hypertrophic lesions on his occipital scalp (Figure 1, inset). The patient had no family history of keloid formation and no other keloids on his body. The hypertrophic mass on his scalp had been present for 10 years and had not been a result of any type of mechanical, surgical, or laser treatment. It corresponded to the distribution of a large vascular malformation over the occiput (Figure 1). The vascular malformation extended from the occipital scalp to the right parietal scalp, the right side of the face, neck, upper chest, and right arm, with varicosities and hypertrophy of the right upper extremity (Figure 2). The vascular malformation over the right parietal scalp and ear was characterized by bleb formation and hypertrophy of the right ear. The patient reported that no manipulation, including laser treatment, of the vascular malformation had been previously performed. He did state that a previous dermatologist had attempted serial surgical excision of the cerebriform nodules but retired during the course of treatment. He stated that the appearance of his keloid formation and port-wine stain had not changed during the past 10 years. A previous biopsy of a hypertrophic lesion showed histologic findings consistent with folliculitis keloidalis nuchae. Cephalexin 500 mg 4 times daily for 14 days was prescribed for the purulent drainage. A Doppler ultrasound was ordered of the right upper extremity to evaluate for an arteriovenous malformation and showed no evidence of venous thrombosis or arteriovenous malformation. On a second visit 2 weeks later, the hypertrophic lesions continued to show drainage. Clindamycin gel to be applied twice daily to the scalp was added. The patient also had magnetic resonance imaging with and without gadolinium contrast (Figure 3) ordered, which showed a large hypertrophic giant scalp keloid overlying the occipital and suboccipital region measuring 12x 19 cm. There was soft tissue thickening involving the right external ear, extending inferior to the right ear, overlying an intact parotid gland. There was no evidence of muscular or skull invasion.

The importance of reviewing pathology specimens before Mohs surgery.

BACKGROUND: The review of outside biopsy slides before performing surgery is the standard of care in many surgical specialties. Previous studies have shown high discrepancy rates between the original and second-opinion diagnoses. The frequency with which this practice changes the diagnosis and management of patients undergoing Mohs surgery is undocumented in the literature. It is standard practice at our institution to review all outside biopsy slides before Mohs surgery. OBJECTIVE: To investigate how often review of outside biopsies by an internal dermatopathologist changes patients' initial referral diagnosis and subsequent management. METHODS & MATERIALS: This is a retrospective review of all patients referred to Mohs surgery from January 2003 through March 2007. The number of cases in which the diagnosis changed and how this change affected management were recorded. RESULTS: Seventy-four of 3,345 (2.2%) cases were identified in which the diagnosis changed after review of the biopsy slides. Management was affected in the majority (61%) of cases. Board-certified dermatopathologists originally read nearly half of the biopsies. CONCLUSION: Review of outside biopsy slides before surgery can change the diagnosis in a large proportion of patients, with a resulting change in management. This quality-assurance practice may improve patient care.

Keratoacanthoma: hyperplasia, benign neoplasm, or a type of squamous cell carcinoma?

Keratoacanthomas are common self limited squamous proliferations. They have been considered a benign neoplasm with involution and complete resolution within few months. Although considered the prototypical example of cutaneous pseudomalignancy, some believe that these tumors are squamous cell carcinomas and through the years there have been sporadic reports of "metastasizing keratoacanthomas". The question has been raised as to whether keratoacanthoma is an unreliable histological diagnosis or these tumors have a latent, albeit rare, malignant potential. To date, just a handful of "metastasizing keratoacanthomas" have been reported. Since a benign lesion is incapable of metastasis, some other explanation must be considered; the most likely one being a misdiagnosis. While it is clear that in some cases, the histological and cytological features of squamous cell carcinoma and keratoacanthoma are difficult to distinguish by current techniques, these occasional limitations in diagnosis do not make keratoacanthomas a carcinoma. We believe the evidence supports that keratoacanthomas are benign squamous proliferations. The diagnosis can be made with confidence in appropriate biopsies and using well established clinicopathological criteria.

Multiple primary atypical vascular lesions occurring in the same breast.

Atypical vascular lesions (AVLs) of the breast are rare cutaneous vascular proliferations that appear as flesh-colored or erythematous papules or macules in women who have undergone radiation treatment for breast carcinoma. These lesions can develop in the irradiated area up to 20 years after the radiation treatment but most commonly occur within 3 to 6 years. The general consensus agrees on the benign nature of AVLs; however, their identity as benign lesions has been a source of controversy over the years, with some investigators proposing that AVLs may be a precursor lesion to postirradiation angiosarcomas. Currently, there are no specific guidelines to direct clinicians on the effective treatment of AVLs, but most AVLs are treated with total excision. This rare case describes the development of 4 AVLs within the same breast and stresses the relevance of the field effect in AVL development as well as the importance of field monitoring.

Onychomatricoma: a rare case of unguioblastic fibroma of the fingernail associated with trauma.

Onychomatricoma (OM) is a rare tumor originating from the nail matrix. Fingernail involvement is twice as common as toenail involvement. Onychomatricoma is the only tumor that actively produces a nail plate. Clinically, it presents with yellow discoloration along the entire nail plate, proximal splinter hemorrhages, and a tendency toward transverse overcurvature of the nail plate with prominent longitudinal ridging. We report a rare case of OM in which the etiology was associated with trauma. Onychomycosis can be present, which may serve as a predisposing factor or be secondary to the deformed nail plate. Histologically, the tumor is fibroepithelial or biphasic with stromal and epithelial components. Onychomatricoma has been further classified into 3 types, including unguioblastoma, unguioblastic fibroma, and atypical unguioblastic fibroma. Our case represents the unguioblastic fibroma type.

Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura. The recommended management of levamisole-induced vasculitis currently involves withdrawal of the culprit along with supportive treatment. We describe a patient with levamisole-induced vasculitis who continued to develop skin lesions despite self-reported cocaine cessation. Complete resolution of cutaneous disease occurred with the addition of oral prednisone and vardenafil hydrochloride, suggesting the possibility of a new treatment option in patients with refractory disease. In addition, we review the clinical presentation, disease course, diagnostic approach, laboratory findings, histology, and management of levamisole-induced vasculitis. The harmful effects of levamisole-cut cocaine are serious enough that public alerts have been issued to increase awareness. Clinicians should consider the possibility of levamisole exposure in cocaine users presenting with any combination of fever, neutropenia, and necrotic skin lesions, especially in acral areas including the ears.