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Local and generalized allergic reactions following laser tattoo removal have been documented, but are rare. To our knowledge, this is the fourth documented case of widespread urticarial eruptions following laser tattoo removal treatment. Unlike previously documented cases, this patient's reaction was found to be associated with titanium dioxide within the tattoo and her symptoms were recalcitrant to medical therapy.A 46-year-old female experienced diffuse urticarial plaques, erythema, and pruritis following multiple laser tattoo removal treatments with an Nd:YAG laser. The systemic allergic reaction was recalcitrant to increasing doses of antihistamines and corticosteroids. The tattoo was finally surgically excised. The excised tissue was analyzed by scanning electron microscopy and energy-dispersive X-ray analysis and contained high levels of titanium dioxide. Two weeks following the excision, and without the use of medical therapy, the patient had complete resolution of her generalized urticaria.Ours is the first documented case of a diffuse urticarial reaction following laser tattoo removal treatments that shows a strong association to titanium dioxide within the tattoo pigment. Herein, we describe a novel surgical approach to treat recalcitrant generalized allergic reaction to tattoo pigment.

Recent advances in laser surgery and our understanding of wound healing have ushered in a new era of trauma and burn scar management. Traditional therapy has centered around scar excision followed by primary closure or tissue replacement with flaps and grafts. This approach represents a perpetuation of the common fallacy that extensive scar improvement requires extensive surgical intervention. Laser surgery in conjunction with pharmacotherapy and minor tissue-conserving surgery produces well-healed and remodeled existing tissue that provides the most natural appearance and function of the skin. Now, patients' hypertrophic, contracted, and disfiguring scars represent their most valuable reconstructive anatomy. With this paradigm shift, dermatologists are uniquely positioned to provide transformative and cost-effective scar therapy due to their proficiency in the necessary treatment modalities and expertise in the utilization of local anesthesia. We hope to further expand military and civilian patient access to such care in their local community through peer education and advocacy. We present a brief overview and outline of scar treatment practices that can be performed by dermatologists in office using devices and techniques they often already possess.

Background: Porokeratosis (PK) is a clinically heterogeneous entity associated with sharply demarcated, annular, or serpiginous lesions with a hyperkeratotic ridge. This disorder is associated with aberrant keratinocyte differentiation that histologically manifests as a stack of parakeratin termed the cornoid lamella; this structure represents the peripheral hyperkeratotic ridge of clinical lesions. Histologically, the keratinocytes forming the cornoid lamella demonstrate an altered differentiation program. However, the molecular basis of PK remains incompletely understood. Methods: As a first step in characterizing PK at the molecular level, gene expression profiling was performed on a cornoid lamella isolated from a large, Mibelli-type porokeratotic lesion. As a control, gene expression profiling of peripheral uninvolved epidermis was also performed. The gene expression profile of cornoid lamellar keratinocytes was compared with similar profiles obtained from a psoriatic plaque and cutaneous squamous cell carcinoma. Results: Our study demonstrates a striking similarity between the gene expression profiles of PK and psoriasis. In addition, novel markers of the porokeratotic keratinocytes were identified, including keratin 16, S-100 A8 and A9, and connexin 26. Conclusions: This study supports the hypothesis that PK is a disorder of hyperproliferative keratinocytes exhibiting similarity at the molecular level to psoriasis. Consequently, some therapeutic modalities efficacious for psoriasis may be of benefit in PK.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare disorder that manifests as the cutaneous formation of nodules composed of light-chain amyloid. Although the type of amyloid deposit is similar to primary systemic amyloidosis, there seems to be little, if any, crossover between the 2 diseases. Because reports of PLCNA are sparse, there is no established protocol for treating this disease. This case report presents a 42-year-old man with a visually striking presentation of PLCNA on both feet with some of the lesions possibly being secondary to trauma, a rare phenomenon. The lesions had been present for more than 4 years, and there were no signs or symptoms of systemic amyloidosis. The lesions responded well to a combination of complete curettage followed by CO2; laser ablation. Primary localized cutaneous nodular amyloidosis is rare and difficult to treat, with high rates of recurrence and a concern for progression to systemic amyloidosis. The diagnosis, workup, treatment, and monitoring of PLCNA also are discussed.

Importance Fractionated, ultrapulsed carbon dioxide (CO 2 ) laser therapy is a powerful tool for the treatment of scars. Common adverse effects of this therapeutic modality have been previously documented. We describe 2 unreported adverse effects of ultrapulsed CO 2 laser treatment of mature scars in a patient previously treated with silver-impregnated dressings. Observations A teenage survivor of toxic epidermal necrolysis presented with faint but diffuse dyschromia clinically and histologically consistent with localized argyria secondary to silver-impregnated dressings used years earlier. The patient was subsequently treated with fractionated CO 2 for her scarring, but her hyperpigmentation worsened with each treatment. A subsequent biopsy specimen revealed a zone of dystrophic calcification with adjacent pseudo-ochronotic fibers that were not appreciated on biopsy specimens taken before CO 2 laser treatment, suggesting unique complications not previously reported. Conclusions and Relevance We present 2 unique complications secondary to ultrapulsed, fractionated CO 2 laser treatment in a patient previously treated with silver-impregnated dressings: (1) the appearance of pseudo-ochronotic fibers in areas of worsening pigmentation and (2) evidence of dystrophic calcification limited to columns of fractionated laser ablation. Therefore, a history of argyria or treatment with silver-impregnated dressings should be considered before treatment with fractionated CO 2 lasers.

Although basal cell carcinoma (BCC) of the nipple-areola complex (NAC) is rare, it is important for dermatologists to be aware of this potential malignancy, as it is thought to behave more aggressively than BCC arising in other anatomic locations and also can mimic a number of more serious conditions. A review of the literature failed to generate a consensus regarding staging or treatment of BCC of the NAC; current therapies range from simple excision of the lesion to mastectomy with sentinel lymph node biopsy. We report the case of a 23-year-old man who presented with a pigmented BCC of the nipple to highlight several important aspects of the diagnosis; we also review 49 cases of BCC of the NAC from the literature and give our recommendations for treatment approach.

Preterm birth is the leading cause of perinatal morbidity and mortality in otherwise healthy infants, and the rate of pregnancies complicated by a premature delivery continues to rise. Subsequently, attempts have been made to reduce this rate by using progesterone supplementation during pregnancy. 17alpha-Hydroxyprogesterone caproate (17P), a metabolite of progesterone, also has been used as supplementation during pregnancy to prevent preterm births. We report a case of iatrogenic autoimmune progesterone dermatitis (APD) in a pregnant woman who received 17P therapy. Due to the increased use of 17P, our case could represent an increasingly prevalent entity that dermatologists and obstetricians should recognize. In this article, we discuss our findings and provide a basic review of APD.

The patient had been treated with topical antifungals and steroids without relief, but a more detailed history suggested a serious infectious etiology.

Background Generalized vaccinia and benign exanthems are 2 adverse events that have been associated with the smallpox vaccination. Accurate incidence and prevalence rates of each are not readily available, but these events are thought to be uncommon. To our knowledge, this is the first case series to provide clinical as well as pathologic descriptions of multiple papulovesicular eruptions occurring after receiving the second-generation smallpox vaccine, ACAM2000 (Acambis, Canton, Massachusetts), among a vaccinia-naive military population. In addition, we report the first confirmed case, to our knowledge, of generalized vaccinia following administration of the ACAM2000 vaccine. Observations All patients received primary smallpox immunization as well as 1 to 3 concurrent or near-concurrent (within the preceding 21 days) immunizations for typhoid, anthrax, hepatitis B, and/or seasonal influenza. One patient presented with a flulike prodrome and diffuse vesiclopustules covering the face, neck, chest, back, and upper and lower extremities. Vaccinia polymerase chain reaction confirmed generalized vaccinia. The remaining 7 patients presented with unusual, painful, and pruritic papulovesicular eruptions occurring on the extensor surfaces of their upper and lower extremities without systemic symptoms. Histologic findings revealed 2 general patterns, including a dermal hypersensitivity reaction with lymphocytic vasculitis and a vesicular spongiotic dermatitis with eosinophils. Conclusions We present the first confirmed case of generalized vaccinia following immunization with the second-generation smallpox vaccine ACAM2000. In addition, we describe 7 cases of benign, acral, papulovesicular eruptions thought to be associated with ACAM2000 administration. Further research is needed to discern the pathogenesis of these benign eruptions as well as their incidence and prevalence and that of generalized vaccinia with ACAM2000.

Chronic granulomatous disease (CGD) is a rare, inherited disorder, in which phagocytic cells, through an enzyme defect, are unable to produce microbicidal oxidants; affected individuals are thereby unduly susceptible to certain life-threatening bacterial and fungal infections and require lifelong antibiotic and antifungal prophylaxis. We present the case of an adolescent CGD patient whose recalcitrant acne vulgaris and subsequent recurrent facial abscesses were successfully treated with isotretinoin; swift resolution of this and similar patients' acne lesions is paramount, as these lesions may serve as a portal of entry for systemic infections and may pose a significant risk for scarring. Isotretinoin is associated with an increased rate of cutaneous Staphylococcus aureus carriage as well as exuberant granulation responses, both of theoretical concern in CGD patients. The therapeutic outcome of isotretinoin in treatment-resistant cases of acne in CGD patients has not been reported in the literature; we present this case to advocate an underreported use of isotretinoin in the prevention of acne, its subsequent cyst formation, and scarring patients with CGD.

Terbinafine is a synthetic oral allylamine that is used for systemic treatment of microscopy- or culture-proven dermatophyte infections of skin and nails. It is normally well-tolerated and side effects include transient gastrointestinal symptoms and skin reactions that can occur in up to 2.3% of treated patients. Subacute cutaneous lupus erythematosus (SCLE) is a skin reaction that has been reported secondary to use of a variety of drugs. The number of reports of SCLE with terbinafine is limited. We demonstrate 2 patients in one dermatology clinic who presented with a predisposing autoimmune diathesis within 3 months of each other.

The key to diagnosing hyperthyroidism from a dermatologic perspective is based on having a high index of suspicion that excess thyroid hormone is responsible for the patient's signs and symptoms. As there are no definitive cutaneous manifestations of hyperthyroidism, a careful review of systems may yield important clinical clues to the diagnosis: Is the patient intolerant of heat? Has there been weight loss? Has the patient experienced any palpitations? Have the bowel habits changed? The unique challenge lies in when systemic symptoms are absent or vague, and the skin manifestations are subtle. Should one routinely check a thyroid-stimuating hormone (TSH) level when the only dermatologic finding is onycholysis? Should one obtain a TSH level before administering botulinum toxin for axillary hyperhidrosis with an otherwise unremarkable review of systems? Should you check thyroid function studies for patients presenting with alopecia areata? There are no definitive answers to these questions. Obviously, the yield will be higher in those patients who have several signs and symptoms referable to a hyperthyroid state. It is my opinion that for isolated findings, such as onycholysis or palmoplantar hyperhidrosis, with an unremarkable review of systems, screening for hyperthyroidism is not mandatory. On the other hand, I believe that it is appropriate to check a TSH level in a woman presenting with alopecia, even if there are no associated constitutional symptoms. When patients present with other autoimmune diseases (i.e., chronic idi-opathic urticaria, dermatitis herpetiformis, lichen sclerosus, etc.) in which there is an increased risk for autoimmune thyroid disease, I think it is reasonable to check for thyroid autoantibodies (anti-thryroglobulin, antithyroid peroxidase), especially if there is a positive family history for autoimmune diseases (notably diabetes mellitus or autoimmune thyroid disease). If positive, these patients may be at a greater risk for the development of autoimmune thyroid disease and should be screened periodically (every 3–5 years) with a TSH assay unless clinical circumstances dictate otherwise. In this era of evidence-based medicine, diagnosing hyperthyroidism is still founded on clinical acumen. Fortunately, a clinician's suspicions are easily confirmed or refuted by straightforward laboratory testing. Maintaining an appropriate index of suspicion for hyperthyroidism will allow patients to be diagnosed and treated expediently, thereby greatly increasing their quality of life

On any given day, in any given bustling dermatology practice, it is highly likely that at least one patient has clinically overt or subclinical hypothyroidism. The cutaneous manifestations of hypothyroidism are protean, affecting the skin and its appendages, as outlined in this chapter. Hypothyroidism literally affects all organ systems and has a profound effect on a patient's overall health and quality of life. While it is not necessary to check a thyroid-stimuating hormone (TSH) level on every individual with dry skin in the wintertime, when recognizing any cutaneous feature of hypothyroidism, we should ask ourselves the following questions: Are any other cutaneous findings present to suggest the diagnosis? Does the patient have any systemic symptoms consistent with the diagnosis of hypothyroidism? A simple screening for TSH and free thyroxine (FT4) levels can change a person's life. If the diagnosis of hypothyroidism is confirmed and treated, clinicians must remain alert for the many associations that accompany this diagnosis over the course of a lifetime.

Skin findings in childhood sarcoidosis vary greatly, but only a few occurrences have been published in which the histopathology has been characterized well. We describe a child with sarcoidosis in whom the cutaneous findings were atypical, resembling granuloma annulare. Histologic examination of these cutaneous lesions, however, revealed areas of sarcoid-like epithelioid cell granulomas, a palisading granulomatous process with features of granuloma annulare, as well as palisading neutrophilic and granulomatous dermatitis and interstitial granulomatous dermatitis. This underscores the variability of skin findings in childhood sarcoidosis--even within the same patient--and suggests that sarcoidosis should be considered in the differential diagnosis of children initially diagnosed with granulomatous skin lesions, such as granuloma annulare, palisading neutrophilic, and granulomatous dermatitis or interstitial granulomatous dermatitis, who demonstrate associated signs of systemic disease.

Epidermal nevi are benign congenital lesions that follow Blaschko lines. Epidermal nevi can demonstrate a variety of histologic patterns and contain hamartomatous hyperplasia of any epidermal or adnexal structure. We present the first reported instance of acne arising strictly within a preexisting keratinocytic epidermal nevus during puberty, which is a demonstration of genetic mosaicism in pilosebaceous units.

Background Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a fibrosing cutaneous disorder recently recognized to have systemic manifestations. The disease is characterized clinically by an acute onset of hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures, and histologically by an increase in spindle-shaped cells, collagen, and sometimes mucin deposition in the dermis. The only common exposure amongst patients is acute or chronic renal failure. The pathophysiology of the disease remains to be elucidated, and there is currently no consistently effective treatment for this unremitting disease. Methods We report a case series of nine patients seen at the University of Pennsylvania between 1998 and mid-2004. The clinical, laboratory, and pathologic data of these patients are reviewed. Results All patients had renal disease, received peritoneal or hemodialysis, and five had received at least one renal transplant. All patients had characteristic fibrotic cutaneous lesions involving the trunk, extremities, or both, and eight of the nine patients had scleral plaques. There were no other common findings amongst the histories, medications, or laboratory results of the patients. Conclusion Our report confirms the clinical and histologic characteristics of NFD that have been described previously, and raises new issues regarding the possible subtypes. A review of the current literature stresses that further basic science and translational studies are necessary to understand the disease mechanism and to propose effective therapy, and emphasizes the importance of recognizing the systemic effects of NFD.

A full-term 1-day-old boy weighing 3140 g was transferred to our tertiary care children’s hospital with large areas of skin defects on his scalp, trunk, and extremities. His parents were nonconsanguineous, and the results of prenatal maternal laboratory screening for human immunodeficiency virus, hepatitis B, group B streptococci, and rapid plasma reagin were negative. Intrauterine fetal death of a twin had been reported at 16 to 18 weeks of gestation. No other complications were noted during the pregnancy. On physical examination, a full-thickness ulcer of the scalp vertex was observed extending from the anterior fontanel to the posterior fontanel in an area overlying the sagittal sinus. The defect measured 5 7 cm and revealed exposed dura and subcutaneous vessels (Figure 1). Two symmetric, vertically oriented, full-thickness defects, each measuring 3.2 cm, with minimal surrounding erythema, were noted on the lateral aspect of the abdomen (Figure 2). Examination of the knees revealed symmetric full-thickness defects that appeared as yellow-red, indurated ulcers. Symmetric, linear scars were observed bilaterally in the axillary and inguinal creases. Magnetic resonance imaging of the head and a magnetic resonance venogram revealed patent major intracranial venous sinuses and slight hypoplasia of the left transverse sinus (considered a normal congenital variation). A relative lack of subcutaneous fat over the superior aspect of the skull was also observed, consistent with the clinical presentation. Concern that the extensive open defect posed a high risk for sagittal sinus thrombosis, hemorrhage, or meningitis prompted plastic surgery and neurosurgery consultations. The scalp defect was repaired by plastic surgery with a skin graft, while the remaining areas of aplasia cutis congenita were allowed to heal by secondary intention. Over the next 3 weeks, the scalp healed well, without complications, as did the other areas of aplasia on the abdomen and knees. DIAGNOSTIC CHALLENGE

Necrolytic acral erythema is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma, and nutritional deficiency syndromes. Necrolytic acral erythema is distinguished by its association with hepatitis C infection and its predominantly acral distribution. We describe a pediatric patient with necrolytic acral erythema whose eruption resolved with hyperalimentation and combination interferon and ribavirin therapy, despite the persistence of detectable viral load and continued hepatic and renal insufficiency.

A 67-year-old woman had a 4-year history of dermato-myositismanifestingasaheliotroperash,shawlsign,Got-tronpapules,proximalupperandlowerextremitymuscleweakness, and dysphagia. The creatine kinase level was424 U/L (reference range, 2-150 U/L) and the erythro-cyte sedimentation rate was 40 mm/h (reference range,0-25 mm/h). Results of the remainder of the laboratoryexamination,includingglobulinlevels,werewithinnor-mal limits. Recent screening colonoscopy, mammogra-phy,Papanicolaousmear,andcancerantigen125levelsshowed no evidence of malignancy. She was refractorytoatreatmentregimenofprednisolone,60mg/d;myco-phenolate mofetil, 750 mg twice daily; and chloro-quine, 250 mg/d. She had not tolerated azathioprine ormethotrexate,andtrialsofquinacrineandhydroxychlo-roquinehadfailed.Shehadchronichypertension,whichwas controlled with ramipril, 5 mg/d. She was a non-smoker, with no personal or family history of hyperco-agulability.Worsening muscle weakness prompted an admis-sion to the Hospital of the University of Pennsylva-nia. She was infused intravenously with 1 g/kg per day(60g/d)of10%intravenousimmunoglobulin(IVIG)inisotonic sodium chloride solution (saline) (Polygam;Baxter Healthcare Corp, Glendale, Calif; distributed byAmericanRedCross,Washington,DC)for2successivedays at rates beginning at 3 g/h, doubling each hour tomaximum rates of 12.5 g/h. After 4 days in the hospitalshe was discharged on her prior treatment regimen.Within 2 weeks of the infusion there was marked sub-jectiveandobjectiveimprovementofhercutaneoussymp-toms and muscle weakness, including swallowing. Thecreatine kinase level had fallen to 123 U/L.She was readmitted 4 weeks after the first infu-sion for another course of IVIG at the same dosage. Atotal infusion of 60 g of 10% IVIG in saline was startedat a rate of 3.5 g/h for the first 2 hours, increased to7 g/h for the next 2 hours, and increased to 12.5 g/hfor the next 2

CASE 1 A 45-year-old man infected with the human immunodeficiency virus (HIV) presented with a 3-month history of violaceousovalmaculesandpapulesonthetrunkandextremities (Figure 1) as well as multiple lichenoid papules over the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints and elbows (Figure 2). Brownish-red scaly patches were observed onthepalms.HismostrecentCD4cellcountwas158cells/ mL, and his viral load was 175102 copies/mL. The patient’s history was significant for Kaposi sarcoma, treated with liposomal doxorubicin, and syphilis, which was treated 15 years earlier with 3 courses of intramuscular penicillin G. The patient had recently developed hypercholesterolemia, induced by highly active antiretroviral therapy, and subsequently had a myocardialinfarction.Secondarysyphiliswassuspected,butfindings from the rapid plasma regain (RPR) test, performed with appropriate dilution to rule out a prozone reaction, were negative. Biopsy specimens of the abdomen and dorsal surface of the right hand both revealed a bandlike and perivascular lymphohistiocytic infiltrate with scattered plasma cells, acanthosis, and exocytosis (Figure 3). WarthinStarry staining revealed several silver-impregnated spirochetalorganisms(Figure 4).Spirocheteswerenotseen on examination of the retina or cerebrospinal fluid. A treponemal-specific serologic test, such as the fluorescent treponemal antibody absorption test, was deferred because this patient had a history of syphilis infection. The patient experienced rapid and complete resolution of his cutaneous disease with 3 weekly courses of intramuscular penicillin G at a dose of 2.4 million IU. CASE 2 A 36-year-old HIV-positive man presented to the dermatology clinic with a 1-year history of pruritic weeping buttock lesions. He had been previously treated by his primary care physician with topical corticosteroids and had completed a 7-day course of famciclovir with

A 34-year-old human immunodeficiency virus (HIV)– seropositive man with a history of Kaposi sarcoma and ocular cytomegalovirus presented with an 8to 10-month history of multiple, progressively enlarging verrucous papules on the proximal nail fold and the distal interphalangeal joint of the right third finger (Figure 1). His CD4 cell count was 21/μL during retroviral therapy. A clinical diagnosis of verruca vulgaris was made, and the patient was treated unsuccessfully with a variety of therapeutic modalities, including liquid nitrogen cryosurgery and hyperthermia, as well as topical imiquimod, tretinoin solution, 0.1% tazarotene gel, and 25%, 50%, and 75% podophyllin, singly and in combination. Subsequently, the patient developed molluscum contagiosum, condylomata accuminata, and periungual warts on 6 other fingers. These conditions were also treated with multiple destructive and topical modalities, without improvement. The verrucous papules on the right third finger coalesced to form a 2-cm, fungating plaque, with resultant nail deformity. The findings of a biopsy that was performed to rule out squamous cell carcinoma showed verruca vulgaris. Because multiple treatments had failed, the verruca was treated with 3% cidofovir ointment, under occlusion, for 8 to 10 hours per day. The molluscum contagiosum and condylomata were treated with topical cidofovir ointment without occlusion. Despite this therapeutic approach, the verrucous plaque on the right third finger enlarged to 2.5 cm. This entire wart was surgically removed, and treatment with topical cidofovir, under occlusion, was reinstituted immediately after reepithelialization. Pathologic examination again showed verruca vulgaris. Within 1 month of debulking, the verruca recurred and enlarged to 3.5 cm. The molluscum contagiosum and condylomata also failed to respond to topical cidofovir therapy.