Chad M. Hivnor

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis : a case series of nine patients and review of the literature

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.

Gene expression profiling of porokeratosis demonstrates similarities with psoriasis

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.

Adverse Events Following Smallpox Vaccination With ACAM2000 in a Military Population

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-naïve 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.

Teledermatology From a Combat Zone

Results. Retinoic acid receptor , RAR , RAR , and RAR 2,4 amplified in 28 of 28 BCC samples and 22 of 22 SCC samples; RAR 1, in 27 of 28 BCCs and 22 of 22 SCC; and RAR 1 , in 24 of 28 BCC and 2 of 22 SCC. Quantitatively, RAR was 3.46-fold increased (P .001); RAR , 1.63-fold increased (P=.001); and RAR 1 , 23.73fold increased in BCCs compared with SCCs (P=.03) (Figure 1 and Figure 2), but only 2 SCCs showed amplification for RAR 1 . The findings for RAR , RAR 1, and RAR 2,4 were indistinct.

Induction of De Novo Hair Regeneration in Scars After Fractionated Carbon Dioxide Laser Therapy in Three Patients

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Subacute radiation dermatitis.

SUBACUTE RADIATION DERMATITIS Subacute radiation dermatitis is a rare cutaneous disorder secondary to radiation-induced tissue damage. The histologic characteristics of this entity have been described in a few reports, and demonstrate an interface reaction with similarities to graft-versus-host disease or a fixed drug reaction. Given the increase in interventional radiologic procedures, it is important to be familiar with this entity, which may result from the cutaneous radiation exposure. In this report, we present 2 instructive cases of subacute radiation dermatitis that highlight this rarely reported disorder.

Psoriatic onychopachydermoperiostitis: improvement with etanercept

SIR, Psoriatic onychopachydermoperiostitis (POPP) is a recently described variant of psoriatic arthritis. It is characterized by psoriatic nail changes (usually onycholysis), painful swelling of the soft tissue close to the distal phalanx, and radiographic changes of the distal phalanges with periosteal reaction and bone erosions. Distal interphalangeal (DIP) joint involvement of the affected digits is characteristically absent. POPP is an important entity for physicians to be aware of in patients with or without other manifestations of psoriasis. Although there is currently no definitive therapy for this condition, the fully humanized anti-TNF(tumour necrosis factor) antibody, adalimumab, has provided improvement in one recent case. A 26-year-old man with a history of scalp and intertriginous psoriasis and a family history of psoriasis presented to our department with pain, swelling and anonychia of both great toes (Fig. 1a) and onychodystrophy of the left index finger. Bacteriological cultures of discharge grew Corynebacterium, Enterococcus and Escherichia coli from the left index finger and group B Streptococcus from the right toe and left index finger. However, the patient responded poorly to antibiotics including dicloxacillin, ciprofloxacin and Augmentin (amoxicillin + clavulanic acid). X-ray of the left index finger showed soft tissue swelling about the DIP joint. X-ray of the great toes showed periostitis of the distal phalanges with soft tissue thickening and absence of DIP joint involvement (Fig. 1b). Phalangeal erosions were also present. The patient was diagnosed as having POPP of the great toes and DIP joint psoriatic arthritis of the left index finger with secondary bacterial infections. After a 6-month trial of subcutaneous etanercept 25 mg twice weekly there was significant symptomatic improvement and normalization of nails in both his left index finger and affected toes. Although his left index fingernail completely normalized, the nails of his great toes have improved from anyonychia to onychodystrophy. This is similar to the case treated with adalimumab, where the great toenails showed only partial improvement. In 1977, Resnick and Broderick first described osseous proliferation of the distal toe phalanges, the ‘ivory’ phalanx, in the absence of adjacent DIP joint abnormalities in patients with psoriasis. In 1989, Fournie et al. advocated the term POPP to describe psoriatic onychosis associated with painful periungual soft tissue swelling and distal phalangeal periostitis without adjacent DIP joint involvement. DIP joint arthritis differs from POPP by having DIP joint involvement and usually by lacking soft tissue swelling and periosteal reaction of the distal phalanx. About 15 cases of POPP have been reported in the medical literature. The great toes are most commonly involved, but POPP can involve other digits. As in our case, patients with POPP may also have DIP joint arthritis of other digits, and affected digits can have secondary bacterial infections. Although POPP is usually seen in patients with psoriasis, it can also be found in patients without psoriatic skin lesions. Given the lack of DIP joint involvement and the common presence of nail involvement, the bony changes in POPP are hypothesized to be caused by the spread of inflammation from the subungual dermis to the bone. The fibrous septum, which directly joins the subungual dermis and the distal phalanx and deeply inserts into the bone, may provide a route for the transmission of the inflammation. Treatment of POPP is difficult. Nonsteroidal anti-inflammatory drugs are usually not effective. Methotrexate has had variable responses. Retinoids, ciclosporin and subungual corticosteroid therapy have not shown benefit. Although etanercept is known to improve psoriatic arthritis, this case represents the first report of etanercept, and only the second case of a TNF inhibitor, benefiting POPP.

Cutaneous Manifestations of Hyperthyroidism

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 patients 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 clinicians 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

Cutaneous Manifestations of Hypothyroidism

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 patients 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 persons 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.