Clonal cutaneous and neurosyphilis: A pitfall in pseudolymphoma diagnosis.

Syphilis is a sexually transmitted infectious disease caused by the bacterium Treponema pallidum and can cause a wide variety of cutaneous manifestations, most commonly, a papulosquamous eruption of the trunk and extremities. Treatment with penicillin is curative. We report a case of a 69-year-old man who presented with recent onset of blurry vision and a nonpainful, nonpruritic eruption of pink-to-violaceous dermal nodules on his upper trunk and upper extremities. Biopsies of two separate locations revealed a dense superficial and deep perivascular atypical lymphocytic infiltrate with admixed plasma cells, histiocytes, and eosinophils. Some scattered cells expressed CD30, PD1, BCL-6, and ICOS. T-cell receptor (TCR)-rearrangement showed an identical TCR-gamma clone between both biopsy specimens. The patient was subsequently seen by ophthalmology and diagnosed with acute anterior uveitis. Rapid plasma reagin was reactive and cerebrospinal fluid studies showed findings consistent with a diagnosis of neurosyphilis. A T. pallidum immunostain of the skin biopsies was performed upon re-review, and was diffusely positive for spirochetes at the dermal-epidermal junction and within injured vessels. The patient was treated with penicillin G with near-resolution of his skin lesions. This case highlights the unusual ability of syphilis to mimic a T-cell lymphoma with matching clones across two different biopsy sites.

IgG/IgA pemphigus in a patient with a history of pemphigus vulgaris: An example of epitope spreading?

We report a case of IgG/IgA pemphigus presenting as pemphigus foliaceus following diagnosis and treatment of classic IgG-mediated pemphigus vulgaris. The dual presentation of IgG and IgA positivity on direct immunofluorescence (DIF) constitutes a rare form of pemphigus with a wide variety of clinicopathologic manifestations. The progression of pemphigus vulgaris is commonly because of epitope spreading. However, the patient's disease was partially refractory to rituximab and showed a change in the DIF with dual staining for IgG and IgA. This indicates that class-switching may have occurred with epitope spreading or that there was autoreactive IgA at the onset of disease below the threshold of detection by DIF. Our case indicates that in progressive autoimmune disease refractory to treatment, re-evaluation of the patient for antibody isotypes absent on initial diagnosis may offer key information in better identifying the cause of progression as well as in directing the necessary treatment.

Cutaneous mucormycosis with suspected dissemination in a patient with metastatic adrenocortical carcinoma.

Mucormycosis is a frequently lethal fungal infection that most commonly affects patients with poorly controlled diabetes or other immunosuppressed states. We report the case of a suspected disseminated Rhizopus infection in a patient who was pursuing naturopathic treatment including mud baths for metastatic adrenocortical carcinoma. He was empirically treated with liposomal amphotericin B but opted to stop treatment following multiorgan failure. The patient passed away on the tenth day of his hospital admission.

Lymphomatoid Papulosis With T-cell Receptor-Gamma Delta Expression: A Clinicopathologic Case-series of 26 Patients of an Underrecognized Immunophenotypic Variant of Lymphomatoid Papulosis.

Lymphomatoid papulosis (LyP) has several histopathologic presentations. LyP featuring gamma-delta (γδ) T-cell receptor expression may masquerade as and may be misdiagnosed as aggressive cutaneous T-cell lymphoma, particularly primary cutaneous γδ T-cell lymphoma (PCGDTL) or γδ mycosis fungoides. We performed a clinicopathologic analysis of the largest series of LyP featuring γδ T-cell expression. We identified 26 patients with a diagnosis of LyP with γδ T cells from our institutions, as well as through a comprehensive review of the literature, and characterized these cases. Most cases were treated with topical steroids or not treated at all. The majority of cases showed a CD4 - CD8 + phenotype and featured at least one cytotoxic marker. Histopathologic features included an intraepidermal or dermal infiltrate with large cells and frequent angiotropism. One case was initially misdiagnosed as PCGDTL, requiring further therapy. Our case series, the largest international cohort of γδ T cell predominant LyP cases, confirms marked clinicopathologic heterogeneity that may contribute to misdiagnosis, reasserting the need to identify classic clinical features, CD30 + T-cell components, and markers of cytotoxicity when dealing with this differential diagnosis. A limitation of this study includes somewhat limited follow-up, histologic, and immunophenotypic information for some cases.

Possible role of hypercoagulability in calciphylaxis: review of the literature.

BACKGROUND: The role of a hypercoagulable state in the pathogenesis of calciphylaxis has yet to be determined. OBJECTIVE: We sought to find evidence of an association between hypercoagulability and calciphylaxis. METHODS: We reviewed the primary literature for review articles, studies, and case reports that discussed or demonstrated a possible relationship between calciphylaxis and a hypercoagulable state. RESULTS: Review of the primary literature showed that in cases of calciphylaxis with reported levels of protein C and S, 38% of the patients had decreased protein C levels and 43% had decreased levels of protein S. From review of case reports, 3 cases of improvement of skin lesions with low molecular weight heparin treatment, and a fourth case of healing of skin lesions with tissue plasminogen activator treatment, were found. Calciphylaxis was also found in a patient with antiphospholipid antibody syndrome, and a patient with cryofibrinogenemia had clinical and histologic findings consistent with possible calciphylaxis. LIMITATIONS: A limited number of reports were available for review. CONCLUSION: Our review of the literature found sufficient evidence to suggest a possible role of a hypercoagulable state in the pathogenesis of calciphylaxis. A prospective study with serial testing of all relevant clotting factors in patients with calciphylaxis is needed to more definitively establish this role.

A case of pityriasis rubra pilaris with associated focal acantholytic dyskeratosis complicated by Kaposi's varicelliform eruption.

The clinical and histopathological diagnosis of pityriasis rubra pilaris (PRP) can be difficult because clinical findings are often subtle in early stages, and microscopic findings can overlap with those of other skin diseases. Focal acantholytic dyskeratosis (FAD) can rarely be seen in PRP and can mimic Darier's disease, Grover's disease or other disorders characterized by these histopathologic features. Kaposi's varicelliform eruption is a widespread infection due to herpes simplex virus (HSV) types 1 and 2, coxsackievirus A16 or vaccinia virus, occurring in a preexisting dermatosis; only one case has been reported in PRP. We report a patient with PRP whose biopsies showed both herpes simplex infection and FAD. A complete understanding of the mechanism behind this eruption evolved gradually, aided in great measure by the histopathologic findings.

Granulomatous Facial Dermatoses.

Noninfectious facial papular granulomas can be the presentation of several conditions, including granulomatous periorificial dermatitis, granulomatous rosacea, lupus miliaris disseminatus faciei, and papular sarcoidosis. Although these entities are treated distinctly from one another, they share several clinical and histological characteristics. We present 2 cases of facial papular granuloma: one patient presented with granulomatous rosacea, and the other had a presentation consistent with sarcoidosis but also demonstrated features of granulomatous periorificial dermatitis and had a protracted course of treatment. Such cases exemplify heterogeneity in the evaluation and management of this cutaneous lesion and highlight the necessity of appreciating its various potential causes.

Sports Dermatology: Skin Disease in Athletes.

There are numerous disorders of the skin that occur in athletes. These include infections, mechanical injury, and inflammatory skin diseases such as dermatitis, urticaria, and others. This paper discusses some of the most common athletic skin diseases.

Dermatology and skin disease in the American Civil War.

The Civil War happened at the end of the medical dark ages or, conversely, at the beginning of the modern medical era. The story of how physicians and nurses of the time approached a number of cutaneous diseases of importance in the military context is related. Entities discussed include the army itch/camp itch phenomenon, sexually transmitted diseases, scurvy and nutritional disorders, smallpox and spurious vaccination, and hospital gangrene.

The "army itch:" a dermatological mystery of the American Civil War.

The army itch was a chronic, severely pruritic dermatosis which first appeared among soldiers and some civilians early in the American Civil War (1861-1865). As the war progressed, so did army itch, becoming epidemic in the Potomac Valley of Maryland in 1862 and in Virginia in 1864. Immediately after the war, civilian cases traceable to contact with returning soldiers focused attention on the disorder, but the postwar outbreaks were short-lived and the army itch disappeared by the end of 1867. The origin of army itch eluded medical observers of the time, though many considered epidemic scabies to be the cause. Many cases of army itch were successfully treated with scabicides, but the disease had a well-deserved reputation for intractability. After a review of the chronology of army itch and excerpts from the writings of physicians who saw and treated the disease, it is possible to conclude that army itch was epidemic scabies, though the clinical picture was frequently confounded by coexisting pediculosis, prurigo, and other pruritic dermatoses.

Nephrogenic Systemic Fibrosis Manifesting a Decade After Exposure to Gadolinium.

IMPORTANCE: Nephrogenic systemic fibrosis (NSF) is a fibrosing skin disorder that develops in patients with kidney failure and has been linked to exposure to gadolinium-containing contrast agents. The time between exposure to gadolinium and the initial presentation of NSF is typically weeks to months but has been documented to be as long as 3½ years. We report a case of NSF developing 10 years after exposure to gadolinium. OBSERVATIONS: A long-term hemodialysis patient was exposed to gadolinium several times between 1998 and 2004 during magnetic resonance angiography of his abdominal vessels and arteriovenous fistula. In 2014, he was seen at our clinic with new dermal papules and plaques. Biopsy of affected skin showed thickening of collagen, CD34+ spindle cells, and increased mucin in the dermis, supporting the diagnosis of NSF. CONCLUSIONS AND RELEVANCE: The clinical history and histopathological features of this case support the diagnosis of NSF 10 years after exposure to gadolinium. Although the use of gadolinium contrast agents in patients with kidney failure has markedly decreased, patients with exposure to gadolinium years to decades previously may manifest the disease.