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African tick bite fever, resulting from Rickettsia africae inoculation, is endemic in sub-Saharan Africa. We present a United States traveler with African tick bite fever 5 days after she returned from a mission trip to Zimbabwe. The patient exhibited symptomatic hypotension in addition to more typical findings, including fever, fatigue, and a necrotic eschar. The diagnosis was supported by histopathological findings and the patient's symptoms rapidly resolved with oral doxycycline therapy. We believe this case represents the first African tick bite fever diagnosis associated with symptomatic hypotension. This case additionally serves as a reminder of the importance of evaluating patient travel history.

Myxedema is a rare, cutaneous complication of autoimmune thyroid diseases that most often affects the anterior shins. Herein, we report a patient with a history of Graves disease and Hashimoto thyroiditis who presented with boggy, alopecic patches associated with scalp pruritus. Punch biopsies from these lesions showed increased interstitial mucin in the reticular dermis, consistent with localized myxedema. This report showcases a rare presentation of localized myxedema of the scalp, highlighting the diverse cutaneous manifestations of autoimmune thyroid diseases.

Trichodysplasia spinulosa (TS) is a rare skin condition that occurs mainly in immunosuppressed patients. Although initially postulated to be an adverse effect of immunosuppressants, TS-associated polyomavirus (TSPyV) has since been isolated from TS lesions and is now considered to be the causative agent. Trichodysplasia spinulosa presents with folliculocentric papules with protruding keratin spines, most commonly on the central face. Trichodysplasia spinulosa can be diagnosed clinically, but the diagnosis can be confirmed with histopathological examination. Histological findings include the presence of hyperproliferating inner root sheath cells containing large eosinophilic trichohyaline granules. Polymerase chain reaction (PCR) can also be used to detect and quantify TSPyV viral load. Owing to the paucity of reports in the literature, TS is frequently misdiagnosed and there is no high-quality evidence to guide management. Herein, we present a renal transplant recipient with TS that did not respond to topical imiquimod but improved upon treatment with valganciclovir and reduction of the mycophenolate mofetil dose. Our case highlights the inverse relationship between immune status and disease progression in this condition.

Orf virus causes a self-limited infection in humans that resolves without scarring within 6-12 weeks. However, lesions in the immunocompromised can be progressive and disfiguring. The lesions frequently recur after treatment. To our knowledge, there are eleven published cases of these infections. We propose the name orf progressiva to call attention to this progressive, treatment-resistant entity. We present a 43-year-old male ranch owner with a history of renal transplantation who contracted an orf infection from his lamb. The infection recurred despite attempts at debridement, but achieved near complete resolution after treatment with imiquimod and valacyclovir. The histologic findings of orf progressiva are identical to the early stages of classic orf infection and are characterized by epithelial hyperplasia, intracytoplasmic eosinophilic inclusions, and an edematous, vascular dermis. There is no standard treatment for orf progressiva. Surgical excision has frequently resulted in rapid reoccurrence. Topical therapies such as imiquimod and cidofovir cream in combination with excision have been successful in some cases. Acyclovir or valacyclovir with imiquimod has been reported to be effective. Two patients achieved cure with imiquimod alone. We summarize these cases to prompt recognition of orf progressiva as a distinct clinical entity that requires treatment.

We report a patient with new-onset ulcerated granuloma annulare with concomitant involvement of B-cell leukemia. A granuloma annulare-like eruption with concomitant B cell chronic lymphocytic leukemia involvement in the skin is extremely rare, as only three cases have been previously reported in the literature to our knowledge. Given the rarity of ulceration in conventional granuloma annulare, it is possible this finding may serve as a diagnostic clue for underlying malignancy.

Porokeratosis ptychotropica is a rare and commonly misdiagnosed subtype of porokeratosis involving the body folds. We present a 53-year-old man with systemic mastocytosis who presented with a pruritic, verrucous plaque in the gluteal fold that showed multiple cornoid lamellae on histopathologic evaluation, diagnostic of porokeratosis ptychotropica. Various treatments have been reported, including topical corticosteroids, retinoids, vitamin D analogs, calcineurin inhibitors, imiquimod, phototherapy, cryotherapy, or ablative laser therapy, but recurrences are common.

Lichen planus follicularis tumidus (LPFT) is a rare subtype of lichen planus (LP) that has been most commonly described in middle-aged women. LPFT clinically manifests as recurrent cystic follicular nodules that preferentially involve the retroauricular area; concurrent classic LP lesions on the extremities and mucosal surfaces may also be present. Histologically, LPFT demonstrates epithelial-lined follicular cysts filled with orthokeratotic keratin surrounded by a dense lichenoid infiltrate. We present a case of a 67-year-old man with clinical and histopathologic findings consistent with LPFT and discuss differential diagnostic considerations for entities resembling LPFT. Lastly, treatment options for LPFT are reviewed.

The original article was published on July19, 2017 and corrected on May 15, 2018. The revised version of the article includes a funding source for Dr. Maija Kiuru's participation in this case report, awarded by the National Cancer Institute, National Institutes of Health grant K12CA138464. This change appears in the revised online PDF copy of this article.

As the list of anti-tumor immunotherapy agents and the list of cancers treated by these novel agents grow, a subset of patients are experiencing immune-related adverse events as a result of prolonged stimulation of the immune system. Many different immune related adverse events including colitis, hepatitis, pneumonitis, thyroiditis, hypophysitis, and cutaneous reactions can result from blocking these inhibitory pathways. The full spectrum of cutaneous immune related adverse events secondary to checkpoint inhibitor therapy is still being defined. The reported varied presentations include lichenoid reactions and bullous pemphigoid, amongst others. We present a severe cutaneous reaction, a case of debilitating erosive lichenoid dermatitis. This case emphasizes both the wide range of possible cutaneous reactions and the potential severity of these reactions.

Fixed drug eruption (FDE) is an adverse drug reaction characterized by the development of well-circumscribed, round, dusky erythematous macules and plaques on cutaneous or mucosal surfaces. The reaction occurs on the same mucosal or cutaneous site with subsequent exposures to the offending drug. Although FDE usually manifests as a single lesion, in rare instances, more than one lesion may arise and this is referred to as a generalized eruption. Herein, we present a 31year-old man with history of cystic fibrosis who developed a generalized fixed drug eruption to piperacillin/tazobactam (Zosyn, Pfizer). We discuss our patient's course and review causes and outcomes of generalized fixed drug eruptions in the literature.

Acquired zinc deficiency can develop as a consequence of poor nutritional intake or from dependence on total parenteral nutrition. Acquired zinc deficiency dermatitis classically manifests with erosions and scaly plaques in a periorificial and acral distribution. We present a case of a woman on parenteral nutrition who presented with bullous acrodermatitis mimicking pemphigus foliaceus histopathologically. This case highlights clinical and histopathologic variants of zinc deficiency that may lead to a delay in diagnosis.

We present a rare case of acute febrile neutrophilic dermatosis, also known as Sweet syndrome, associated with recurrence of metastatic cervical cancer. This report highlights similar reports and serves as an important reminder of the relationship between Sweet syndrome and cervical cancer. Increasing awareness of Sweet syndrome assists clinicians in recognizing characteristic findings and encourages evaluation of patients for new-onset or recurrent neoplastic disease. Additionally, we discuss the typical presentation of the syndrome, the proper workup and treatment, and a common pitfall encountered in the diagnosis of Sweet syndrome.

A 60-year-old man with chronic lymphocytic leukemiadeveloped a deeply violaceous annular patchwith a halo of erythema on the right thigh duringhospitalization for neutropenic fever. Associatedsymptoms included chronic cough and fatigue.Bilateral lung opacities with hilar lymphadenopathywere noted on chest computed tomographyscan. Punch biopsy and tissue culture confirmeda diagnosis of secondary disseminated cutaneousmucormycosis. Although rare, physicians shouldinclude mucormycosis in the differential diagnosisof purpuric patches in immunosuppressed patients.Prompt skin biopsy and tissue culture may optimizethe success of treatment.

Cyclosporine-induced sebaceous hyperplasia (SH) is a well-documented entity, occurring in up to 30% of renal transplant patients treated with cyclosporine and has also been reported to occur following heart or hematopoetic stem cell transplantation (HCST). Cyclosporine has a stimulatory effect on undifferentiated sebocytes, resulting in the clinical and histologic findings in these patients. Sebaceous hyperplasia most commonly presents as asymptomatic papules over the face, chest, or groin. Herein we describe a case of a 27-year-old man who developed facial sebaceous hyperplasia five months after completing cyclosporine therapy for cutaneous graft versus host disease (GVHD) following HSCT.

Importance: Granuloma annulare is typically a benign, self-limited disease. Atypical presentations have been reported in association with systemic disease, including malignancy. Such patients may require additional diagnostic studies to assess for underlying malignancy. We report a patient with extensive sporotrichoid granuloma annulare-like dermatitis in association with systemic B-cell lymphoma., Observations: An 83-year-old man with a three-year history of progressive sporotrichoid annular plaques and nodules on the arm developed ipsilateral retroauricular palpable lymphadenopathy, the latter consistent with B-cell lymphoma. Multiple skin biopsies of the plaques and nodules revealed granuloma annulare-like dermatitis. Lesions were unresponsive to intralesional and intramuscular corticosteroids, antibiotics, and antifungal agents, but rapidly improved following initiation of rituximab to treat his underlying lymphoma., Conclusions and Relevance: Atypical presentations of granuloma annulare including granuloma annulare-like dermatitis warrant evaluation for systemic malignancy in a subset of patients.

Coccidioidomycosis (AKA "Valley fever") is a primary pulmonary infection via airborne spores released from coccidioides immitis in the soil. Reactive cutaneous eruptions resulting from the pulmonary infection are difficult to diagnose because skin biopsies do not contain the organism. We present an adolescent male with primary pulmonary C.immitis infection manifesting with biopsy proven subcorneal pustular dermatosis. Serological studies revealed increasingly positive titers for coccidioidomycosis and symptoms resolved promptly following initiation of systemic antifungal therapy. Our unique case presentation illustrates subcorneal pustular dermatosis as a reactive eruption owing to primary pulmonary coccidioidomycosis. An association between the two conditions warrants further investigation.

Necrolytic acral erythema (NAE) is a rare cutaneous sign of hepatitis C virus infection and has recently been linked to zinc deficiency. It presents as well-demarcated erythematous plaques in a sandal-like distribution on the dorsal feet with psoriasiform epidermal hyperplasia on histology. Our patient reported a 9-month history of progressive bilateral lower extremity erythema, swelling, erosions, and nail dystrophy that failed to improve despite multiple courses of antibiotics for presumed lower extremity cellulitis. Serum studies revealed zinc deficiency. This case supports the association of NAE with both HCV infection and zinc deficiency and highlights the pitfalls in the diagnosis of chronic unrecognized NAE. Suspected cases of NAE should prompt evaluation for underlying HCV and zinc deficiency to avoid treatment delay and associated complications.

Although the dermatologic community rarely uses "Weber-Christian Disease" as a diagnosis, it still appears in the internal medicine literature. Herein we present a patient with recurrent cutaneous and subcutaneous nodules who was initially treated with aggressive immunosupression for a presumptive diagnosis of Weber-Christian Disease. After more than a decade the patient was diagnosed with cutaneous Mycobacterium chelonea. This case is an excellent example of the difficulty in diagnosing mycobacterial infections and underscores the importance of having a high suspicion for infectious etiologies for unresponsive cutaneous eruptions in patients on immunosuppressive medications.

The dimorphic fungus Sporothrix schenckii commonly causes localized cutaneous disease with lymphocutaneous distribution. However, disseminated sporotrichosis occurs predominantly in immunocompromised patients. We report a case of disseminated cutaneous sporotrichosis in a patient with newly diagnosed HIV with a CD4 count of 208. The patient presented with multiple cutaneous and subcutaneous nodules as well as fever and malaise. Tissue culture and skin biopsy confirmed the diagnosis of sporotrichosis. He was started on itraconazole 200mg twice a day with rapid resolution of fever along with cessation of the development of new lesions.

An 81-year-old woman with a history of renal cell carcinoma and years of slowly, progressively enlarging pulmonary nodules of uncertain etiology presented with several weeks of painful lower extremity nodules. A biopsy revealed changes consistent with nodular vasculitis. A purified protein derivative and QuantiFERON test were positive, favoring the diagnosis of erythema induratum of Bazin. Treatment with a standard four-drug antituberculous regimen resulted in radiographic and clinical improvement. This case emphasizes the importance of dermatologic manifestations in the detection of systemic disease.

Fusarium is a filamentous fungus found naturally in the soil and as a contaminant of plumbing systems. In immunocompetent patients, Fusarium causes localized infection, most commonly onychomycosis. Fusarium tends to be resistant to commonly used antifungal medications. In immunocompromised patients, this antifungal resistance is of particular concern because localized infection quickly becomes disseminated disease with high mortality rates. Disseminated fusariosis presents with skin lesions more often than any other systemic fungal infection. We present a case of fusariosis in an 11-year-old neutropenic patient with acute lymphoblastic leukemia whose only symptom of the fungal infection consisted of painful, indurated nodules and plaques on her legs. The diagnosis and treatment of this invasive fungal infection is also discussed.

An 88-year-old woman presented with an 8-year history of a slowly enlarging growth on the suprapubic area. A shave excision of this lesion demonstrated fibroepithelioma of Pinkus (FEP). Fibroepithelioma of Pinkus is an indolent growing tumor that traditionally has been classified as a variant of the basal cell carcinoma. More recent evidence favors the classification of FEP as a variant of trichoblastoma. In this case report, we identified a large FEP with benign histologic features suggestive of a follicular origin as demonstrated in part by CK 20 staining and the presence of scattered Merkel cells within the tumor. The pathogenesis of FEP remains enigmatic, and the genetic basis has yet to be fully elucidated. Complete surgical resection is considered curative.

A 12-year-old girl with trisomy 21 (Downs syndrome) presented with an approximate 2-year history of an asymptomatic eruption on the right upper arm and left lateral neck that was clinically and histologically diagnostic of elastosis perforans serpiginosa (EPS). EPS is a perforating dermatosis affecting the face, ear, neck, extremities, or trunk. It occurs with greater frequency in persons with trisomy 21, as well as some other genetic syndromes and after D-penicillamine therapy. It has also been reported as a sporadic, presumably unrelated disorder in several acquired diseases.

A 65-year-old man with a history of multiple neoplastic and pre-neoplastic gastrointestinal lesions as well as urogenital carcinoma presented for evaluation of two asymptomatic skin-colored papules in the head and neck region. Biopsy revealed sebaceous neoplasms and immunohistochemical staining was negative for the presence of hMSH-2 protein in both specimens. These findings were consistent with a diagnosis of Muir-Torre syndrome in the setting of a prior history of visceral malignancies. Muir-Torre Syndrome is a rare autosomal dominant genodermatosis associated with mutations in mismatch repair proteins, hMSH-2 and hMLH-1, which predispose affected patients to visceral malignancies as well as sebaceous gland neoplasms.

Background: Numerous treatments have been used to treat the mycosis fungoides (MF) and Sezary syndrome (SS) variants of cutaneous T-cell lymphoma (CTCL). The relative efficacy of different treatments is largely unknown., Objective: To determine the frequency of therapies ranked most effective and least effective for treatment of MF and SS in a sampling of dermatologists in the United States., Methods: Fellow members in the American Academy of Dermatology in the United States between the ages of 35 and 65 years were surveyed regarding demographic variables and treatment efficacy for patch/plaque MF (stages Ia-Ib CTCL), tumor stage MF (stage IIb), and erythrodermic MF/SS (stage III)., Results: Based on 1,399 responses, PUVA was preferred for the management of patch/plaque MF. Total skin electron beam (TSEB) therapy was preferred for tumor stage MF. Photopheresis (extracorporeal photochemotherapy) was preferred for erythrodermic MF/SS, followed closely by TSEB. Especially for tumor stage and erythrodermic MF/SS, physicians without a clear preference comprised the largest group. No significant variation depending on practice type, degree of practitioner experience, or geographic location was identified., Conclusion: PUVA was the preferred treatment for Stage Ia and Ib MF. The predominance of surveys with indeterminate responses suggests limited experience and in the treatment of CTCL, especially in advanced stages.