A detailed analysis of the distribution, morphology, and histopathology of complex purpura in hospitalized patients: A case series of 68 patients.

BACKGROUND: Purpura in inpatients commonly leads to dermatologic consultation. The differential diagnosis is broad and algorithms are intricate. OBJECTIVE: We evaluated inpatient consultations for complex purpura to document the most common diagnoses and to validate the true diagnostic utility of histopathology, clinical morphology, and distribution. METHODS: We reviewed a case series of 68 inpatients during a 4-year period with a dermatologic consultation for purpura and biopsy findings of vasculitis or microvascular occlusion. RESULTS: Key features of complex purpura are nonbranching (round) versus branching (retiform) morphology, dependent versus acral or generalized distribution, and leukocytoclastic vasculitis versus microvascular occlusion (with emphasis on depth of involvement). Dependent nonbranching purpura with only superficial vessels involved by leukocytoclastic vasculitis was most often due to IgA vasculitis or cutaneous single-organ small-vessel vasculitis. In contrast, deeper involvement by leukocytoclastic vasculitis was suggestive of systemic disease (eg, antineutrophil cytoplasmic antibody-associated vasculitis). Branching purpura was concerning, with greater than 90% sensitivity and specificity for microvascular occlusion and associated high mortality (≈50%). The majority of patients who died had acral branching lesions. LIMITATIONS: Small sample size, inpatients at a tertiary care center, and retrospective nature are some limitations. CONCLUSION: Nonbranching dependent purpura corresponded to leukocytoclastic vasculitis, with the most common diagnoses being IgA vasculitis or skin-limited small-vessel vasculitis; patients with deep involvement often had systemic diseases. In this series, branching purpura was due to microvascular occlusion rather than medium-vessel vasculitis, and had associated high mortality.

Nodular amyloidosis.

Nodular amyloidosis is the rarest form of primary cutaneous amyloidosis. We report the case of a 74-year-old woman with an eight-year history of asymptomatic, hyperpigmented plaques on the pretibial areas. A skin biopsy specimen showed deposits of amorphous eosinophilic material that extended throughout the dermis with apple-green birefringence with a Congo-red stain, which established a diagnosis of nodular amyloidosis. Patients with nodular amyloidosis should be evaluated for systemic disease and followed appropriately due to a small risk of progression to systemic amyloidosis.

Sarcoidosis.

We present a 28-year-old man with a one-year history of cutaneous lesions in old scars and tattoos with concomitant subcutaneous nodules and myopathy. A skin biopsy specimen showed cutaneous sarcoidosis. We discuss the multiple aspects of this case, which represent unique presentations of this systemic disease as well as review isomorphic and isotopic responses.

Possible photoactivated dermatitis with features of post-inflammatory pigmentary alteration (PIPA) and rosacea.

Cutaneous flushing and facial erythema are common dermatologic conditions that elicit a wide differential diagnosis that includes rosacea, seborrheic dermatitis, photodermatitis, connective-tissue diseases, carcinoid syndrome, and mastocytosis. Herein we present an usual case of a mask-like rosacea-PIPA overlap that occurred in a patient with prior history of rectal carcinoid tumor and a negative systemic evaluation.

Metastatic squamous cell carcinoma presenting as an erythematous nodule in a man with lung adenocarcinoma.

Skin metastases from visceral malignancies have been well documented in the literature, and may be the presenting sign of an occult internal malignancy. Lung cancer in particular is a relatively common cause of skin metastases, which are considered a poor prognostic sign. Rarely, patients with lung cancer develop a second primary lung cancer that may require a novel chemotherapeutic regimen. The frequency of second primary malignancies presenting with metastatic skin lesions is not documented in the literature. We present a case of a 50-year-old man with a history of metastatic lung adenocarcinoma who was referred for evaluation of a nodule overlying his right mandible, which had been progressively enlarging for two weeks. Biopsy demonstrated metastatic squamous cell carcinoma. Subsequent CT-guided biopsy of a left retroperitoneal lymph node was conducted and notable for squamous cell carcinoma. Therefore, this patient's skin lesion was the presenting sign of a second primary visceral tumor, likely originating in the lung. We present this case to raise clinical awareness of the rare phenomenon that cutaneous metastasis may be the first sign of a visceral cancer, even in the setting of a previous distinct primary malignancy.

White fibrous papulosis of the neck.

We present a 76-year old woman with a five-year history of asymptomatic, white papules that were grouped on the lateral and posterior aspects of the neck, inferior axillae, and central mid-back. The histopathologic findings showed thickened collagen bundles. A diagnosis of white fibrous papulosis of the neck was made, which is believed to be a manifestation of intrinsic aging. There are no treatments for white fibrous papulosis of the neck. One hypothesized approach is the application of a topical anti-oxidant to reduce free-radical induced aging.

Vegetative pyoderma gangrenosum.

Vegetative pyoderma gangrenosum is a rare, superficial variant of pyoderma gangrenosum that is more commonly found on the trunk as single or multiple, non-painful lesions. There is typically no associated underlying systemic disease. Compared to classic pyoderma gangrenosum, vegetative lesions are more likely to heal without the use of systemic glucocorticoids, although up to 39% of patients required a short course of prednisone in a review of 46 cases. Treatments for vegetative pyoderma gangrenosum include topical and intralesional glucocorticoids, minocycline or doxycycline, dapsone, colchicine, and, rarely, alternative steroid-sparing immunosuppressants. We present a case of multiple vegetative pyoderma gangrenosum lesions arising in prior surgical sites in a patient found to have IgA monoclonal gammopathy and abnormal urinary protein electrophoresis.

Adiposis dolorosa.

We report a 46-year-old woman with a nine-year history of obesity; chronic diffuse pain in the adipose tissue of her medial upper arms, lower trunk, and thighs; multiple biopsy-proven lipomas and angiolipomas; and a lipomatous pseudomass of the breast. Her systemic symptoms included generalized weakness, fatigue, memory impairment, and arthralgias. Although some of the lesions were tender, most were only appreciated with palpation. Her clinical history and histopathologic data suggested adiposis dolorosa (Dercum's disease). Owing to the chronic pain, an interdisciplinary approach with the use of analgesics and doxycycline has been initiated.

Nail lichen planus in a patient with alopecia totalis.

A 67-year-old man with a three-year history of non-scarring alopecia that progressed to alopecia totalis despite intralesional glucocorticoid injections is presented. He developed 20-nail dystrophy that was recalcitrant to antifungal and anti-inflammatory treatments. Biopsy of the nail matrix showed histopathologic features of lichen planus. Alopecia totalis and isolated lichen planus of the nails are uncommon subtypes of common dermatologic disorders. Rarely reported concurrently, we provide a review of the literature of their association, which is most likely attributed to their autoimmune pathogeneses.

Necrolytic acral erythema.

Necrolytic acral erythema is a rare, cutaneous manifestation of hepatitis C virus infection that is characterized by erythematous, violaceous or dusky papules, blisters, and/or erosions in the early stages and by well-demarcated, hyperkeratotic, targetoid plaques with a peripheral rim of macular erythema, secondary lichenification and hyperpigmentation, and overlying fine micaceous or necrotic-appearing scale in the later stages. Because most topical modalities prove ineffective, treatment of the underlying viral infection or therapeutic zinc supplementation are required for clinical improvement.

Steatocystoma.

Steatocystoma multiplex is a rare condition that is characterized by cutaneous cysts and may be inherited in an autosomal dominant manner or may occur sporadically. The pathogenesis is hypothesized to involve mutations in the keratin 17 gene. There are no internal manifestations. The lesions are usually asymptomatic. However, a suppurative variant exists in which the lesions become inflamed and suppurative after minor trauma. Treatments include cryosurgery, aspiration, surgical excision, laser therapy, and modified surgical incision techniques. This report presents a case of steatocystoma multiplex, the suppurative variant, in a 26-year-old woman with involvement of rare locations on the buttocks, groin, and extremities.

Granulomatous dermatitis related to silicone implant.

Silicone in liquid and gel implantation may induce granuloma formation and migration. Although there are many complications associated with solid silicone implantation, there have been no published reports of distant granuloma formation. We present a case of a woman with clinical and serologic findings that are consistent with systemic lupus erythematosus and a histopathologic diagnosis of foreign body granulomatous dermatitis 20 years after solid silicone nasal implantation. We review the literature on silicone granulomas and their treatment and speculate on the potential etiologies of a challenging case presentation.

Pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is an inflammatory condition of uncertain etiology. We present a case of PRP that demonstrates acantholysis on histopathologic examination, which is a characteristic feature that increasingly is recognized to aid in the diagnosis. We also review the clinical presentations and treatment options for this condition.

Neutrophilic Dermatoses: a Clinical Update.

Purpose of Review: Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology, clinicopathological features, diagnosis, and management of four disorders: Sweet syndrome, pyoderma gangrenosum, Behçet syndrome, and neutrophilic eccrine hidradenitis. Recent Findings: Recent studies have provided insight into the complex pathogenesis of neutrophilic dermatoses. Evidence supports an intricate interplay of abnormal neutrophil function and inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic predisposition. Summary: Neutrophilic dermatoses have diverse cutaneous and extracutaneous manifestations and may be associated with significant morbidity and mortality. Common underlying associations include infectious, inflammatory, and neoplastic disorders, as well as drug reactions. Emerging diagnostic and therapeutic frameworks identify an expanding role for biologic and targeted anti-inflammatory therapies.

A pilot study examining skin cancer education in an underserved population at a free skin cancer screening.

BACKGROUND: Minority populations are increasingly diagnosed with skin cancer and often in later stages with more aggressive subtypes. OBJECTIVE: We sought to pilot a study to evaluate the effectiveness of providing a skin cancer screening and education module to address potential barriers to dermatologic care for an underserved population in New Haven, Connecticut. METHODS: At a free clinic, voluntary adults (n = 24) waiting for a skin cancer screening were recruited and consented to participate. Participants completed a 16-question survey prior to the total body-skin examination and the educational module, as well as a survey after the examination. RESULTS: Most participants were uninsured (79%) and Hispanic (71%). Pre- and postintervention surveys indicated significant increases in knowledge, risk awareness, and confidence for self-screening. CONCLUSION: This study establishes an effective public health education intervention to promote the prevention of skin cancer. A multicenter study with a larger sample size and longer follow-up period to assess knowledge retention could further address limitations in this initial pilot study.