Gout Affecting the Nail Unit: Report of Two Cases and Literature Review.

Background: Gout is a depositional, inflammatory disorder that is rarely reported to affect the nail unit. Cases of gout involving the nail unit are likely under-recognized and therefore underreported. We present two cases of tophaceous gout affecting the nail unit and a literature review of the various presentations. Summary: Five cases of gout were identified to affect the nail unit. In all cases, these presented as white hyperkeratotic papulonodules with associated nail dystrophy. Chalky discharge was seen in three of the five cases. Nine cases were identified to have demonstrated pseudocarcinomatous changes that histopathologically mimic squamous cell carcinoma (SCC). Literature review highlights a range of findings including subclinical deposits of uric acid in the nail, onychoschizia, onychorrhexis, and Beau's line. Key Messages: Physicians should be aware of the subtle and nonspecific clinical findings of gout, which may be easily misconstrued for other pathological entities.

Onychodystrophy as the Presenting Sign of Steal Syndrome.

A man in his 70s presented to the dermatology nail clinic with a 1-month history of worsening onychodystrophy, leukonychia, and pain in his left fifth finger. Physical examination revealed a cool hand and absent radial pulse. Ischemia was suspected, and the patient was sent to the emergency department where the diagnosis of steal syndrome was made and his previously required arteriovenous fistula was ligated. This case highlights the clinical features of steal syndrome, that nail changes should be recognized as clinical features, and that urgent triage of these patients to vascular surgery is of critical importance.

Expert Consensus on Nail Procedures and Selection of CPT Codes.

BACKGROUND: Dermatologists specialize in treating conditions of the skin, hair, and nails; however, it is our experience that the field of nail diseases is the least discussed facet of dermatology. Even less acknowledged is the complexity of nail procedures and how best to accurately code for these procedures. OBJECTIVE: To convene a panel of experts in nail disease to reach consensus on the most accurate and appropriate Current Procedural Terminology (CPT) codes associated with the most commonly performed nail procedures. METHODS: A questionnaire including 9 of the most commonly performed nail procedures and potential CPT codes was sent to experts in the treatment of nail disease, defined as those clinicians running a nail subspecialty clinic and performing nail procedures with regularity. A conference call was convened to discuss survey results. RESULTS: Unanimous consensus was reached on the appropriate CPT codes associated with all discussed procedures. LIMITATIONS: Although this article details the most commonly performed nail procedures, many were excluded and billing for these procedures continues to be largely subjective. This article is meant to serve as a guide for clinicians but should not be impervious to interpretation in specific clinical situations. CONCLUSION: Billing of nail procedures remains a practice gap within our field. The authors hope that the expert consensus on the most appropriate CPT codes associated with commonly performed nail procedures will aid clinicians as they diagnose and treat disorders of the nail unit and encourage accurate and complete billing practices.

Lipoatrophic panniculitis in an adolescent.

Lipoatrophic panniculitis (LP) is a rare childhood panniculitis characterized by sclerotic, atrophic plaques on the extremities. We present a case of LP diagnosed during the inflammatory phase that was difficult to distinguish clinically from eosinophilic fasciitis. This report adds to the limited phenotypic spectrum of LP by differentiating the clinical features of disease activity from disease damage and highlighting the importance of biopsy in establishing a diagnosis.

Clinical, Imaging, and Pathologic Features of Conditions with Combined Esophageal and Cutaneous Manifestations.

A variety of clinically significant conditions can affect both the esophagus and the skin. Esophageal and cutaneous manifestations may directly reflect the underlying disease process, as in infections such as herpes simplex virus, bullous diseases such as epidermolysis bullosa and mucous membrane pemphigoid, connective tissue diseases such as systemic sclerosis, and inflammatory diseases such as lichen planus. Alternatively, esophageal and cutaneous findings may result from conditions that are closely associated with and potentially pathognomonic for but distinct from the underlying disease process, as in genetic diseases such as Cowden syndrome or paraneoplastic syndromes such as acrokeratosis paraneoplastica. Other diseases such as Crohn disease may have cutaneous manifestations that directly reflect the same underlying inflammatory process that affects the gastrointestinal tract or cutaneous manifestations that represent reactive or associated conditions distinct from the underlying inflammatory process. The cutaneous manifestations of disease may precede, coincide with, or follow the esophageal manifestations of disease. The authors present the characteristic clinical features and imaging findings associated with common and uncommon conditions that have esophageal and cutaneous manifestations. Each condition is presented with a brief overview, discussion of salient clinical and cutaneous manifestations, and description of the typical esophageal imaging findings, with particular attention to implications for diagnosis, prognosis, and treatment. Recognition of potential associations between cutaneous lesions and esophageal imaging findings is important for establishing a specific diagnosis or generating a meaningful differential diagnosis.

Palisaded Neutrophilic Granulomatous Dermatitis Leading to Diagnosis of Hodgkin Lymphoma: Report of Rare Case and Literature Review of Paraneoplastic Granulomatous Dermatitides.

Palisaded neutrophilic granulomatous dermatitis (PNGD) presents as skin-colored to erythematous papules symmetrically distributed on extensor extremities. Histologically, interstitial and/or palisaded granulomatous infiltrates are present with neutrophils and leukocytoclasis. Together with interstitial granulomatous dermatitis (IGD), PNGD is considered a reactive granulomatous dermatitis to a systemic trigger including connective tissue disease, infection, medication reaction, and less commonly malignancy. We present the second case of PNGD leading to diagnosis of Hodgkin lymphoma. A 47-year-old woman presented with a new asymptomatic eruption of erythematous, nonscaly papules and thin plaques with variable annular configuration, symmetrically distributed on extremities. Biopsies revealed mid-dermal focal degeneration of collagen and leukocytoclasis in association with a palisaded mixed inflammatory infiltrate of neutrophils, lymphocytes, and histiocytes, some forming small granulomas. She had associated fevers, night sweats, weight loss, arthralgias and malaise. Imaging revealed retroperitoneal adenopathy. Excisional lymph node biopsy led to diagnosis of Hodgkin lymphoma. Paraneoplastic PNGD is rare with only 9 reported cases. In all cases, PNGD presented concurrently or before presentation of clinical signs/symptoms of underlying neoplasm. We reviewed a total of 37 cases of paraneoplastic PNGD/IGD, summarizing clinical presentation, histology, associated neoplasm, and response of the skin lesions to treatment. The most commonly associated neoplasia is hematologic, with myelodysplastic syndrome (9 of the 37 cases or 24%) being the most frequent. Seven cases of solid tumor malignancies (prostate, esophageal, hypopharyngeal, breast, endometrial, and lung cancers) have been reported. Recognition of paraneoplastic PNGD/IGD may allow dermatopathologists, pathologists, and dermatologists to direct further workup that reveals underlying malignancy.

Tender finger nodule.

A male in his twenties presented with a tender finger nodule that had been present for 3 months. Histopathological examination revealed a dermis with nodules of necrotic, athypical epithelaia cells with high nuclear to cytoplasmic ratios. Glandular formation was present with lumens lined by columnar epithelium, consistent with digital papillary adenocarcinoma. Digital papilary adenocarcinoma is a rare malignant adnexal tumor arising from sweat glands and requires further work-up.

Inflammatory diseases of the nail unit.

inflammatory disorders of the nail unit are frequently encountered in clinical medicine and are a cause of significant morbidity. Psoriasis, lichen planus, chronic paronychia, and trachyonychia will be discussed. An approach to diagnosis and management of these disorders requires knowledge of nail unit anatomy, consideration of associated systemic manifestations, and patient education with respect to prognosis and management of factors that will maximize disease improvement. The nail unit responds with a limited number of signs to disparate clinical entities such that there is some overlap in clinical presentation between inflammatory conditions. Nail unit biopsy may therefore be useful in establishing a specific diagnosis.

Eccrine Porocarcinoma Treated by Mohs Micrographic Surgery: Over 6-Year Follow-up of 12 Cases and Literature Review.

BACKGROUND: Eccrine porocarcinoma (EPC) has a poor prognosis after standard wide local excision (WLE), with 20% local recurrence, 20% regional and 12% distant metastatic rates. Mohs micrographic surgery (MMS) has been used as a promising treatment. OBJECTIVE: To review the use of MMS for EPC and assess treatment outcomes. MATERIALS AND METHODS: This was a retrospective case series of 12 EPC patients treated by MMS between 1984 and 2013 in the institution. Furthermore, a literature review revealed an additional 17 cases of EPC managed by MMS. RESULTS: Of 29 cases of EPC treated by MMS, outcome was established in 27 cases. The patients had a significantly longer mean follow-up period of 6 years (range, 4-206 months), as compared with 19 months (range, 2-48 months) in reported cases. Two patients had regional lymph node metastasis after MMS. The regional metastatic rates to lymph nodes were 7% (2/27). There was no local recurrence, distant metastasis, or disease-specific death in the 27 cases studied. CONCLUSION: To the best of the authors' knowledge, this is the single largest case series of EPCs treated by MMS and the authors' data demonstrated that MMS may be superior to the standard WLE.

Lipoatrophic panniculitis: case report and review of the literature.

BACKGROUND: Lipoatrophic panniculitis (LP) is a rare disease of childhood characterized by eruption of tender erythematous nodules and plaques followed by circumferential bands of lipoatrophy often seen on the arms and legs. This condition has also been known as lipophagic panniculitis of childhood, annular atrophy of the ankles, and partial lipodystrophy. OBSERVATIONS: A previously healthy 8-year-old boy was evaluated for tender, raised plaques on the ankles, which progressed to circumferential atrophy of the distal lower extremities. Biopsy specimen analysis revealed a dense mixed infiltrate extending into the subcutaneous tissue as well as lipophages within the fatty lobules. A diagnosis of LP was made, and the patient began treatment with prednisone and hydroxychloroquine. Methotrexate was added later to the regimen as a steroid-sparing agent, and the dose was increased over the course of 3 months, by which time the cutaneous disease progression was nearly halted. However, the patient continued to have lower leg pain with bone changes demonstrated on magnetic resonance imaging. CONCLUSIONS: We report this case and review of the literature to call attention to the clinical features of LP and its association with skeletal changes. Our patient's response to combination therapy is of interest and contributes to the limited literature about management of this disease.