Folliculocystic and Collagen Hamartoma: A Distinct Hamartoma Associated With Tuberous Sclerosis Complex.

ABSTRACT: Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by cutaneous and extracutaneous hamartomas. Dermatologic evaluation is critical for early diagnosis because mucocutaneous manifestations account for 4 of 11 major and 3 of 6 minor diagnostic criteria. Folliculocystic and collagen hamartoma (FCCH) is a recently described entity associated with TSC. We herein describe the case of a 28-year-old woman with a history of TSC who presented with a scalp lesion present since childhood. Physical examination revealed a solitary, well-circumscribed exophytic tumor over the occipital scalp measuring 9 × 8 cm and covered with comedones and cyst-like structures. Biopsy of the lesion demonstrated thickening of the collagen bundles throughout the dermis, concentric perifollicular and perivascular fibrosis, an increased number of dilated vessels, and keratin-filled cysts lined by the infundibular epithelium. Clinicopathologic correlation was diagnostic for FCCH. The patient was referred for surgical excision. In addition, we review 11 other cases of FCCH previously reported in the literature.

Treatment of erythrodermic psoriasis with biologics: A systematic review.

BACKGROUND: Biologic medications for plaque psoriasis have been used to treat erythrodermic psoriasis (EP). Since the guidelines for management of EP were published, new biologic medications have been approved for the treatment of plaque psoriasis. OBJECTIVE: To analyze the evidence of biologic medications in the treatment of EP based on response and tolerability. METHODS: A comprehensive search was conducted with the PubMed, Cochrane Library, Embase, and Scopus databases through December 31, 2018. Studies reporting 1 or more cases of EP, defined as >75% body surface area involvement, in patients aged ≥18 years treated with biologics were included. Baseline Psoriasis Area and Severity Index score, score improvement, and adverse events were documented. Adequate response to treatment was defined as Psoriasis Area and Severity Index ≥50. RESULTS: Included were 43 articles, yielding a total of 179 patients. Most patients responded at some point during treatment, with a higher level of evidence for infliximab, ustekinumab, ixekizumab, and guselkumab. Infection was the most common adverse event (n = 35). LIMITATIONS: Data are limited to case reports, case series, and uncontrolled studies. CONCLUSION: Patients with EP treated with biologics demonstrated positive responses and treatment was well-tolerated, with a weak recommendation and limited quality of evidence in favor of infliximab, ustekinumab, ixekizumab, and guselkumab.

Role of Skin Biopsies in the Diagnosis of Hemophagocytic Lymphohistiocytosis.

This is a report of a 15-year-old female who presented with a skin eruption and clinical features consistent with severe sepsis and septic shock. A diagnosis of acquired hemophagocytic lymphohistiocytosis (HLH) was made and confirmed by bone marrow analysis. Skin biopsy showed hemophagocytosis and CD163-positive staining of macrophages. We briefly review the clinical, histologic, and laboratory findings of hemophagocytic lymphohistiocytosis in addition to the potential role of skin biopsies in this condition.

Extraocular Sebaceous Carcinoma-A Clinicopathologic Reassessment.

Sebaceous carcinoma is an aggressive adnexal neoplasm with sebaceous differentiation. Few reports have described the histopathologic characteristics of the sebaceous carcinoma occurring extraocularly. Seventy-two cases of extraocular sebaceous carcinoma were identified from the database of a Dermatopathology Laboratory from January 1, 2007 to May 31, 2013. More cases occurred in men (60%), with a mean age at diagnosis of 65.8 years (range 39-99 years). Neoplasms were histopathologically classified as well-differentiated (22%), moderately differentiated (67%), and poorly differentiated (11%). Sixty-seven percent (67%) of cases demonstrated a squamoid growth pattern and thirty-three percent (33%) demonstrated a basaloid growth pattern. A majority of the neoplasms histopathologically classified as well-differentiated (94%) and moderately differentiated (65%) demonstrated a squamoid growth pattern. Ten percent (10%) of cases exhibited cystic histopathologic changes. The histopathological features reported in this study aid in the understanding of extraocular sebaceous carcinoma and its eventual diagnosis and classification.

Refractory dissecting Cellulitis of the Scalp Successfully controlled with Adalimumab.

Dissecting cellulitis of the scalp (DCS) is an uncommon inflammatory disease that often results in scarring alopecia. Numerous therapies have either proved ineffective or only temporarily effective in the management of this condition. Recent reports show adequate responses to tumor necrosis factor (TNF) inhibitors in cases of DCS. We report a case of severe recalcitrant DCS successfully treated with adalimumab.

Bilateral Granulomatous Iridocyclitis Associated with Early-Onset Juvenile Psoriatic Arthritis.

Purpose: The purpose of this study is to report on a case of bilateral granulomatous iridocyclitis in a patient with early-onset juvenile psoriatic arthritis (JPsA). Methods: The method used is an observational case report. Observations. A 3-year-old Hispanic girl was sent to our uveitis service for further evaluation of her granulomatous uveitis. The initial ophthalmologic examination revealed bilateral band keratopathy, large mutton-fat keratic precipitates, multiple posterior synechiae, and 4+ anterior chamber cells. The physical exam was notable for left knee edema and right axillary rash. Laboratory testing was remarkable for an erythrocyte sedimentation rate of 80 mm/h, positive antinuclear antibodies (1 : 1, 280), and negative human leukocyte antigen B27. A cutaneous biopsy was obtained, which confirmed the diagnosis of a psoriatic rash. Treatment with oral prednisolone and topical prednisolone acetate with atropine sulfate resulted in the complete resolution of the uveitis. Conclusion and Importance. Bilateral granulomatous iridocyclitis may be a rare presentation of ocular involvement in patients with early-onset JPsA.

Failure of Biologic Therapy in Psoriasis.

OBJECTIVE: This study aims to describe the frequency of biologic therapy failure in psoriasis patients along with associated patient demographics and characteristics. METHODS: This was a retrospective medical-record review of psoriasis patients evaluated from January 1st, 2013, through May 1st, 2018, and who failed at least once to adhere to their biologic therapy. RESULTS: Seventy-seven patients with psoriasis who had discontinued biologic therapy at least once were included in this study. Hypertension (58.4%), diabetes (37.7%), dyslipidemia (27.3%), and psoriatic arthritis (23.4%) were the main comorbidities observed. Adalimumab (ADA, 80.5%), ustekinumab (UST, 70.1%), and etanercept (ETA, 14.2%) were the most frequently used biologics in our cohort. The biologic with the longest mean duration of use prior to its discontinuation was UST (17.0 months), followed by ADA (15.9 months) and ETA (13.6 months). CONCLUSION: The most common reason for discontinuing biologic therapy was that said therapy was not effective, though for ETA and UST, the fact that biologic therapies are not universally covered by insurance company was found to be associated with their discontinuation, as well. There were no statistically significant associations found between biologic therapy discontinuation and age, gender, or comorbidities, which last included obesity, class I. Larger studies are warranted to identify risk factors associated with biologic therapy failure to help guide drug selection, decrease morbidity associated with such nonadherence and improve patient outcomes.

Psoriatic Alopecia in a Patient With Crohn Disease: An Uncommon Manifestation of Tumor Necrosis Factor α Inhibitors.

Tumor necrosis factor α (TNF-α) inhibitors are used to treat multiple inflammatory diseases including rheumatoid arthritis, inflammatory bowel disease, and psoriasis, among others. This family of medications can cause various side effects, some as common as injection-site reactions and others as rare as the paradoxical induction of psoriasiform skin lesions. Alopecic plaques recently have been described as an uncommon adverse effect of the TNF-α inhibitors adalimumab and infliximab. We present the case of a 12-year-old girl treated with adalimumab for Crohn disease who developed an alopecic crusted plaque on the scalp 6 months after increasing the dose of the medication. Biopsies, special stains, and sterile cultures yielded a diagnosis of psoriatic alopecia secondary to TNF-α inhibitor. A literature review for similar cases found 24 additional patients presenting with similar findings, of which only 6 were part of the pediatric population.

Biological therapy for pustular psoriasis: a systematic review.

BACKGROUND: The anecdotic evidence of the benefits from biologic agents for psoriasis is extensive. However, data on the efficacy of biologic agents for pustular psoriasis are limited. METHODS: To update the data on the efficacy and safety of biologic agents for the management of pustular psoriasis. A systematic review of published data regarding biologic therapies on PubMED database, used in the management of pustular psoriasis from 2012 was undertaken. RESULTS: A total of 209 articles were identified, and 43 articles were selected for inclusion. TNF-α inhibitors were used in 205 patients, and 86 patients received ustekinumab, secukinumab, brodalumab, ixekizumab and IL-1 inhibitors. Overall response was favorable for most modalities. No serious adverse events were reported. Inconsistent measures of treatment response and study variability limited the overall evaluation of data. CONCLUSIONS: Infliximab and ustekinumab have the most evidence of efficacy and safety for the treatment of pustular psoriasis. Recent evidence supports the use of IL-17 antagonists. Prospective controlled and comparative trials are needed to further explore the efficacy and safety of biologic agents in order to establish objective recommendations for the management of this challenging condition.

Focal dermal hypoplasia with unusual cutaneous features.

Focal dermal hypoplasia (Goltz syndrome) is a rare genetic condition characterized by numerous malformations in different organ systems derived from the ectoderm and mesoderm. We present an infant with focal dermal hypoplasia who, besides having a constellation of anomalies commonly encountered in patients with this syndrome, manifested additional unusual features such as an early inflammatory vesicular stage and a cleft lip and palate. We emphasize that a prompt, well-orchestrated and effective multidisciplinary intervention can help improve the quality of life in patients afflicted with this condition.

Acral syringomas presenting as a photosensitive papular eruption.

A 43-year-old woman presented with a recurrent and remitting eruption of reddish-brown papules in the sun-exposed areas of the arms and forearms that had been present for 2 years. Results of a histopathologic examination revealed syringoma. To the best of our knowledge, this is the first case report of syringomas confined to sun-exposed areas with a recurrent and remitting course over time. We also review the literature on acral syringomas.

New insights into imiquimod's mechanisms of action.

Imiquimod, an immune response modifier with potent antiviral and antitumor properties, has been reported to be effective in the treatment of various cutaneous neoplasms. Besides stimulating the production of pro-inflammatory cytokines through Toll-like receptors on the surface of dendritic cells of monocyte-macrophage lineage, novel antiapoptotic mechanisms have been identified.

Granular parakeratosis.

This is a report of a 60 year-old black female patient presenting with pruritic brownish crusted plaques on both axillae of one month evolution. Histopathology revealed findings characteristic of axillary granular parakeratosis. This entity was first described by Northcutt et al in 1991. Since then, involvement of other intertriginous areas have also been reported. A review of the literature was performed and the term granular parakeratosis is suggested to emphasize its pathognomonic histopathologic features.

Modified curettage technique for molluscum contagiosum.

Molluscum contagiosum is a frequently occurring, virally induced cutaneous condition that affects infants, children, and adults. Although historically considered a self-limiting entity, many patients experience prolonged infections, often resistant to varied therapeutic interventions. Topical treatments achieve complete clearance in only a limited number of patients. Because of the associated pain, affected children are characteristically uncompliant to commonly used destructive modalities. We describe a modification of the traditional curettage technique to remove lesions of molluscum contagiosum, which has proven to be effective and well tolerated by a significant number of patients in our practice.

Imiquimod: an effective alternative for the treatment of invasive cutaneous squamous cell carcinoma.

BACKGROUND: Some patients with invasive cutaneous squamous cell carcinoma are either not adequate surgical candidates or present with lesions in cosmetically sensitive areas in which a surgical procedure might result in an apparent and/or a cosmetically unacceptable scar. OBJECTIVE: To evaluate the effectiveness of imiquimod 5% cream in the treatment of an invasive squamous cell carcinoma in the nose of a young man. METHODS: Imiquimod 5% cream was applied to the lesion five times a week for 12 weeks. RESULTS: Complete clinicopathologic tumor clearance and an excellent cosmetic result were achieved after 12 weeks of imiquimod treatment. CONCLUSION: Imiquimod 5% cream may represent a reasonably effective alternative for the management of invasive squamous cell carcinoma in selected patients.