A Report of 10 Individuals with Weathering Nodules and Review of the Literature

Weathering nodules are a benign skin condition that usually present as papules on the helices of patients with significant prior sun exposure. They are easily recognized clinically and blanch upon application of pressure to the adjacent helical rim: a positive blanch sign. We describe the clinical presentation of weathering nodules in 10 patients, nine men and one woman, aging from 38 to 70 (median 59), and their associated risk factors. Eight patients had a history of actinic keratosis, three had a history of nonmelanoma skin cancer, and all patients had increased Sun exposure through outdoor activities. Weathering nodules are rarely mentioned in the literature and may be confused with other cutaneous disorders. Therefore, it is paramount for clinicians to become familiar with weathering nodules and include them in the differential diagnosis of ear nodules. Appropriate diagnosis will help avoid unnecessary biopsies while reassuring the patient that the lesions are benign.     

Trichoepithelioma and Basal Cell Carcinoma with Squamous Differentiation: Is it Causal or Coincidental?

Trichoepitheliomas (TEs) are benign cutaneous tumors that occur either as solitary non-familial or multiple familial. We report a case of multiple familial trichoepithelioma (MFT) in a 55-year-old female patient and her son who came with complaints of single ulcerated mass involving the left nasolabial fold and cheek. She had multiple papules and nodules all over the face and neck since 25 years. Histopathological examination of an ulcerated lesion revealed features of basal cell carcinoma (BCC) with squamous differentiation, which was confirmed by immunohistochemistry. A skin biopsy obtained from the papule on neck showed features of TE. However, whether BCC developed independently or by transformation from TE was uncertain. Her 36-year-old son presented with similar lesions on the face and a skin biopsy showed features of TE. Though malignant transformation of TE is quite rare, awareness of the potential for evolution of carcinoma in patients with MFT is important for management of these patients.     

Extensive cutaneous manifestations: presenting feature of chronic myelocytic leukemia in second blast crisis

Leukemia cutis is the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutis, resulting in clinically identifiable cutaneous lesions. We describe a case of CML who presented with extensive cutaneous manifestations at the time of second blast crisis with multiple subcutaneous skin nodules over the face and trunk with extensive violaceous papules and plaques over all four limbs and the trunk, with scalp showing extensive crusting and scaling with foul smelling discharge.     

Nonfamilial Multiple Trichoepithelioma: Few and Far Between

Trichoepithelioma is a rare benign adnexal tumor which may be of solitary nonfamilial type or multiple familial trichoepitheliomas. Here, we describe a rare presentation of nonfamilial case of trichoepithelioma in a 50-year-old female with multiple skin colored facial papules and nodules over the face, upper back, and extremities.     

Angiolymphoid Hyperplasia with Eosinophilia Mimicking Multiple Cylindromas: A Rare Case Report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder. It is characterized by red to brown papules and nodules, typically localized on the head and neck, particularly around the ear as singular or multiple lesions. Although ALHE is a benign disease, lesions are often persistent and difficult to eradicate. Young to middle age women are more commonly affected. The histological examination corresponds to a florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate. We describe an elderly male with multiple nodular lesions over the scalp mimicking cylindromas; the histological examination was consistent with ALHE.     

Multiple Dermatofibromas: Dermoscopic Patterns

Dermatofibromas are benign skin lesions that consist of pigmented papules or nodules. They produce the dimple sign when laterally squeezed and are usually found on the legs. These clinical features lead to the diagnosis in most cases. However, the differential diagnosis with other lesions, such as atypical nevi and melanoma can be difficult, and the dermoscopy may help the diagnosis. There are several dermoscopic patterns associated with dermatofibromas, the most common being a central white scar like patch with delicate pigment network at the periphery. This article describes the case of a patient who had eleven clinically similar dermatofibromas, with four distinct patterns when submitted to dermoscopic examination.     

Genital leiomyomas of the male nipple: two cases

BACKGROUND: Genital leiomyoma is a rare benign solitary skin tumor, not painful, developed from smooth muscle. Genital leiomyoma arising from the nipple is extremely rare, especially in males.CASES REPORT: A 47 year-old male had a 2 cm cutaneous plaque with nodules located on the right nipple. This plaque was circumscribed, erythematous, pruriginous and was not painful. The lesion had been noticed by the patient two years ago. A 37 year-old male showed a 1.5 cm cutaneous plaque located on the left nipple. The plaque was slightly erythematous, pruriginous, not painful and had been noticed by the patient 5 years earlier. Histology provided the diagnosis of genital leiomyoma in both cases. No surgical therapy was performed.DISCUSSION: Cutaneous leiomyomas are classified in 3 types regarding their origin: multiple or solitary piloleiomyoma, arising from arectores pilorum muscles, solitary genital leiomyoma, arising from the dartoic, vulvar, or mammillary muscles, and solitary angioleiomyoma, arising from the vein muscles. Clinically, genital leiomyoma is a 1 cm diameter solitary erythematous, firm nodule. According to many authors, genital leiomyoma is asymptomatic, but in the 2 patients, the lesions were pruriginous. Surgical excision is usually performed.     

MULTIPLE CUTANEOUS LEIOMYOMAS: PAIN RELIEF WITH PULSED HYSOCINE BUTYL BROMIDE

A 35-year-old male patient presented to our outpatient department, complaining of multiple, raised skin lesions on the forehead and back, associated with intermittent pain, especially on exposure to cold. A diagnosis of cutaneous leiomyoma (type 2 segmental) was made, which was confirmed by skin biopsy. The patient was started on a trial of pulsed Hyoscine Butyl bromide tablets, following which the patient had significant relief from pain associated with the lesions.     

Solitary Pilar Leiomyoma of the Nasal Dorsum: Case Report and Literature Review

Cutaneous leiomyoma is an infrequently occurring benign tumor that arises from smooth muscle in the skin. Pilar leiomyoma, a subtype of cutaneous leiomyoma, arises from the arrector pili muscle associated with hair follicles. Pilar leiomyoma, particularly in the head and neck region, is rare and frequently misdiagnosed. We report one patient who developed pilar leiomyoma of the nasal dorsum, explore the differential diagnosis and review the characteristics of previously reported individuals with pilar leiomyoma of the nasal dorsum.     

CRUSTED PILOLEIOMYOMA WITH MENTAL RETARDATION: A RARE ASSOCIATION

Piloleiomyoma is an uncommon benign smooth muscle neoplasm arising from arrector pili muscle. It is clinically defined by the presence of solitary or multiple reddish brown, dome-shaped, smooth papules or nodules, ranging in size from a few millimeters to a centimeter. The patients are otherwise healthy; but mental retardation developing in some patients with multiple Piloleiomyomas has been emerging as an intriguing matter for analysis by the scientists. In this case report, a mentally retarded patient with Piloleiomyoma is described, who, besides the characteristic smooth and dome-shaped lesions on the anterolateral aspect of the dorsum of the right foot, had developed crusting on one of the largest lesions. The histopathological features were consistent with Piloleiomyoma. The occurrence of Piloleiomyoma in a mentally retarded child and its unusual crusted nature has been rarely reported. The association between Piloleiomyoma and mental retardation is further stressed in this case report.     

Multifocal Annular Tufted Angioma: An Uncommon Clinical Entity

Tufted angioma (TA) is a localized benign hamartomatous vascular proliferation usually presenting in the childhood as an erythematous plaque. We report here a rare case of multifocal TA in an 8-year-old boy who presented which two large annular lesions as well as multiple papules and nodules on the back for the duration of 4 years. Histology showed typical well circumscribed poorly canalized vascular lobules with ‘cannon ball’ configuration.     

Huge Nevus Lipomatosus Cutaneous Superficialis on Back: An Unusual Presentation

Nevus lipomatosus cutaneous superficialis (NLCS) is a benign dermatosis, histologically characterized by the presence of mature ectopic adipocytes in the dermis. We hereby report a case of a 10-year-old boy who presented with multiple huge swellings on the scapular regions and lower back. The lesions were surmounted by small papules, along with peau-d orange appearance at places. Histology showed features consistent with NLCS. The case is being reported for the unusual clinical presentation.     

Leiomyoma cutis: a clinicopathological series of 37 cases

Background:

Cutaneous leiomyomas are benign smooth muscle tumors that comprise three distinct types such as piloleimyoma, angioleiomyoma, and genital leiomyoma.

Aim:

The objective of this study was to report a series of cases seen in last 8 years in a tertiary care hospital in north India and to discuss their clinicopathologic findings.

Material and Methods:

Paraffin-embedded blocks of cases reported as cutaneous leiomyoma from 1999 to 2007 were retrieved from the Institute of Pathology, New Delhi, and their clinical parameters were noted. Their histopathological features were reviewed on hematoxylin-eosin stained slides. Immunohistochemistry was performed where necessary.

Results:

Twenty-seven cases of piloleiomyoma, three cases of angioleiomyoma, five breast leiomyomas, and two scrotal leiomyomas were seen in patients ranging from 21 to 65 years of age, with an average of 38.2 years at presentation. There was a male predominance with 26 males and 11 females (M:F = 2.2:1). Solitary lesions (n = 21) were more common than multiple ( n = 16). The trunk and upper limbs were involved most commonly, comprising 23 of 37 (62.2%) cases. This was followed by lower limb, face, breast, and scrotum.

Conclusion:

Cutaneous leiomyomas are rare lesions and form an important clinical differential diagnosis of painful papulonodules. These must be biopsied in order to differentiate them from other spindle cell lesions.     

Blastic plasmacytoid dendritic cell neoplasm presenting as fever with diffuse cutaneous nodules

A young man, presented with high-grade fever and disseminated asymptomatic skin lesions of 6-weeks duration. Cutaneous examination revealed multiple infiltrated monomorphic skin-colored papules and nodules upto 2×2 cm all over scalp, face, trunk and extremities. Light microscopy of nodules showed diffuse infiltration of dermis and subcutis by a tumor composed of medium to large cells with round to ovoid nuclei with fine chromatin, few with visible nucleoli and scanty to moderate amounts of eosinophilic cytoplasm. Tumor cells were positive for CD4, CD8, CD56 and negative for CD30, terminal deoxynucleotidyl transferase and Alk-1. Excised axillary lymph node showed similar morphologic and immunohistochemical findings. There was bone marrow involvement with infiltrate of large atypical/immature lymphoid cells. Diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. This is a rare neoplasm. presenting commonly in the skin, with or without concurrent extracutaneous disease.     

Primary Systemic Amyloidosis with Unusual Dermatological Manifestations: A Rare Case Report

Amyloidosis is a group of heterogeneous diseases characterized by pathological deposition of proteinaceous substance extracellularly in various tissues. The clinical presentation depends on the site of amyloid deposition, with predominant involvement of mesenchymal elements and cutaneous findings in 30–40% of patients in case of primary systemic amyloidosis. We present a case of idiopathic primary systemic amyloidosis presenting with an unusual finding of nodulo-ulcerative lesion over tongue along with multiple skin-colored nodules, mimicking squamous cell carcinoma of tongue with secondary cutaneous metastasis, as well as lacking the classical presentation of purpura, macroglossia, waxy papules, and plaques.     

Disseminated cutaneous histoplasmosis in an immunocompetent adult

Histoplasmosis, a systemic mycosis caused by the dimorphic fungus Histoplasma capsulatum var capsulatum and Histoplasma capsulatum var duboisii is endemic to many parts of the world. The clinical manifestations range from acute or chronic pulmonary infection to a progressive disseminated disease. After initial exposure to the fungus, the infection is self-limited and restricted to the lungs in 99% of healthy individuals. The remaining 1%, however, progress to either disseminated or chronic disease involving the lungs, liver, spleen, lymph nodes, bone marrow or rarely, the skin and mucous membranes. Mucocutaneous histoplasmosis is frequently reported in patients with acquired immune deficiency syndrome (AIDS), but it is rare in immunocompetent hosts. A 60-year-old male presented with asymptomatic swelling of the hard palate and crusted papules and nodules over the extremities, face and trunk. Clinically, the diagnoses of cutaneous cryptococcosis versus histoplasmosis was considered in this patient. A chest X-ray revealed hilar lymphadenopathy. Enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was nonreactive. Skin biopsy revealed multiple tiny intracellular round yeast forms with a halo in the mid-dermis. Culture of the skin biopsy in Sabouraud's dextrose agar showed colonies of Histoplasma capsulatum. Despite an investigation including no evidence of underlying immunosuppression was found, he was started on IV amphotericin-B (0.5 mg/kg/day). However, the patient succumbed to his disease 2 days after presentation. We report a rare case of disseminated cutaneous histoplasmosis in an immunocompetent individual.     

Acquired multifocal tufted angiomas in an immunocompetent young adult

Tufted angioma (TA) is a rare benign vascular neoplasm, localized to the skin and subcutaneous tissues, occurring primarily on the trunk and extremities of children. The lesions are usually asymptomatic but, rarely, paroxysmal painful episodes may be associated. The occurrence of eruptive TA is still rarer and had been described almost exclusively in association with immunocompromised states. We report here a case of acquired painful multifocal tufted angiomas on the face and neck in an immunocompetent young adult.     

Multiple Familial Trichoepithelioma with an Adjacent Basal Cell Carcinoma,Transformation or Collision – A Case Report and Review of Literature

Trichoepithelioma is a benign tumor of follicular origin that presents as small, skin-colored papules predominantly on the face. When more than one family member is affected, the disease is known as multiple familial trichoepithelioma (MFT). It is a rare autosomal dominant skin disease. Malignant transformation is very rare. We describe here a case that developed malignant neoplasm in a setting of multiple trichoepithelioma.     

Clinical Presentation and Evaluation of Dermatomyositis

Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50–70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron''s papules, Gottron''s sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, “mechanic''s hands”, palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35–40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.     

Congenital cutaneous candidiasis: a rare and unpredictable disease

Congenital cutaneous candidiasis (CCC) is an extremely rare disorder that presents within the first 6 days of life. The manifestations ranges from diffuse skin eruption without any systemic symptoms to respiratory distress, hepatosplenomegaly, sepsis, and death. We report a neonate who presented with generalized skin eruptions at birth, characterized by erythematous macules and papules. The eruption involved head, face, neck, trunk, and extremities. Candida albicans was demonstrated on direct KOH smear, skin biopsy. The disease implies a congenital intrauterine infection and is different from neonatal candidiasis, which manifests as thrush or diaper dermatitis. The infection is acquired from the maternal genital tract in an ascending fashion. Clinical features, direct smear examination of specimen, and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period. Topical antifungal therapy is sufficient unless systemic candidiasis is present. Prognosis for congenital cutaneous candidiasis is good.