Report of Two Cases of Recurrent Scalp Dermatofibrosarcoma Protuberans and Literature Review

Dermatofibrosarcoma protuberans (DFSP) are rare malignant skin tumor, and scalp DFSP is even lesser than 5% of all DFSP, therefore, being seldom reported. We recently treated two cases of recurrent scalp DFSPs. One was a 38-year-old male, who accept lumpectomy for the first time; however, it recurred 9 months later. We then performed a wide excision resulting in no recurrence in the subsequent 4 years. Another patient was a 26-year-old female, who accept an in situ tumorectomy for the first time, and 2 years later; the recurrent mass became 9 × 9 cm in size. We gave her another operation, but only 3 months later local recurrence appeared. For the both cases, we collected their case histories, intraoperative findings, pathologic detections, and follow-up results, all of which may help the dermatologists to extend knowledge about this rare disease. Moreover, an exhaustive review of the literature is included with emphasis on diagnosis, different diagnosis and treatments.     

Giant melanoacanthoma mimicking malignant melanoma

Melanoacanthoma denotes a rare variant of pigmented seborrheic keratosis. A 65-year-old male farmer had pigmented, verrucous, itchy, highly painful, progressively growing irregularly oval plaque on left side of lower back for the past five years. The indurated lesion, measuring maximum diameter 10 cm × 5 cm, had no discharge, bleeding, ulceration, or associated lymphadenopathy. Dermoscopy showed regular pigmentary network and cribiform pattern of ridges without any feature of malignant melanoma. Histopathology showed well-defined islands of basaloid cells interspersed with large and richly dendritic melanocytes. The lesion was totally excised followed by skin grafting. Our patient was unique in its massive size and clinical resemblance with malignant melanoma. The diagnosis was confirmed by dermoscopy and skin biopsy.     

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.     

A study on the management of hidradenitis suppurativa with retinoids and surgical excision

Background:

Hidradenitis suppurativa is a chronic skin condition involving the apocrine glandular zones. Affected patients may present with acute abscesses, but the condition often progresses to a chronic state with persistent pain, sepsis, sinus tract, fistula formation, purulent discharge, and dermal scarring. The treatment of patients with severe disease can be difficult and may require complex surgical intervention.

Materials and Methods:

For this study, we selected 30 patients from the outpatient department. The patients were divided into two groups of 15 patients each. In patients of group I, oral acitretin 0.5 mg/kg body weight was given alone. Oral acitretin was given for a period of 12 weeks, and follow-up of the patients was done every 4 weeks for a period of 6 months. In patients of group II, oral acitretin 0.5 mg/kg was given plus a wide surgical excision was done.

Results and Discussion:

In our study, the commonest site of involvement of hidradenitis suppurativa was axilla in 83.3% patients, perineum was involved in 13.3% patients, and periumbilical involvement was seen in 3.3% patients. The commonest clinical feature was nodules seen in 90% patients; pain was seen in 60% patients, dermal scarring in 73.3% patients, malodorous discharge in 33.3% patients, abscess in 30% patients, and fistulous tracts were seen in 20% patients. The recurrence rate was low (20%) in group II patients in whom oral acitretin was given plus surgical excision was done as compared with group I (40%) in whom oral acitretin was given alone.     

SCALP MENINGIOMA

Primary extracranial meningiomas occur very rarely. We present a rare case of extracranial meningioma of the transitional variant which was excised satisfactorily. There was no suggestion of any connection to the intracranial compartment or cranial nerves. The underlying galea was uninvolved, suggesting the true extracranial nature of this tumour. This rare diagnosis should nonetheless be kept in the differential diagnosis of scalp tumors.     

Nevus Comedonicus on Scalp: A Rare Site

Nevus comedonicus is rare hamartoma of the pilosebaceous unit. Curiously the scalp is rarely involved. Here we are reporting 33-year-old male presenting with nevus comedonicus arranged linearly on the scalp. There was positive family history of similar lesion on the similar site. This case has been presented for its sheer rarity and atypical site.     

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.     

Primary hydatid cyst in the soft tissue of the face: an exceptional occurrence

To emphasize that solitary hydatid cyst can be localized in the soft tissue and present as a soft tissue mass even in an unusual site like face, we report the case of a 42-year-old male patient presenting with a slowly growing mass in right temporal region. Computed tomography (CT) scan showed an encapsulated mass with multiple cysts. Histopathological examination revealed the characteristic findings, which were consistent with soft-tissue hydatid disease. In the absence of visceral organ involvement, this is the first reported case of a primary subcutaneous hydatid cyst in the skin of face in India. In the English literature, only one case of this kind has been reported till date. When imaging methods confirm cystic nature of a swelling, even in unusual sites, one should always keep a possibility of hydatid cyst and manage accordingly during surgery to avoid precipitation of acute anaphylaxis.     

Twelve Years’ Observation of Multiple Familial Trichoepithelioma with Squamous Carcinoma

Long-standing trichoepithelioma lesion on scalp with 12 years follow-up. The patient was observed for 12 years, and the carcinoma recurred 3 times during 8 years after surgical excision and radiation therapy. Malignant transformation may occur in multiple familial trichoepitheliomas; Moderate radiation therapy should be given for malignant transformation patients with lower radiation dose and shorter time. Removing the trichoepithelioma as soon as possible if the tumor suddenly or continuously increased is recommended.     

Multiple Familial Trichoepithelioma with Malignant Transformation

Trichoepithelioma (TE) 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 (AD) skin disease. Malignant transformation is very rare. We present a case of MFT in a female patient and her father with malignant transformation to basal cell carcinoma (BCC) in the father. We summarized the main histological differential parameters between TE and BCC and applied immunophenotyping for both by administration of Bcl2, CD34, CD10 and androgen receptor (AR) antibodies.     

A clinical and histopathological profile of patients with cutaneous tuberculosis

Extrapulmonary tuberculosis constitutes about 10% of all cases of tuberculosis, and cutaneous tuberculosis makes up only a small proportion of these cases. Despite prevention programs, tuberculosis is still progressing endemically in developing countries. Commonest clinical variant of cutaneous tuberculosis in our study was lupus vulgaris seen in 55% patients followed by scrufuloderma seen in 25% patients followed by orificial tuberculosis, tuberculosis verrucosa cutis, papulonecrotic tuberculid, and erythema induratum seen in 5% each. The commonest site of involvement was limbs seen in 50% patients followed by neck seen in 25% patients, face in 15%, and trunk in 10% patients. Maximum percentage of patients (55%) had duration of cutaneous tuberculosis between 6-12 months followed by 35% between 13-24 months, 5% had duration of cutaneous tuberculosis less than 6 months, and the rest 5% had duration more than 24 months. The commonest histopathological feature in our study was tuberculoid granuloma with epitheloid cell and Langhans giant cells seen in 70% patients, hyperkeratosis was seen in 15% patients and AFB bacilli were seen in 5% patients.     

Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.     

CLEAR CELL SARCOMA: A CASE MIMICKING PRIMARY CUTANEOUS MALIGNANT MELANOMA

Clear cell sarcoma (CCS) is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22) (q13;q12) has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.     

Poroma with Sebaceous Differentiation: Dermoscopy for the Diagnosis of Skin Tumor with Sebaceous Differentiation

Although divergent adnexal differentiations are occasionally seen in poroma, poroma with sebaceous differentiation is extremely rare. We present here the second case of dermoscopy on poroma with sebaceous differentiation. A 38-year-old Japanese female presented with a 2-year history of a slow-growing nodule on her left forearm. Dermoscopically, fine hairpin-like vessels, beige lobular structures were seen in the nodule. Many small yellow dots were scattered between beige lobular structures, giving orange-beige in color as a whole. On the basis of histopathologic findings, a diagnosis of poroma with sebaceous differentiation was made. Some sebaceous tumors are known to exhibit yellowish structures on dermoscopy. Tumors with sebaceous differentiation, as well as conventional sebaceous tumors, can show yellow structures on dermoscopy.     

Elephantine Psoriasis with Papillomatosis and Alternating Hypogranulosis and Hypergranulosis

Psoriasis is a disease of considerable clinical and histopathological diversity. We report a rare case of elephantine psoriasis responding very well to methotrexate. Histopathology revealed abnormal papillomatosis with finger-like projections in addition to alternating orthokeratosis with overlying hypergranulosis and parakeratosis with overlying hypogranulosis. We believe that this finding may represent an odd histopathologic type in elephantine psoriasis.     

Screening for Skin Cancer: A Pilot Study in Tehran,Iran

Background:

Early detection of skin cancers by screening could be very beneficial to decrease their morbidity or mortality. There is limited study about skin cancer screening in Iran.

Aim:

This essay was planned as a pilot skin cancer screening campaign in Tehran, Iran to evaluate its profit and failure and further design large-scale screening program more definitely.

Materials and Methods:

Thirty one public health centers of Shahid Beheshti Medical University were selected in different areas of Tehran. The project was announced via media and invited all the people above 40 years old to come for the whole-body skin examination in a one-week period. Patients with any suspected lesions were referred to the dermatology clinics of the university.

Results:

1314 patients, 194 males (14.8%) and 120 females (85.2%), with mean age of 51.81 ± 10.28 years participated in this screening campaign. Physicians found suspected lesions in 182 (13.85%) of participants. The diagnosis of skin cancer was confirmed in 15 (1.14%) patients. These malignancies included 10 (0.76%) cases of basal cell carcinoma, 2 (0.15%) cases of squamous cell carcinoma and 3 (0.23%) cases of malignant melanoma.

Conclusion:

Skin cancer screening seems to be valuable to detect skin malignancies in their early course. Regarding the considerable amount of facilities needed to perform skin cancer screening program, it might be more beneficial to perform the targeted screening programs for the high-risk groups or emphasis more on public education of skin cancer risk factors and their early signs.     

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.     

Malignant Eccrine Poroma of the Vulva: An Intriguing Case of a Rare Tumor at an Unusual Site

Malignant eccrine poroma is a rare malignancy of the eccrine sweat glands, occurring most frequently on the lower extremities. It affects both sexes equally usually in the 6^th to 7^th decade of life. Metastasis to regional lymph nodes may occur in 20% that may be fatal in 60% cases. Its aggressive nature, rarity of occurrence, and unusual presentations make it very important to be evaluated properly by the clinician. We hereby report a case of a 75-year-old female presenting with two exophytic tumors over her vulva with local extension. On histopathological examination, it was diagnosed as malignant eccrine poroma. On magnetic resonance imaging of the pelvic region, metastatic extension in regional lymph nodes was found. She was treated by radical vulvectomy with bilateral inguinal and femoral lymph node dissection followed by radiotherapy.     

Severity of Psoriasis Among Adult Males is Associated with Smoking,Not with Alcohol Use

Context:

Lifestyle factors such as tobacco smoking and alcohol use can affect the presentation and course of psoriasis. There is a paucity of data on this subject from India.

Aims:

To find out whether increased severity of psoriasis in adult Indian males is associated with tobacco smoking and alcohol use.

Settings and Design:

Cross-sectional study in the Department of Dermatology of a Tertiary Care Teaching Hospital.

Subjects and Methods:

Male patients above 18 years of age attending a psoriasis clinic between March 2007 and May 2009 were studied. Severity of psoriasis (measured using Psoriasis Area and Severity Index – PASI) among smokers and non-smokers was compared. We also studied the correlation between severity of psoriasis and nicotine dependence (measured using Fagerström Test for Nicotine Dependence) and alcohol use disorders (measured using Alcohol Use Disorders Identification Test–AUDIT).

Statistical Analysis:

Z-test, Odd''s ratio, Chi-square test, Spearman''s correlation coefficient.

Results:

Of a total of 338 patients, 148 were smokers and 173 used to consume alcohol. Mean PASI score of smokers was more than that of non-smokers (Z-test, z = −2.617, P = 0.009). Those with severe psoriasis were more likely to be smokers (χ² = 5.47, P = 0.02, OR = 1.8, Confidence Interval 1.09-2.962). There was a significant correlation between PASI scores and Fagerström score (Spearman''s correlation coefficient = 0.164, P < 0.01). Mean PASI scores of persons who used to consume alcohol and those who did not were comparable.(Z-test, z = −0.458, P = 0.647). There was no association between severity of psoriasis and alcohol consumption.(χ² = 0.255, P = 0.613, Odds Ratio = 1.14, CI 0.696-1.866). There was no correlation between PASI scores and AUDIT scores (Spearman''s correlation coefficient = 0.024, P > 0.05).

Conclusions:

Increased severity of psoriasis among adult males is associated with tobacco smoking, but not with alcohol use.     

Lethal systemic degos disease with prominent cardio-pulmonary involvement

A 48-year-old man presented with acute abdominal pain underwent laparotomy that revealed two perforated ulcers in jejunum. He had skin lesions with porcelain white atrophic scar which were ignored for 3 years, whereas the disease revealed own malignant nature and progressed to nervous, gastrointestinal, and cardiopulmonary systems. The diagnosis of Degos disease was established on the basis of clinical and histopathological features. He expired due to cardio-pulmonary insufficiency after 5 months from the onset of systemic involvement. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs. Distribution of skin lesions that involved palmoplantar surfaces, genitalia and scalp and, furthermore, course of disease as rapid progressive cardio-polmunary involvement are remarkable point in this patient. On the other hand, this case highlights importance of clinicopathologic correlation, specially in the dermatologic field.