Anusree Gangopadhyay

Cutaneous manifestations of mixed connective tissue disease: Study from a tertiary care hospital in Eastern India

Context: Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe. Aims: This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe. Settings and Design: Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years. Materials and Methods: The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients. Statistical Analysis Used: SPSS (Version 17) and MedCalc (Version 11.6). Results: The Male: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynauds phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this disease Conclusions: Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease.

Phakomatosis pigmentovascularis presenting with Sturge-Weber syndrome and Klippel-Trenaunay syndrome

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype ‘a’ present only with cutaneous form and subtype ‘b’ also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

Zosteriform palisaded encapsulated neuroma: An unusual presentation

Palisaded encapsulated neuroma (PEN) is an infrequent benign cutaneous neural tumor, which usually presents as solitary, asymptomatic, papule or nodule, often localized on the faces, around the orifices, in middle age with no sex preponderance. Rarely, it can be multiple. Both solitary and multiple lesions are not associated with neurofibromatosis or multiple endocrine neoplasica syndrome type 2B (MEN 2B). We hereby report, a rare case of multiple cutaneous PEN in a 30-year-old female who presented with multiple asymptomatic soft-to-firm papules and nodules in a dermatomal distribution on the face along the supply of the ophthalmic division of the trigeminal nerve with no signs of neurofibromatosis or MEN 2B.

Eccrine angiomatous naevus revisited.

Hamartomatous, circumscribed swellings of the extremities make an interesting study. Presentations are manifold and the naevi are not always present from birth. Excessive growth of hairs leads to remarkable appearance of such swellings. A young woman presented to the dermatology department, complaining of tenderness over 4th finger of her left hand. The defect was present for the last couple of years and was typified by visible hyperhidrosis on gentle tapping. Counseling of the affected lady made her agree for a skin biopsy. The histopathology revealed it to be of the nature of eccrine angiomatous hamartoma. Blood vessels were scarce. Eccrine ducts were plentiful without other associated anomalies. The deformity was removed by simple excision with good result.

A clinical rating scale for the assessment of facial aging in Indian population.

Background: Estimation of facial aging has assumed growing importance due to the advent of several antiaging therapies. Evidence-based estimation of global facial aging is often necessary, especially for validation of these treatment modalities. Most available methods are expensive and have been used in fair skinned individuals. Aim: We attempted to develop a clinical rating scale for the estimation of global facial aging applied on an Indian population which has brown to black skin. We have also measured the association of this rating scale score with the chronological age. Methods: Initially, a 14- item summated rating scale was developed with inputs from five dermatologists and a clinical pharmacologist. The rating scale was applied to 105 consenting subjects with healthy facial skin between 30 to 90 years of age. Intra- and inter-rater reliability was assessed. Results: The summated rating score showed a significant positive correlation with the chronological age (Pearson′s correlation coefficient 0.834, P < 0.001). We omitted one item from the scale due to a low inter-rater agreement. The resulting 13-item rating scale was internally consistent (Cronbach′s alpha: 0.905), with substantial inter- and intra-rater reliability (intraclass correlation coefficient: 0.973 and 0.788, respectively). Principal components and predictive equation for perceptible age were identified on further computation. Limitations: Participants of this study were limited to a particular ethnic group from West Bengal and other neighboring states of Eastern India. Conclusions: We have developed and validated a 13-item rating scale for the quantification of global facial aging suitable for Indian (brown to black) skin type. This scale can be utilized effectively for clinical estimation of global facial aging.

Epidermoid cyst containing molluscum bodies in a 12-year-old Indian boy: A chance phenomenon or inducer?

Indian Journal of Paediatric Dermatology | Volume 18 | Issue 2 | April-June 2017 140 or subcutaneous nodule with a surface punctum through which cheesy material can be expressed. It commonly affects adult men or women. Chest, back, head and neck region are the most common sites of involvement.[2] On the other hand, MC is a viral infection caused by a poxvirus, subgenus molluscipox virus (MCV), which comprises four genetically distinct viral subtypes. It is characterized by shiny, pearly white, hemispherical, umbilicated papule over skin and mucosa. MC is more common among children. It is common in the head and neck region, followed by flexural areas and the genitalia.[5] Extensive lesions can occur in immunocompromised patients, especially in HIV, patients receiving immunosuppressive therapy, and in atopics.[5,6] However, our patient was neither atopic nor had history of use of any topical or systemic medication. After entering through the basal epidermis, the MCV starts cellular proliferation and forms lobulated epidermal growths which further compress the papillae and, thus, fibrous septae develop between the lobules. Ultimately, cells at the center of the lesion get destroyed and appear as large hyaline bodies containing eosinophilic, cytoplasmic masses of virus material.[5] Spontaneous regression usually occurs within 1 year due to cell‐mediated immune response. Antibody to the virus is present in almost 60% of patients, which suggest the role of humoral immunity Sir, A 12‐year‐old otherwise healthy boy presented to us with an untreated asymptomatic raised lesion on his shoulder since last 2 years. Initially, the lesion was small which gradually increased to attain the present size. There was no history of atopy. No similar history was present in the family. Cutaneous examination revealed a soft to firm, nontender sessile nodule of 1.5 cm diameter on his shoulder [Figure 1a]. The lesion was free from underlying structures. There was no erythema, scaling, or erosion on the surface of the lesion. However, a punctum was seen over the nodule. Rest of the cutaneous or systemic examination was noncontributory. Routine blood examinations were within normal limits. Screening for HIV was negative. Histopathological examination (HPE) of the excised nodule, stained by hematoxylin and eosin [Figure 1b‐d], revealed multiple big to small cystic structures in the dermis. The overlying epidermis was normal with irregular proliferation of rete pegs. The cysts were lined by single‐layered granular cells of surface epithelium and contained keratin debris, hair shaft remnants, and eosinophilic globular structures suggestive of molluscum bodies. Molluscum bodies were also seen in the walls of the lining epithelium. There was a sparse inflammatory cell infiltrate consisting of eosinophils, lymphocytes, and histiocytes surrounding the cystic structures. Based on the clinical with HPE findings, a diagnosis of epidermoid cyst (EC) containing molluscum bodies was made.

Generalized bullous drug eruption to faropenem - hitherto unreported

References 1. Daoud MS, Pittelkow MR. Lichen planus. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. p. 296-312. 2. Weedon D. Weedon’s Skin Pathology. 3rd ed. London: Churchill Livingstone Elsevier; 2010. 3. Hanau D, Sengel D. Perforating lichen planus. J Cutan Pathol 1984;11:176-8. 4. Gutte R, Khopkar U. Perforating lichen planus. Indian J Dermatol Venereol Leprol 2011;77:515-7. 5. Gutte R, Khopkar U. Predominant palmoplantar lichen planus: A diagnostic challenge. Indian J Dermatol 2014;59:343-7.

Darier's disease in gastric malignancy: An unusual paraneoplastic phenomenon

Dariers disease is an autosomal dominant genodermatosis resulting from ATP2A2 gene mutation. A 62-year-old male presented at our outpatient (OPD) with sudden-onset numerous dirty, warty papules over the head, neck, and back since 2 months. Histopathology of the skin lesions revealed acantholytic dyskeratosis suggestive of Dariers disease. He was referred to the gastroenterology department for some gastrointestinal (GI) symptoms where he was diagnosed with adenocarcinoma of the stomach and was subsequently operated. On his next visit to our department for follow-up, we found a marked diminution of the skin lesions in the absence of any specific treatment. In view of the above finding, we concluded that paraneoplastic dermatosis in the form of Dariers disease occurred in this patient. Paraneoplastic Dariers disease with gastric adenocarcinoma is not yet described in dermatology literature and is hence reported here.