Cerebriform Cutaneous Lesions in Pemphigus Vegetans

Pemphigus vegetans is an autoimmune bullous disorder characterized by vegetating lesions commonly over the flexures. A 42-year-old female patient came with pemphigus vegetans presenting with interesting cerebriform morphology of the cutaneous lesions over the flexures. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue is a well-known sign seen in pemphigus vegetans. Interestingly, we noticed the typical sulci and gyri pattern in the skin lesions of pemphigus vegetans over the flexures of the body. This clinical sign can be used as a clue in the diagnosis of pemphigus vegetans. Morphology and physical characteristics are important for the diagnosis of the disease. Clinical signs always give a clue to the probable or possible diagnosis in most of the dermatological conditions.     

Long-lasting Localized Pemphigus Vulgaris without Detectable Serum Autoantibodies Against Desmoglein 3 and Desmoglein 1

Pemphigus vulgaris (PV) is an autoimmune blistering disease elicited by anti-desmoglein (DsG) 3 antibody. Although skin lesions tend to be distributed over the entire body, in some patients, they are confined to a restricted area. We report two patients who presented with long-lasting localized PV without detectable anti-DsG antibodies after suffering antibody-positive systemic PV. Initial treatment with prednisolone (PSL) was successful in both patients, but a local relapse occurred on the cheek or lower lip after a reduction in the PSL dose. Biopsy of the localized lesions showed suprabasal acantholysis; no serum DsG antibodies were found. Local immunosuppression therapy was effective in both patients. Based on our findings, we suggest that localized PV without detectable antibodies can develop after systemic PV.     

Pemphigus Foliaceus Masquerading as IgA Pemphigus and Responding to Dapsone

A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.     

Pemphigus Vegetans Involving the Esophagus

A 44-year-old man with pemphigus vegetans had severe odynophagia. He received an endoscopic examination for esophageal involvement. Many white plaque-like lesions with an erythematous base were seen on the esophageal mucosa. Biopsy from the mucosal epithelial layer showed rounded epidermal cells with large nuclei and numerous inflammatory cells including eosinophils.     

A Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence

Background:Pemphigus is a group of chronic autoimmune vesico-bullous disorders in which the epidermis and the basement membrane zone are the focus of attack resulting in cutaneous and mucosal blister formation. Direct immunofluorescence (DIF) test is a very sensitive test for the diagnosisAim:To study the clinico histopathological patterns of pemphigus in eastern India. The study also aims to correlate DIF with clinical and histologic findings as well as severity of skin involvement [scoring systems].Results:In our study Pemphigus vulgaris (PV) was the predominant type with 32 cases followed by 8 cases of pemphigus foliaceus (PF) and a single case of IgA pemphigus. Mean age at presentation was late middle age. Majority of the patients, 26 (63.41%) initially had cutaneous involvement followed by mucosal involvement. In this study group 36 (87.80%) patients showed acantholytic cells on histopathological examination. Most patients of PV showed suprabasal blister 20 (62.50%) followed by intraspinous 5 (15.62%) and subcorneal 5 (15.62%) blister. In majority 28 (87.50%) of the PV patients IgG and C3 antibodies were deposited throughout the epidermis. The strength of antibody positivity was strong in most of the patients (71.87%). In cases of PF mostly IgG 6 (75%) antibodies were deposited in the upper epidermis. DIF intensity had poor correlation with disease activity/severity except in PF.Conclusion:Almost 85.36% cases of pemphigus were diagnosed clinicopathologically. But 6 cases couldn’t be diagnosed accurately on clinicopathological basis and in them DIF was confirmatory. Two cases of pure mucosal PV and 1 case of IgA pemphigus was confirmed by DIF. Two cases of bullous pemphigoid clinico-histologically mimicking PV were also excluded by DIF. So it appears from our study that DIF is confirmatory for diagnosis of pemphigus in all cases.     

Serum Angiotensin Converting Enzyme in Pemphigus Vulgaris

Background:

Pemphigus vulgaris is an autoimmune blistering skin disease with unknown etiology. Drugs such as angiotensin-converting enzyme (ACE) inhibitors may contribute in the pathogenesis of pemphigus.

Objective:

We plan this essay to evaluate the serum ACE level in pemphigus vulgaris patients in comparison with healthy controls to recognize its possible role in disease pathogenesis or activity.

Methods:

This study was planned and performed in the dermatology clinics of Shahid Beheshti University of MedicalSciences’ Hospitals between July 2010 and June 2011. Patients with new onset of pemphigus vulgaris were enrolled in our study. Control subjects were frequency-matched to cases by sex and age. Serum ACE was determined by the spectrophotometric method.

Results:

Thirty-four patients with pemphigus vulgaris and 35 healthy individuals were recruited in the study. No statistical significant difference was detected in the mean level of serum ACE of the two groups (t-test, P = 0.11). The mean ACE level was significantly lower in male patients compared with male controls (P = 0.04). Moreover, a significant higher serum ACE level of patients with cutaneous involvement was observed compared to patients with mucosal involvement (P = 0.02).

Conclusions:

Despite lack of any significant difference of serum ACE level between pemphigus and control group, the serum ACE level was considerably lower in male pemphigus vulgaris patients compared with male controls. Therefore, ACE might have some association with pemphigus vulgaris especially in male patients; however, further studies are required to confirm this association.     

副肿瘤性天疱疮合并局灶性Castleman''s病和呼吸系统损害

副肿瘤性天疱疮（PNP）是一种与自身免疫相关的获得性大疱性疾病。本文报告4例同时合并局灶性Castleman‘s病和呼吸系统损害的PNP患者，其中3例合并闭塞性细支气管炎（BO）。他们的免疫学表现较为一致，血清中抗体可识别人角质形成细胞及边缘肺组织提取物中的210kD和190kD抗原。肿瘤切除后患者的皮肤逐渐消失，3例合并BO的患者仍有喘憋及咳嗽。     

Asymptomatic Papulo-nodules Localized to One Finger

Subcutaneous or deep granuloma annulare is a benign asymptomatic condition characterized by firm asymptomatic nodules in deep subcutaneous tissues that may be associated with intradermal lesions. A 53-year-old female presented with asymptomatic skin-colored, firm nodules over the right ring finger. Histopathology revealed a palisading granuloma with central degenerated collagen and mucin deposition in the dermis suggestive of granuloma annulare. Isolated and unilateral involvement of a single digit with clusters of nodules of subcutaneous granuloma annulare (GA) in an adult is rare and differentiation from its simulator rheumatoid nodule is essential.     

Acquired Bilateral Nevus of ota-like Macules with Mucosal Involvement: A New Variant of Hori's Nevus

Acquired bilateral nevus of Ota-like macules (ABNOM) or Hori''s nevus, a rare form of acquired dermal melanocytoses, presents as bilateral facial blue-gray macules without ocular or mucosal involvement. This condition is mostly found in women of Asian descent and usually appears in the fourth or fifth decade of life. Pathogenesis is unknown, though few theories have been proposed. Effective treatment has been found to be achieved with pigment-specific lasers. Herein, we report a case of Hori''s nevus with mucosal involvement. A 42-year-old male patient, presented to us with blue-gray discoloration on either side of his face, both eyes, and in the mouth since the age of one year. Histopathological examination showed clusters and singly dispersed pigmented melanocytes within the upper and mid-dermis regions. Special staining of melanocytes using Masson-Fontana stain was positive. Diagnosis of Hori''s nevus was made by correlating clinical and histopathological findings. Patient was informed of his treatment options, but refused treatment. A similar case of Hori''s nevus with mucosal involvement has not been reported so far.     

Pulse Therapy in Pemphigus: Ready Reckoner

Pulse therapy for the treatment of pemphigus has been in vogue for several years and is administered by many dermatologists across the world. However, even though there is enough evidence about its efficacy and methodology, there continue to be doubts and questions regarding the rationale of use of high dose intravenous steroids and steroid-sparing immunosuppressants. This article has aimed to provide clarity to young dermatology residents on the administration of pulse therapy, and the various controversies and modifications that have been mentioned in literature over the past couple of years.     

Pathogenesis of dermatophytoses

Dermatophytes can survive solely on outer cornified layers of the skin. The ability of certain fungi to adhere to particular host arises from numerous mechanisms and host factors, including the ability to adapt to the human body. Natural infection is acquired by the deposition of viable arthrospores or hyphae on the surface of the susceptible individual. After the inoculation in the host skin, suitable conditions favor the infection to progress through the stages of adherence and penetration. Development of host response is mostly by a T-cell mediated response of delayed-type hypersensitivity. Antibody formation does not seem to be protective. Natural defenses against dermatophytes depend on both immunological and nonimmunological mechanisms.     

A Clinical and Mycological Study of Dermatophytic Infections

Background:

Dermatophytoses refer to superficial fungal infection of keratinized tissues caused by keratinophilic dermatophytes. According to observations worldwide, dermatophytoses are the most common of the superficial fungal infections. It is common in tropics and may present in epidemic proportions in areas with high rates of humidity. Although common, the precise size of the problem defies measurement.

Aims:

The present study was undertaken to assess the clinical profile of dermatophytic infection and to identify the species of fungi that are prevalent in this region.

Materials and Methods:

A total of 100 patients clinically suspected for dermatophytoses were selected for the study. Direct microscopy in 10% potassium hydroxide (KOH) and culture was done in each case.

Results:

Out of 100 patients, the maximum were seen in the age groups of 16-30 years. Tinea corporis was the most common clinical type (44.3%) followed by tinea cruris (38.2%). Overall positivity by culture was 39% and by direct microscopy 96%.

Conclusion:

Trichophyton rubrum was the predominant species isolated (67.5%) in all clinical types followed by Trichophyton mentagrophytes. Culturing the fungus may identify the species, but it is not essential for the diagnosis as it is not a sensitive test.     

PYODERMA VEGETANS WITH lg A DEFICIENCY

A 41-year-old male with known congenital IgA deficiency developed pyoderma vegetans during a subtropical holiday. He responded well to topical treatment with aluminium subacetate.     

Diffuse Cutaneous Mastocytosis with Bullous Lesions and Pulmonary Involvement: A Rare Case

Mastocytosis is defined as a heterogeneous group of disorders characterized by an accumulation of mast cells in one or more organs, particularly in the skin, bone marrow, liver, spleen and lymph nodes. However here we describe an 11-month-old girl child presented with diffuse cutaneous mastocytosis with bullous lesion, having pulmonary involvement, which is very rare and there is no available case report from India.     

Non Comparative Study on Various Pulse Regimens (DCP,DAP and DMP) in Pemphigus: Our Experience

Background:

Pemphigus has been treated with Dexamethasone Cyclophosphamide Pulse (DCP) Therapy since 1981. Various modifications have been suggested in the original regimen. These include Dexamethasone Azathioprine Pulse (DAP) and Dexamethasone Methotrexate Pulse (DMP) therapies.

Aims:

To report our experience on the noncomparative study of various Pulse regimens DCP, DAP AND DMP therapies in patients with Pemphigus.

Materials and Methods:

The patients were put on three regimens depending upon the situation-Conventional DCP, DAP in the reproductive age group, DMP in patients who showed prolonged Phase I more than 12 months while on DCP.

Results:

30 patients were put on DCP therapy. The duration of phase I was on an average six months. Relapse was seen in 3 patients in phase IV. 12 patients on DAP therapy were considered. In Phase III 5 patients relapsed in phase IV four patients relapsed. Five patients were put on the DMP. Disease activity was poorly controlled and in three DMP was discontinued.

Conclusion:

DCP remains the most effective regimen with quickest onset of remission and continuance of remission. In DAP therapy fixation of dose of azathioprine at 50 mgs daily may be counterproductive. DMP does not fulfil the promise of a viable treatment option in recalcitrant pemphigus and this lacunae needs to be plugged.     

A Comparative Effectiveness Research of Azathioprine and Cyclophosphamide on the Clinical and Serological Response in Pemphigus Vulgaris

Context:A prospective study was carried out to examine the efficacy of cyclophosphamide and azathioprine in pemphigus vulgaris.Aims:To compare the clinical and serological effect of azathioprine and cyclophosphamide in pemphigus patients.Results:Azathioprine had a slower onset of action with a statistically significant improvement seen by 6 months (P = 0.016). Cyclophosphamide had a faster onset of action (3 months) though there was no statistical difference in the efficacy between the two at the end of 6 months. The (RonT) was 33.3–44.4% for azathioprine and 28.8–42.9% for cyclophosphamide at 6 months. Though ELISA had a high sensitivity and specificity for diagnosis, as a tool for assessing therapeutic response a significant decrease was seen only till 3 months. This was restricted to Dsg1 for the azathioprine group and both Dsg3 and Dsg1 levels for the cyclophosphamide group. There were two deaths, both in the cyclophosphamide group.Conclusions:Azathiorpine and cyclophosphamide are equally effective for mucosal and cutaneous disease in pemphigus after 6 months of therapy. Dsg ELISA is useful for diagnosis of pemphigus but is not a useful tool for monitoring response to therapy.     

Characteristics of Mixed Type Basal Cell Carcinoma in Comparison to Other BCC Subtypes

Background:

There are limited data exploring the characteristics of mixed type basal cell carcinoma (BCC).

Objectives:

To explore different characteristics of mixed type BCC.

Design:

Cross sectional study.

Materials and Methods:

825 patients with BCC enrolled in this study.

Results:

Among 825 patients, 512 (62%) were male. Three hundred and fifty five (43%) presented with nodular subtype, 267 (32.4%) with mixed subtype, 25 with superficial and the 178 remaining presented with other subtypes. Four hundred and eighty three (58.6%) of the lesions were on the face, 243 (29.5%) on scalp, 52 (6.3%) on ears, 20 (2.4%) on neck, 15 (1.8%) on trunk and 12 (1.4%) on extremities. Anatomic distribution of mixed type was as follows: 137 on face, (51.4%), 100 (37.3%) on scalp, 19 (7%) on ear, 6 (2.1%) on neck, 4 (1.5%) extremity and 1 (0.7%) on trunk, which the difference from non mixed types was statistically significant (P = 0.002). The mean diameter of the mixed types and non mixed type BCCs were significantly different (2.7 ± 2.1 cm vs. 2.2 ± 1.6 cm; P = 0.01. The prevalence of necrosis in mixed type BCC was two times higher than non mixed type BCCs (OR = 2.3, CI 95% 1.3-3.9, P = 0.001). The most frequent combined subtypes were nodular-infiltrative (P < 0.001).

Conclusion:

Mixed type BCC has differences with other BCC subtypes in anatomical distribution and tumor diameter. Indeed, mixed type BCCs are frequently composed of aggressive subtypes than nonaggressive subtypes.     

Mal de Meleda with Lip Involvement: A Report of Two Cases

Mal de Meleda is a rare autosomal recessive transgradient palmoplantar keratoderma characterized by transgradient keratoderma with associated scleroatrophy, nail changes, pseudoainhum around digits and perioral erythema, without a tendency for spontaneous resolution. Involvement of the lip by keratoderma has not been reported in the English literature. Here we present two cases of Mal de Meleda with unusual lip involvement. The first case was a 15-year-old girl, born of second-degree consanguineous marriage, who presented with transgradient palmoplantar keratoderma from 6 months of age, with lichenoid papules and plaques on the elbows and knees, conical tapering of the distal digits, flexion deformity of several fingers, digital constriction, knuckle pads and lip involvement. The second case was a 24-year-old male with transgradient palmoplantar keratoderma since birth. He also had scaly plaques on the extensors of bilateral knees and elbows, knuckle pads, pseudosclerodermatous fingers with conical tapering, digital constrictions at various places with mild flexion deformity and lip involvement. Both patients were otherwise normal without any family history.     

Chromoblastomycosis Associated with Bone and Central Nervous Involvement System in an Immunocompetent Child Caused by Exophiala Spinifera

Chromoblastomycosis is a chronic granulomatous infection of the skin and subcutaneous tissue caused by specific group of dematiaceous fungi. The infection results from traumatic injury and is seen more commonly on feet and lower legs. It is rarely seen in children and metastatic spread to other systems is exceptionally rare. We report a 12-year-old immunocompetent male child diagnosed with chromoblastomycosis on the lower leg, who in a span of few months developed osteomyelitis and left hemiparesis. Fungal culture showed growth of Exophiala spinifera. Child showed good improvement with voriconazole and itraconazole after 1 year of treatment. Skin lesions healed with minimal scarring and his power improved.     

Adult T-cell leukemia/lymphoma: a retroviral malady

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive leukemia/lymphoma of mature T-lymphocytes caused by human T-cell lymphotropic virus type 1 (HTLV-1). At a tertiary healthcare center in South India, a 58-year-old female presented with multiple erythematous, crusted, and umbilicated papules over the body along with cervical lymphadenopathy. The skin biopsy was consistent with cutaneous T-cell lymphoma. Although she responded initially to chemotherapy, the disease relapsed after 3 months, and she developed disseminated infiltrated skin lesions, generalized lymphadenopathy, and leukemia. Due to the unusual clinical findings we did HTLV-1 Enzyme-linked immunosorbent assay (ELISA), which turned out to be positive in high titers. Her mother had died at an early age from a hematological malignancy and her daughter was also found to be seropositive. To the best of our knowledge, this is the first case to be reported from India of the chronic type of ATLL associated with mother-to-child transmission of HTLV-1 in two generations. This case also emphasizes that the chronic type of ATLL can occur in nonendemic areas like India and should be suspected in nonresponding cases of mycosis fungoides. It should be kept in mind that the chronic type often presents without hypercalcemia or the characteristic 'flower cells' in the peripheral smear.