Lupoid cutaneous leishmaniasis: a report of 16 cases.

BACKGROUND: Lupoid cutaneous leishmaniasis (CL) is known as the chronic form of CL. However, keeping its clinical presentation in view, there is a need to revisit this form of disease. AIMS: To redefine/describe lupoid CL in view of clinical features. METHODS: It was a case series seen in Muzaffarabad (Pakistan) from Jan 2006 to May 2008. All patients clinically suggestive and consistent with laboratory diagnosis of CL were registered. Patients of all age groups and either sex having cutaneous lesions resembling lupus vulgaris or lupus erythematosus on the face or elsewhere were included in the study. Those having chronic fluctuating/relapsing course or scarring within the lesions were also included. Various demographic features of the patients and clinical patterns were recorded. Descriptive statistics were used for analysis. RESULTS: Of 254 registered patients of CL, 16 (6.3%) were diagnosed as lupoid CL. None of the patients had scarred lesions. Age ranged from 38 to 75 (55 + 15.11) years and duration of lesions varied from 4 to 32 (14.25 + 07.59) weeks. All patients had lesions over the face. Thirteen (81.25%) had a large solitary plaque extending over the nose and a large part of the cheeks and three (18.75%) had multiple lesions. Lesions were central/nasal in two (12.5%), unilateral/asymmetrical in four (25%) and bilateral/symmetrical in 10 (62.5%). Morphological patterns included erythematous/infiltrated (7), psoriasiform (6), ulcerated/crusted (2) and Discoid lupus erythematosus (DLE)[G1] like (1). CONCLUSION: Lupoid CL is not strictly a chronic form of disease, which presents on the face from the very onset and shows no scarring or recurrence.

Rhinophymous leishmaniasis: A new variant.

Cutaneous leishmaniasis is known for its wide clinical spectrum. The nose is one of the usual sites where the disease can present in many forms, such as psoriasiform plaques, furunculoid nodules, lupoid plaques, and erysipeloid or mucocutaneous types. We present a new morphology, i.e. rhinophyma-like plaque in an elderly male patient who presented with a large infiltrated plaque involving his nose and the adjoining area of his upper lip. It appeared to be rhinophyma of the nose but was diagnosed as cutaneous leishmaniasis after the demonstration of leishmania parasites in a skin smear preparation; he was treated satisfactorily with antimonials.

Blaschkolinear dermatoses--an overview of interesting patterned dermatological disorders.

A large number of congenital/nevoid and acquired skin diseases on the human skin and mucosae assume a characteristic morphological pattern following the Blaschko lines. This pattern recognition is important for dermatologists so as to have an idea about the etiopathogenesis as well as to categorize various such disorders. This article is an attempt to overview and classify a variety of blaschkoid disorders described so far in the literature.

Miltefosine in cutaneous leishmaniasis.

OBJECTIVE: To determine the efficacy of oral Miltefosine in patients with cutaneous leishmaniasis and its comparison with the most effective standard treatment, pentavalent antimony compound. DESIGN: A non-randomized, open label comparative clinical trial. PLACE AND DURATION OF STUDY: Dermatology Department, Military Hospital, Rawalpindi, Pakistan from March to October 2005. PATIENTS AND METHODS: Thirty patients, 12 years of age or older clinically and histopathologically diagnosed as cutaneous leishmaniasis were selected. Fifteen patients received orally administered Miltefosine 2.5mg/kg/day for 28 days and remaining 15 received injectable pentavalent antimony 20mg/kg/day for 28 days. Pre-treatment complete physical examination was done along with necessary laboratory investigations in all cases. These were repeated again after 2 weeks and at the end of treatment to note any deviation from the normal limits. Groups were almost matched in terms of age, weight, parasitological score. The efficacy was evaluated by ulcer size, before therapy, at 2 weeks and 4 weeks. Patients were followed-up at 3 and 6 months. Efficacy of two groups was statistically compared by calculating p-value by z-test. RESULTS: All patients completed the study without any serious complication. Lesions improved significantly and only scarring and post-inflammatory pigmentation was left. At 3 months, cure rate was 93% in group A and it was 73.33% in group B while at the end of 6 months, it was 86% and 66.6% respectively. This difference between efficacies of two groups was not found to be statistically significant (p-value>0.5). CONCLUSION: Miltefosine appears to be a safe and effective alternative to currently used therapies. The striking advantage of Miltefosine is its oral administration and it may also be helpful in regions where parasites are resistant to current agents.

Unilateral Blaschkoid lichen planus involving the entire half of the body, a unique presentation.

A large number of congenital as well as acquired skin disorders assume a characteristic morphological pattern following the Blaschko lines. Lichen planus (LP) is an acquired inflammatory disorder that can come with different faces. Linear lesions are frequently seen but cases of zonal (zosteriform) or whorled appearance have rarely been described in the literature. We describe an even rarer presentation in a 30-years-old man who had unilateral pruritic violaceous lesions over right half of body characteristically following lines of Blaschko with clinical and histological morphology typical of LP.

Acquired smooth muscle hamartoma.

Smooth muscle hamartoma is an uncommon, usually congenital, cutaneous hyperplasia of the arrectores pilorum muscles. When it is acquired, it may be confused with Becker's nevus. We report a case of this rare tumor in a 19-year-old man. The disease started several years ago as multiple small skin-colored papules that subsequently coalesced to form a large soft plaque on the back of the left shoulder. The diagnosis of acquired smooth muscle hamartoma was confirmed on histopathology. The patient was reassured about the benign nature of the lesion and was not advised any treatment.

Secondary syphilis clinically mimicking pseudolymphoma of the face.

Syphilis has been given titles such as the great imitator, the great mimic, and the great masquerader. It may lack pathognomonic signs and often presents similar to many other dermatological conditions. We present a case of a young woman who developed asymptomatic papulonodular lesions on the forehead; the lesions were clinically similar to pseudolymphoma but it was determined that she had secondary syphilis exhibiting prozone phenomenon. She also had alopecia of eyebrows. Both the alopecia as well as nodular lesions clinically resembling pseudolymphoma responded to and completely recovered with antisyphilitic treatment.

Correlation of clinical, histopathological, and microbiological findings in 60 cases of cutaneous leishmaniasis.

BACKGROUND: In an endemic area, cutaneous leishmaniasis (CL) is largely diagnosed by its clinical appearance. Diagnostic challenge arises when the lesions appear in nonendemic area, when clinical picture is distorted, or any atypical variant is seen even in endemic regious. In developing countries like ours, the laboratory aid is not widely available and dermatologists mostly have to rely on clinical experience. AIM: The study was aimed to see the correlation of clinical, histological, and microbiological findings in clinically diagnosed cases of CL. METHODS: It was an observational and descriptive study and was conducted over a period of 2 years in two dermatology centers in the country. Seventy-seven patients with clinically suspicious lesions of CL were screened and 60 of these were diagnosed as true clinical cases on the basis of criteria for clinical diagnosis. These cases were then subjected to slit skin smear and histopathological examination. Parasitologically positive and suggestive cases were recorded and descriptive statistics were used to evaluate the findings. RESULTS: Out of 60 registered cases, 36 (60%) were smear-positive and 30 (50%) demonstrated Leishman Donovan (LD) bodies in histological sections. Twenty-six of the remaining (parasite-negative) cases showed one of the recognizable histological patterns seen in CL, 3 did not reveal any suggestive histology but responded to antimonial compound, and 1 turned out to be a case of deep mycosis. CONCLUSION: Considering the magnitude of the problem and limited resources of a developing country like ours, clinical diagnosis alone may be reliable enough in endemic areas.

Keratoderma hereditarium mutilans (Vohwinkel syndrome) in three siblings.

Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare, diffuse, honeycombed, palmar and plantar keratosis usually accompanied by pseudoainhum near the distal interphalangeal creases. The syndrome is reported in three out of five siblings (two brothers and one sister), who developed this problem in early childhood.

Comparative cellular immune host response in acute vs healed lesions of cutaneous leishmaniasis.

BACKGROUND: Cutaneous leishmaniasis (CL) has become a major public health problem in Pakistan and research is now focused to characterize the host's immune response, its clinical correlation and subsequent implications in management in this disease. This study was done to evaluate cellular immune host response in patients with active and healed CL and its possible implications in prevention of disease in susceptibles. METHODS: This cross sectional and comparative study was conducted in Armed Forces Institute of Pathology (AFIP) and Military Hospital (MH) Rawalpindi (1998-2000). 30 biopsies of active skin lesions and 15 biopsies from healed lesions, after processing, were studied for various immunophenotype cells by using monoclonal antibodies. Total and differential T cell counts were recorded in these skin tissues. Non parametric Kruskal-Wallis Test for one way ANOVA was used to compare the median cell counts between active, healed and normal skin and p-value < 0.05 was considered significant. RESULTS: The total cell counts, CD3+ cells and CD57+ (NK) cells were found statistically different (p = < 0.001) when active forms of the disease were compared with healed lesions and normal skin tissue. The difference was not significant (p = > 0.05) on comparing healed lesions with normal skin tissue biopsies except in case of CD3+ cell counts (p = < 0.05). However, CD4+, CD8+ and CD19+ (Plasma cells) counts were never seen significant (p = > 0.05) on comparison. CONCLUSION: NK cells and gamma delta cells seem to be responsible for limitation of the disease and elimination of the parasite from the lesion in cases of acute cutaneous leishmaniasis.

Cellular immune host response in acute cutaneous leishmaniasis.

OBJECTIVE: To evaluate cellular immune host response in various patterns of acute cutaneous leishmaniasis. DESIGN: It was a cross-sectional and comparative study. PLACE AND DURATION OF STUDY: The Armed Forces Institute of Pathology (AFIP) and Military Hospital (MH), Rawalpindi from 1996 to 1999. PATIENTS AND METHODS: Forty biopsies of active skin lesions after processing were studied for various immunophenotype cells by using monoclonal antibodies. Total as well as differential T cell counts were recorded in acute single, acute multiple and sporotrichoid lesions and in normal skin tissues. Non parametric Kruskal-Wallis Test for one way ANOVA was used to compare cell counts in these groups and p-value <0.05 was considered significant. RESULTS: No significant difference was seen in the histopathology, type of infiltrate and the ratio between the immune competent cells in acute single or multiple lesions. The results of analysis of total cell count, CD3+ cells and CD57+ (NK) cells were statistically different (p=<0.05 to p=<0.001) between acute forms of the disease and normal tissue but no difference was seen when acute forms were compared with each other. However, CD4+, CD8+ and CD19+(Plasma cells) counts difference was significant (p=<0.05 to p=<0.01), when sporotrichoid lesions were compared with other acute lesions (single and multiple). CONCLUSION: The sporotrichoid variant of cutaneous leishmaniasis may be due to a different parasite species, which provokes a different cellular immune response.

Pseudoxanthoma elasticum.

Pseudoxanthoma elasticum is an inherited disorder of connective tissue that is associated with numerous systemic manifestations including various cardiovascular abnormalities. We present a case of a 35 years old lady who presented with abnormal wrinkling, pigmentation and laxity of skin. She also had hypertension, peripheral vascular disease, angioid streaks and mitral valve prolapse. She was thoroughly investigated and managed accordingly. She was also educated to minimize the potential risks involved and advised regular follow-up.

Klippel Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a congenital vascular disorder of unknown cause, characterized by port wine stain (capillary malformations), venous malformation and limb hypertrophy. We present a case of this rare syndrome in a young girl.