Chromoblastomycosis from a Non-endemic Area and Response to Itraconazole

Chromoblastomycosis belongs to the heterogeneous group of subcutaneous mycoses. It is caused by various pigmented (dematiaceous) fungi, which gain entry into the skin via traumatic implantation. We would like to share a case report of chromoblastomycosis in a 32-year-old male, who presented to us with 3 years history of slowly progressive, itchy, verrucous, crusted lesions over right forearm and arm. He is being treated with itraconazole 100 mg twice daily. The case is of interest because it has so far not been reported from our region- the northwest arid zone of India. The patient showed favorable response to itraconazole.     

Solitary cylindroma with malignant transformation

A 59-year-old woman presented with a history of rapidly progressive recurrent tumor of 6.5 cm diameter of the scalp. Histopathological examination revealed a case of malignant cylindroma. There has been no recurrence or metastases and the patient is disease free at the end of 15 months postoperatively. Malignant transformation occurs less often in solitary form of cylindroma, but once transformed, tumors behave aggressively with extensive local infiltrative growth or metastases. The case is reported to document a rare case of malignant cylindroma arising in a patient with solitary cylindroma on the parieto-temporal region.     

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.     

SCLEREDEMA DIABETICORUM WITH UNUSUAL PRESENTATION AND FATAL OUTCOME

We present a case of Scleredema Diabeticorum (SD) in a patient with diabetic neuropathy and restrictive respiratory disease with unusual skin lesion distribution. The onset of dermatologic symptoms heralded a progressive respiratory disease with constrictive component. Painful diabetic neuropathy was noteworthy and difficult to relieve. Predominantly, distribution of the skin lesions on the thighs makes the case exceptional. T2-weighted MRI showed abnormal hyperintensities along the muscles of the thighs in correspondence with the skin lesions. Gait and respiratory symptoms progressively worsened. After a transient remitting period, he developed sudden shortening of breath, arrested and expired at home.The atypical distribution of the skin lesions with further involvement of underlying muscles plus concomitant polyneuropathy and respiratory constrictive disease with sudden death is quite unusual and aggressive presentation of SD.     

A solitary crateriform ulcer of the lower lip: a case report with review of literature

Keratoacanthoma (KA) is a relatively common skin lesion. We report a case of KA-mimicking squamous cell carcinoma in a 40-year old smoker, who was also having speckeled leukoplakia on the buccal mucosa. The present case highlights the importance of histopathological diagnosis, as the treatment in latter case is aggressive, which is unnecessary if attempted with KA. Moreover, the present report provides an overview of such lesions, which could be encountered in clinical situations for which definitive diagnosis is of paramount importance before the starting of treatment plan.     

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.     

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.     

HEMATOHIDROSIS �C A RARE CLINICAL PHENOMENON

Hematohidrosis is a very rare condition of sweating blood. A case of hematohidrosis is reported. There are only few reports in the literature.     

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.     

Dermatitis herpetiformis, primary hypothyroidism and pituitary mass mimicking macroadenoma regression after treatment with thyroxin, corticosteroids and dapsone

We report a case of 15-year-old girl who was diagnosed as a case of pituitary macroadenoma on computed tomography and magnetic resonance imaging (MRI) scans for her complains of diminished vision in the left eye and headache. On investigation she was found to have optic atrophy of left eye and primary hypothyroidism. She was started on thyroxin therapy in October 2008 and planned for transcranial excision of the tumor. Just before the date for planned surgery she developed mildly itchy vesico-bullous rash and the surgery was postponed. On histopathology and immunoflourescence studies it was confirmed to be the rash of dermatitis herpetiformis (DH) and treatment was started with corticosteroids in the last week of December 2008 and dapsone was added from mid January 2009. The lesions responded dramatically and within 6 weeks of starting treatment, she had become asymptomatic except for persisting diminution of vision. Repeat MRI scans in mid-February 2009 revealed total regression of pituitary mass. The case is being reported for interesting association of primary hypothyroidism, pituitary mass mimicking macroadenoma and DH; its successful outcome with medical management in the form of total regression of pituitary mass, thereby avoiding an unnecessary surgery.     

Pachyonychia Congenita Type 1: Case Report and Review of the Literature

The case of an 8-year-old boy is hereby reported, who presented with nail dystrophy, subungual hyperkeratosis, oral leukokeratosis, and numerous follicular papules all over the body. The features were consistent with a diagnosis of pachyonychia congenita type 1. The case is being reported for its rarity. We also discuss in a nutshell, the literature till date.     

MUCOSAL PATCH ON ANUS: A RARE SEQUEL OF SODOMY

A case of mucosal patch on the perianal area of a 15-year-old boy with history of frequent sodomy is presented here.     

Acral,Superficial Spreading Melanoma Arising on Melanocytic Nevus in a Pregnant Woman: A Case Report with Review

We are reporting a case of superficial spreading melanoma (SSM) on left palm of a 37-year-old pregnant housewife. She had a small acquired melanocytic nevus on her left palm since childhood, which changed its consistency and color in the last 4 months. Dermoscopy of the lesion indicated malignant changes. The lesion was managed surgically using split-thickness skin graft. The histopathology report was suggestive of SSM with positive HMB-45 cells. SSM is very rare on the acral site, and it is very difficult to differentiate it from acral lentiginous melanoma. The rarity of the site (acral nonchronic sun damage) with evolution during pregnancy and importance of management approach are reasons for publishing this case.     

X-linked Ichthyosis Presenting as Erythroderma: A Rare Case

X-linked ichthyosis is a rare form of dermatological disease and when it presents as erythroderma it is even rarer. History of consanguineous marriage and prolonged labor during birth of patient, generalized scaling which gets better in summer months, flexural involvement, cryptorchidism made a diagnosis of X-linked ichthyosis. We report this case because of its rarity as erythroderma.     

Allergic Urticaria: A Case Report of Rare Skin Allergy with a Common Mouthwash

Chlorhexidine is a widely used antiseptic and disinfectant in medical and non-medical environments. Compared to its ubiquitous use, allergic contact dermatitis from chlorhexidine has rarely been reported and so its sensitization rate seems to be low. Chlorhexidine has been used for more than 50 years but it was only in the last two decades, that reports of immediate- type reactions to chlorhexidine were seen. Reactions ranging from localized urticaria to anaphylactic shock and hypersensitivity reactions, including delayed hypersensitivity reactions such as contact dermatitis, fixed drug eruptions, and photosensitivity reactions, began to appear more frequently. However the prevalence of contact urticaria and anaphylaxis due to chlorhexidine remains to be unknown. In this case report we have reported a case of urticaria due to oral use of chlorhexidine. The adverse reaction was confirmed by a skin prick test.     

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.     

Transient Idiopathic Primary Penoscrotal Edema

We present the case of a male born prematurely at 32 weeks gestation by cesarean section following overt symptoms of maternal preeclampsia. He developed severe penoscrotal edema anew one month from birth. No remarkable exposure or trauma was identified. This unexplained swelling remained uniform till 4 months of age, while the penile edema resolved spontaneously. A small benign hydrocele remained unchanged, since onset of the edema and continued after the edema subsided. This is the first report of persistent, but transient penoscrotal edema resolving in a 3 months course, without any apparent explanation, a possible pathogenetic mechanism was suggested.     

Multiple Carboxylase Deficiency (Late Onset) Due to Deficiency of Biotinidase

Biotinidase is a ubiquitous mammalian cell enzyme occurring in liver, serum and kidney. It cleaves biotin from biocytin, which is a cofactor for biotin dependent enzymes, namely the human carboxylases. Biotinidase deficiency is associated with a wide spectrum of neurological, dermatological, immunological and ophthalmological abnormalities. This is a case of a 3-year-old boy presenting with delayed developmental milestones, tachypnea, progressively increasing ataxia, alopecia and dermatitis, all which dramatically responded to high doses of biotin.     

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.     

Metastatic Fungating Ulcerative Growth on Vulva as a Presenting Feature of Carcinoma Cervix: A Rare Case Report

Carcinoma of the cervix is the second to fourth most common malignancy in women. It metastasizes most often to the lungs, bones, and liver. Skin involvement originating from cervical cancer is rare, even in the terminal stages of the disease. Cutaneous metastasis of cervical cancer usually presents as cutaneous nodules, papules/plaques, maculopapular rash, and diffuse inflammatory rash. We report a rare case (only the second reported case to the best of our knowledge) of a 50-year-old woman with cutaneous metastasis in form of fungating ulcerative growth on mons pubis as presenting feature of carcinoma cervix.