A Case of Ichthyosis Hystrix: Unusual Manifestation of This Rare Disease

Ichthyosis hystrix is a term used to describe an ichthyosiform dermatosis which is characterized by hyperkeratotic spiny scales mainly over extensor aspects of limbs with palmoplantar keratoderma and occasionally associated with deafness and neurological deficit. It is a rare autosomal dominant form of ichthyosis and very few cases are reported in literature. We are presenting a 46 years-old-male patient of ichthyosis hystrix with unusual presentation. He had lesions mainly over the face and scalp with palmoplantar keratoderma and significant nail changes.     

A Case of Lamellar Ichthyosis with Rickets and Carcinoma of the Hypopharynx

Lamellar ichthyosis (LI) is an autosomal recessive disorder rarely associated with systemic organ involvement and development of carcinoma. Rickets has occasionally been described with LI owing to impaired vitamin D synthesis following altered keratinization. There has also been a high association of cutaneous cancers in patients of LI. We as Dermatologists should therefore be very meticulous while doing a full work up of these patients. We report here a case of LI associated with rickets and carcinoma of the hypopharynx.     

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.     

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.     

Neonatal Erythroderma: Diagnostic and Therapeutic Challenges

Erythroderma a life-threatening entity during the first one month, and many a time, a manifestation of genodermatosis, immune deficiency, psoriasis, metabolic diseases, and infections. Atopic dermatitis presenting as erythroderma is usually observed later, after this one-month period, and hence not a common differential for neonatal exfoliative dermatitis. Although a rare entity, there is a paucity of studies on this and in contrast to adults, some may manifest as cardinal signs of primary disease conditions.     

A Study of Correlation Between Clinical and Histopathological Findings of Erythroderma in North Bengal Population

Background:

Erythroderma is a reaction pattern characterized by erythema and desquamation of 90% or more body surface area along with some metabolic alterations.

Materials and Methods:

Here we studied 32 patients of erythroderma at of North Bengal Medical College for a period of 1 year to find the etiology, clinical features and histological changes. Detailed history was taken from all the patients followed by relevant biochemical investigations and histological examination. To correlate the clinical and histopathological findings chi square test was used.

Results:

Male preponderance was present and most of them were in the 4^th or 5^th decade. Etiologically the patients were divided into secondary erythroderma developing over pre-existing dermatoses, and idiopathic erythroderma. Secondary erythroderma (n = 24) cases outnumbered the idiopathic cases (n = 8). Among the pre-existing dermatoses, psoriasis was found to be the most common etiologic agent. Apart from erythema the other common presenting features were scaling and itching. Histopathological categorization was possible in 59.3% cases, rest of the cases showed non-specific dermatitis. The most common histopathologic diagnosis was psoriasis (21.8% of cases).

Conclusions:

Our study of clinicopathological correlation of erythroderma patients among north bengal population corroborates with most of the previous studies done in other areas. As ours is a cross-sectional study in a undefined population so we could not determine the true incidence of erythroderma in north bengal population. We might have missed lymphoma as a cause of erythroderma in idiopathic cases due to lack of long follow-up, so we understand that further studies over a defined population with long follow-up is needed to determine the true incidence and causes of idiopathic erythroderma.     

Psoriatic Erythroderma and Hypothalamus-Pituitary Axis Suppression Due to Misuse of Systemic Steroid: Two Challenging Cases

Adding corticosteroid in homeopathic pills, self medication of steroid in the backdrop permanent cure in cases of psoriasis is not very uncommon in clinical practice in Indian subcontinent. First case a 52 year man, a known case of psoriasis vulgaris with psoriatic arthropathy of 15 years duration received multiple modalities of therapies without any satisfactory response. He was on self medication of tab prednisolone 10 mg daily with Cushingoid features. Second case a 22 year old boy, a known case of psoriasis from last 06 years was on Homeopathic treatment with Cushinoid features. Tapering of systemic steroid in first case and discontinuation of homeopathic drug in second case resulted in erythroderma and features of adrenal insufficiiency. Investigation revealed low morning cortisol and low cortisol following ACTH stimulation suggestive of HPA axis suppression. Planned withdrawal of steroid under the cover of short acting systemic steroid for short duration along with combination of immunosuppressants and supportive care gave an excellent result in both the cases.     

Idiopathic Non-Familial Acro-Osteolysis: A Rare Case Report

A 25-year-old woman patient presented with shortening of fingers with racket nails and numerous yellowish papules over the hands and forearms for 21 years. X-ray of the hands revealed destructive osteolytic changes in all the terminal phalanges. Skin biopsy from the yellowish papules showed epidermal proliferation, perivascular mononuclear infiltrate, thickening of dermal collagen, septal fibrosis and loss of adipocytes mimicking sclerodermatous changes in the dermis and hypodermis. The patient did not have any history of similar illness in the family or occupational exposure to vinyl chloride. After excluding all other possibilities of acral-osteolysis, we diagnosed the case as idiopathic non-familial variety of acro-osteolysis. This is a rare entity characterized by terminal resorption of fingers, sometimes associated with Raynaud''s phenomena and yellowish cutaneous papules.     

Vitamin K Deficiency Bleeding Presenting as Nodular Purpura in Infancy: A Rare and Life-Threatening Entity

Vitamin K deficiency bleeding (VKDB) disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages.^[1] Though skin and mucosal bleeding may occur in 1/3^rd of infants with VKDB, ‘nodular purpura’ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.     

A Rare Case of Subungual Melanoma

A 51-year-old male presented with blackish discoloration of nails of 10 months duration. Examination revealed black dystrophic left thumb finger nail. Detailed examination showed a mass under the dystrophic nail. Hutchinson sign was positive. Histopathology revealed characteristic features of melanoma. A detailed evaluation revealed no features of local or distant metastasis. The entire lesion was then removed surgically along with disarticulation at the interphalangeal joint. Resection-free margin was confirmed. This case is being reported for the rare occurrence of subungual melanoma in the Indian population and also for presentation with a long history of lesion with no evidence of metastasis.     

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.     

Chondroid Syringoma of the Forearm: A Case Report of a Rare Localization

Chondroid syringoma (CS) is an uncommon benign adnexal tumor of the skin with eccrine and apocrine origin, which usually involves the head and neck region. The presentation of CS in other areas of the body is rare. A 45-year-old male patient presented to the dermatology clinic with a chief complaint of a painless, slow-growing mass on his left forearm, which gradually developed over the course of 2 years. A solitary, firm, purple, mobile, non-tender nodule was located in the distal part of left dorsal forearm, which was 1.8 cm in diameter. The tumor was surgically excised and sent for the histopathological evaluation. Results of biopsy and hematoxylin and eosin staining confirmed the diagnosis of CS and showed no evidence of malignancy. Although CS is an uncommon tumor in uppr limb region, it should be considered as one of the differential diagnoses, when dealing with tumors of this area.     

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.     

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.     

A Rare Case of Plantar Epithelioma Cuniculatum Arising from a Wart

A 68-year-old man, a known case of hypertension, coronary artery disease and old cardiovascular accident with right-sided hemiplegia, came with the chief complaints of a large cauliflower like growth with pus discharge on the left heel since 15 years. The patient had sustained a penetrating injury by a thorn on the left heel region few days before the lesion appeared. Dermatological examination revealed a single verrucous lesion measuring 7 × 7 cm on the left heel region associated with discharge of foul smelling cheesy material. There was also a enlarged right inguinal lymph node which was non-tender, firm, measuring 2 cm in diameter with normal overlying skin. X-ray left ankle was done which showed some soft tissue swelling. A skin biopsy showed hyperkeratosis, acanthosis and parakeratosis. Elongated rete ridges with keratinocyte hyperplasia, forming a large mass pressing on the underlying dermis were seen. There was formation of multiple large keratin filled invaginations and crypts. No atypical cells were seen. Based on history, clinical examination and investigations, a diagnosis of epithelium cuniculatum type of verrucous squamous cell carcinoma was made. A wide excision with a flap cover was performed in consultation with the oncosurgeon and the excision sample was sent for histopathological re-examination, which confirmed the diagnosis of epithelioma cuniculatum.     

Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon

An 18-year-old girl presented with multiple itchy hyperpigmented papules and plaques, along with tense blisters over the lower limbs and buttocks for last 3 months. These papules, plaques, and bullae were mostly localized to preexisting scars. The histopathological findings from papule and bulla were consistent with lichen planus (LP) and bullous pemphigoid, respectively. Direct immunofluorescence (DIF) of perilesional skin around bulla showed linear deposition of IgG and C3. Considering clinical, histopathological and DIF findings, diagnosis of LP pemphigoides (LPP) was made. The preferential localization of LPP lesions over preexisting scars was a very interesting finding in our case an extremely rare instance of the isotopic phenomenon.     

Nodulocystic Basal Cell Carcinoma Arising Directly from a Seborrheic Keratosis: A Rare Case Report

Seborrheic keratoses (SKs) are common epidermal tumors composed of benign keratinocytes. Malignant skin tumors including basal cell carcinoma (BCC) rarely arise within SKs. We report a rare case of an 82-year-old man with nodulocystic BCC that appeared at the center of a scaly hyperpigmented SK that had been presented for more than 10 years. It was histologically confirmed that CK19-positive BCC arose directly from the wall of the pseudohorn cyst, a part of the SK. Nodular and/or cystic BCC also rarely arise within SKs while the most common histologic type of BCC within SKs is the superficial type. Careful observation of SKs is important even though it is rarely a background condition for malignant transformation.     

Hypereosinophilic syndrome: cutaneous involvement as the sole manifestation

Hypereosinophilic syndrome (HES) encompasses a group of leukoproliferative disorders with variable involvement of the internal organs. More than half of all patients have cutaneous involvement. In a minority of the reported cases, skin involvement has been the only manifestation of HES . We report one such rare case of HES, with cutaneous involvement as the sole manifestation.     

Reactive Eccrine Syringofibroadenomatosis Presenting as Bilateral Plantar Hyperkeratosis

Eccrine syringofibroadenoma (ESFA) is a rare cutaneous tumor with eccrine differentiation with varied clinical manifestations. We report a case of reactive eccrine syringofibroadenomatosis associated with chronic bilateral plantar ulcers in a patient with diabetes mellitus presenting as plantar hyperkeratosis and verrucous growth at margins.     

Tuberculosis Verrucosa Cutis Presenting as Diffuse Plantar Keratoderma: An Unusual Sight

Tuberculosis verrucosa cutis (TVC) is a common cutaneous form of paucibacillary tuberculosis in an individual with moderate to high degree of immunity to Mycobacterium tuberculosis infection. Clinical appearance of TVC is mostly very typical with well-defined warty plaques presenting mostly on hands, knees, ankle, and buttocks; however several atypical morphology of the lesions have also been described. We hereby report a case of TVC, masquerading as asymptomatic diffuse keratoderma of left foot for nine months, in an otherwise healthy individual, obstructing easy diagnosis of cutaneous tuberculosis. Diagnosis was confirmed by histopathology.