Juvenile hyaline fibromatosis

Juvenile hyaline fibromatosis is a rare, autosomal-recessive disease characterized by papular and nodular skin lesions, gingival hyperplasia, joint contractures and bone involvement in variable degrees. It is a connective tissue disorder with aberrant synthesis of glycosaminoglycans by fibroblasts. We report a 5-year-old female born of first-degree consanguineous marriage who presented with multiple, recurrent, painless, variable-sized nodules. Fine needle aspiration cytology smears and the subsequent histopathological examination from the nodules showed benign spindle cells in a Periodic acid Schiff-positive myxoid background. The disease has a relentlessly progressive course, with most patients surviving only up to the 4(th) decade. As of now, there is no specific treatment for this disorder. Genetic counseling is essential to explain to parents about a 25% chance of having a diseased baby in any pregnancy. With the gene being mapped recently, techniques for antenatal diagnosis are likely to be established.     

Bullous aplasia cutis congenita: case report and review of the literature

Aplasia cutis congenita is a rare condition characterized by the absence of skin and sometimes other underlying structures such as bone or dura. It can be a part of various syndromes and can be associated with multiple genetic diseases, malformation patterns, or a combination of all. It is even considered as a form frustre of a neural tube defect in several literatures. Bullous aplasia cutis congenita is a clinical subtype of the condition, with extremely few cases reported in the literature. It presents as a cystic or bullous lesion at birth, which eventually transforms into an atrophic, flat scar covered by a thin epithelium. Some cases present with a dark collar hair sign around the lesion, which can be even more indicative of an underlying neural tube defect. Management remains controversial and depends on the characteristics of the lesion, but conservative treatment is usually chosen.     

Occupational skin problems in construction workers

Background

Construction workers handle cement which has constituents to produce both irritant contact dermatitis and corrosive effects (from alkaline ingredients, such as lime) and sensitization, leading to allergic contact dermatitis (from ingredients, such as chromium).

Aim:

The present study has been carried out among unorganized construction workers to find the prevalence of skin problems.

Materials and Methods:

The present cross-sectional study was conducted in 92 construction workers of Ahmedabad and Vadodara.

Results and Discussion:

All the workers were subjected to clinical examination after collection of information regarding demographic characteristics, occupational characteristics and clinical history on a predesigned proforma. Of them, 47.8% had morbid skin conditions. Frictional callosities in palm were observed in 18 (19.6%) subjects while 4 (4.3%) subjects had contact dermatitis. Other conditions included dry, fissured and scaly skin, infectious skin lesion, tinea cruris, lesion and ulcers on hands and/or soles.

Conclusion

The skin conditions were common in the age group of 20–25 years, males, those having ≥1 year exposure and those working for longer hours. Half of the workers not using personal protective equipment had reported skin-related symptoms.     

Multiple Hamartoma Syndrome: Clinicoradiological Evaluation and Histopathological Correlation with Brief Review of Literature

Cowden syndrome (CS) or multiple hamartoma syndromes (MHSs) is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system. A thorough evaluation of Cowden''s disease is essential due to increased risk of malignancy in an organ or system of the affected patients. We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems. The diagnosis was confirmed by multimodality diagnostic approach. This rare entity has not being reviewed in Indian literature till date. Being a rare case, we are discussing MHS with its clinicoradiological and histopathological correlation along with brief review of literature.     

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.     

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.     

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.     

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.     

PSEUDOEPITHELIOMATOUS, KERATOTIC, AND MICACEOUS BALANITIS

A 51-year-old circumcised male presented with hard, thick, keratotic, nail-like covering of the skin of his glans penis of 2 year duration. Histology showed acanthosis, papillomatosis, and elongated rete ridges into the dermis suggestive of pseudoepitheliomatous, keratotic, and micaceous balanitis with features of cellular atypia. Partial penile amputation was done. There was no recurrence after 6 months of follow up.     

Giant melanoacanthoma mimicking malignant melanoma

Melanoacanthoma denotes a rare variant of pigmented seborrheic keratosis. A 65-year-old male farmer had pigmented, verrucous, itchy, highly painful, progressively growing irregularly oval plaque on left side of lower back for the past five years. The indurated lesion, measuring maximum diameter 10 cm × 5 cm, had no discharge, bleeding, ulceration, or associated lymphadenopathy. Dermoscopy showed regular pigmentary network and cribiform pattern of ridges without any feature of malignant melanoma. Histopathology showed well-defined islands of basaloid cells interspersed with large and richly dendritic melanocytes. The lesion was totally excised followed by skin grafting. Our patient was unique in its massive size and clinical resemblance with malignant melanoma. The diagnosis was confirmed by dermoscopy and skin biopsy.     

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.     

Alopecia Mucinosa Responding to Antileprosy Treatment: Are we Missing Something?

Three cases with single lesion of Alopecia mucinosa (follicular mucinosis) were treated with antileprosy treatment and showed rapid and complete resolution of the lesions with no recurrence on extended follow-up. Two children, a boy aged 14 years and a girl aged 12 years presented themselves, each, with a single hypopigmented, hypoesthetic patch on the face. Clinically leprosy was suspected, however, skin biopsy from both patients revealed follicular mucinosis as the only pathological finding, without any granulomas. Based on clinical suspicion both were started on multi drug therapy (MDT) for leprosy with complete resolution of the lesions. The third case, male, aged 22 years presented with a single erythematous, hypoesthetic plaque on the forehead. This lesion had been diagnosed as follicular mucinosis with folliculo-tropic mycosis fungoides, in the USA. He too responded completely within 3 months with rifampicin, ofloxacin, minocycline (ROM) treatment, which was given once monthly for a total of 6 months and remains free of disease since the past 1 year. Follicular mucinosis as the only pathology may be seen in facial lesions of clinically suspected leprosy in children and young adults. Based on histological findings these cannot be diagnosed as leprosy and will be considered as Alopecia mucinosa. These lesions, however, are always single and show rapid and complete response to antileprosy treatment. The authors suggest that in regions endemic for leprosy, such as India, single lesion Alopecia mucinosa on the face in children and young adults should be given antileprosy treatment.     

Trichoepithelioma and Basal Cell Carcinoma with Squamous Differentiation: Is it Causal or Coincidental?

Trichoepitheliomas (TEs) are benign cutaneous tumors that occur either as solitary non-familial or multiple familial. We report a case of multiple familial trichoepithelioma (MFT) in a 55-year-old female patient and her son who came with complaints of single ulcerated mass involving the left nasolabial fold and cheek. She had multiple papules and nodules all over the face and neck since 25 years. Histopathological examination of an ulcerated lesion revealed features of basal cell carcinoma (BCC) with squamous differentiation, which was confirmed by immunohistochemistry. A skin biopsy obtained from the papule on neck showed features of TE. However, whether BCC developed independently or by transformation from TE was uncertain. Her 36-year-old son presented with similar lesions on the face and a skin biopsy showed features of TE. Though malignant transformation of TE is quite rare, awareness of the potential for evolution of carcinoma in patients with MFT is important for management of these patients.     

Spontaneous Acroangiodermatitis

Acroangiodermatitis is a rare self-limited angioproliferative lesion which can be associated with congenital vascular malformations or acquired venous insufficiency. Despite of its benign character, differential diagnosis of this lesion is very important because it closely resembles Kaposi sarcoma. Here we present a 26-year-old male patient with unilateral, purplish-red colored papules on his right ankle which diagnosed as acroangiodermatitis and discuss histopathological features, differential diagnosis and treatment of this unusual condition.     

Primary cutaneous B-cell lymphoma successfully treated with highly active antiretroviral therapy alone: a case report and review of the literature

Cutaneous B-cell lymphoma (CBCL) is an unusual skin neoplasm with a great range of clinical presentations. Here, we report a case of CBCL in an AIDS patient presented as a single and nodular/ulcerative lesion in the perianal area. The patient was started on highly active antiretroviral therapy alone with a good clinical and oncological response. Two years later, the patient is asymptomatic with undetectable viral load and immune reconstitution.     

Concurrent Presentation of Erythrodermic Lichen Planus and Squamous Cell Carcinoma: Coincidence or Malignant Transformation?

Lichen planus is a common papulosquamous disorder affecting about 1-2% of the population, neoplastic transformation of cutaneous lichen planus lesions occurs very rarely. A 40 year old female patient presented with a 1 year history of developing multiple, itchy, pigmented lesions over both lower legs which gradually spread to involve the whole body. A few tense bullae were seen on the extremities. An erythematous fleshy lesion was seen on the upper aspect of the left buttock. Skin biopsy from a plaque on the right forearm showed features suggestive of lichen planus. Skin biopsy of a bullae showed a sub epidermal bulla filled with a mixed inflammatory infiltrate. Direct immunofluorescence revealed no immunoreactants along the basement membrane zone. A diagnosis of erythrodermic lichen planus with bullous lichen planus was made. Biopsy of fleshy lesion of left buttock revealed a moderately differentiated squamous cell carcinoma. Erythrodermic lichen planus with bullous lesions and secondary squamous cell carcinoma; these occurences in a single patient is extremely rare and has not been previously reported to the best of our knowledge.     

Pseudoepitheliomatous Keratotic and Micaceous Balanitis: A Rare Condition Successfully Treated with Topical 5-Fluorouracil

A 50-year-old man presented with slow-growing dry, rough, micaceous scaly plaque over glans penis, which was compatible with clinical diagnosis of pseudoepitheliomatous keratotic and micaceous balanitis (PKMB) and histologically suggestive of PKMB without cellular atypia. He was treated successfully with topical 5-fluorouracil with complete clearance of lesion in 3 weeks without recurrence for 10 months. PKMB is an extremely rare and interesting condition; only a handful cases have been reported in world literature.     

Papillon-lefevre syndrome: a combined approach from the dermatologist and dentist - a clinical presentation

Papillon-Lefevre syndrome (PLS) is a rare disease characterized by skin lesions, which includes palmar-plantar hyperkeratosis and hyperhidrosis with severe periodontal destruction involving both the primary and the permanent dentitions. It is transmitted as an autosomal-recessive condition, and consanguinity of parents is evident in about one-third of the cases. This paper describes a 13-year-old male patient who presented to the department of pedodontics, with rapidly progressing periodontitis. A general physical examination revealed scaling on the hands and feet, which had been medically diagnosed as PLS. The incidence of this rare entity is increasing in the recent times, which is associated with irreparable periodontal destruction at an early age, with not so prominent skin lesions in some cases. In such instances, the dentist has a more important role in diagnosing, treatment planning and preservation of the periodontal tissues and, at the same time, referring for the treatment of the skin lesions. This paper emphasizes the combined effort of the two specialities in order to maintain skin as well as dental conditions in health by early intervention and a synergistic treatment approach.     

Zosteriform skin metastases: clue to an undiagnosed breast cancer

Cancer metastases represent the most devastating aspect of malignancy, since the mortality of cancer patients is mainly related to the metastatic behavior of the primary neoplasm. Skin metastases are usually late events in the course of tumor progression. Excluding melanoma, the most common tumor to metastasize to the skin is breast cancer. Patients who develop cutaneous metastases rarely present with a zosteriform distribution. Herein, we present a 60-year-old female, an undiagnosed case of breast cancer, with zosteriform metastases along her right T2-T3 dermatome.