Amyloidosis Cutis Dyschromica: A Rare Reticulate Pigmentary Dermatosis

We are reporting a rare case of amyloidosis cutis dyschromica in a 41-year-old man. This is a rare form of primary cutaneous amyloidosis characterized by reticulate pigmentation with hypopigmented and hyperpigmented macules, onset in childhood, familial tendency in some, occasional mild itching and deposition of amyloid in the papillary dermis. Our case also had multiple bilaterally symmetrical hyperpigmented keratotic papules abutting the axillary vault resembling those seen in Dowling–Deogs disease. The other unusual feature in this patient was the strong family history of vitiligo, which we are unable to explain. We have also tried to explain the mechanism leading to the hyperpigmentation and hypopigmentation in amyloidosis cutis dyschromica.     

Primary systemic amyloidosis presenting as extensive cutaneous ulceration

BACKGROUND: We report a case of primary systemic amyloidosis in a 78-year-old Caucasian woman presented as a nonhealing ulcer on the right thigh for 3 months. Histopathology of the skin revealed widely thickened walls of middermal and subcutaneous vessels from deposition of amorphous eosinophilic material that stained positively with Congo red and crystal violet. OBJECTIVE: This case represents a very unusual presentation of primary systemic amyloidosis, one in which the cutaneous manifestations provided the first signs of a devastatingly widespread multiorgan infiltration of amyloid protein. CONCLUSION: This presentation of the disease may signify an advanced stage with a grave prognosis as our patient passed away 3 months after development of the cutaneous ulceration.     

Nodular amyloidosis in a patient with liver cirrhosis

A 43-year-old Japanese man with alcoholic liver cirrhosis developed a nodule on the face 1 year prior to presentation. Histopathological examination showed amyloid deposition in the entire dermis, with numerous plasma cells. Nodular primary localized cutaneous amyloidosis is a rare form of amyloidosis, which needs long-term follow-up because of the possibility of the development of systemic amyloidosis. Also, this type of cutaneous amyloidosis may have other complications.     

Cutaneous sarcoidosis - a great masquerader: a report of three interesting cases

It is a known fact that cutaneous sarcoidosis is a great imitator in dermatology. We report three cases of cutaneous sarcoidosis without systemic involvement and with varied dermatologic presentation with regard to age and morphology. Lesions mimicked various common dermatologic conditions, causing great confusion for the diagnosis and posing problems for management. Awareness of these varied morphologic presentations is essential for the early diagnosis and management of the master mimicker - cutaneous sarcoidosis.     

Violaceous Maculopapular Rash in a Newborn: Congenital Rubella Syndrome

Congenital rubella syndrome involves a configuration of systemic and cutaneous manifestations in a neonate due to in utero infection caused by the rubella virus. The case of a preterm neonate with blueberry muffin lesions and classical as well as rare systemic features of congenital rubella syndrome is reported.     

Extrinsic apoptotic pathways: a new potential "target" for more sufficient therapy in a case of cutaneous anaplastic large cd30+ alk-T-cell lymphoma

The primary cutaneous T-cell lymphomas (CTCL) represent a clonal T-lymphocyte proliferation infiltrating the skin. CD30⁺T-cell lymphomas present clinically as nodules with a diameter between 1 and 15 cm, mostly in elderly patients. The role of the CD30 molecule in patients suffering from T-cell lymphomas is not completely clear yet. The signal transduction pathway which includes CD30 seems to play a key role in tumor progression. In certain forms of T-cellular lymphomas, the interaction between CD30/CD30-ligand is able to provoke apoptosis of the “tumor lymphocytes”. The modern conceptions of the pathogenesis of T-cell lymphomas include disorders in the pathways involved in programmed cellular death and disregulation in the expression of certain of its regulatory molecules. We are presenting an unusual case of a female patient with a primary cutaneous form of CD30⁺/ALK⁻ anaplastic large T-cell lymphoma. Upon the introduction of systemic PUVA, (psoralen plus ultraviolet light radiation) combined with beam therapy, a complete remission could be noticed. Eight months later, we observed a local recurrence, which was overcome by CHOP chemotherapy (Cyclophosphamide, Hydroxydaunorubicin (Doxorubicin), Vincristin (Oncovin®), Predniso(lo)n). Six months later, new cutaneous lesions had been noticed again. A new therapeutic hope for the patients with anaplastic large CTCL is actually based on the influence of the activity of the different apoptotic pathways. Death ligands, including tumor necrosis factor (TNF)-α, CD95L/FasL, and TRAIL, mediate also some important safeguard mechanisms against tumor growth in patients with CD30⁺ cutaneous anaplastic large T-cell lymphomas and critically contribute to lymphocyte homeostasis.     

UNUSUAL PRESENTATION OF GENERALIZED MACULAR AMYLOIDOSIS IN A YOUNG ADULT

Macular amyloidosis is a common problem seen dermatology out-patient department. Generalized macular amyloidosis presenting with a poikilodermatous appearance is rare. In our case, an 18-year-old male presented with generalized hypopigmented macules with a poikilodermatous appearance of 10-year duration. His developmental milestones were normal with negative family history of similar complaints. Histopathology of hyperpigmented lesions revealed hyperkeratosis and acanthosis of epidermis and hypopigmented lesion showing only hyperkeratosis. Both lesions were showing the deposition of amorphous, hazy material in the tips of papillary dermis with perivascular inflammatory infiltrate. Congo red staining of the amorphous material was positive for amyloid.     

In-transit metastases from squamous cell carcinoma penis

An in-transit metastasis is one that is located between the primary tumor and the closest lymph node region and results from tumor emboli getting trapped in the lymphatic channels. A 65-year-old male patient who had undergone partial amputation of the penis and bilateral inguinal lymph node resection for squamous cell carcinoma of the penis 4 months earlier developed multiple cutaneous metastatic lesions in the pubic region and scrotum. The case is reported for the uncommon presentation of in-transit metastases.     

原发性系统性淀粉样变病1例

报告1例原发性系统性淀粉样变病。患者男，39岁。1年前肩关节突然出现酸痛，症状渐渐加重，双手掌皮肤发硬，肢体上抬及下蹲困难，舌不能外伸，吞咽时有梗阻感。曾经患有胸椎椎体骨髓瘤。皮肤科检查示面颈部、双侧腋下、腹部、阴茎、双大腿内侧泛发对称性的蜡样半球形丘疹，有融合，呈肤色或琥珀色。口腔黏膜肥厚，舌弥漫性浸润、质硬，呈巨舌。皮损组织病理检查示表皮萎缩，真皮与皮下组织淀粉样物质沉积，甲基紫染色阳性，诊断为原发性系统性淀粉样变病。给予泼尼松和雷公藤治疗，症状明显改善。     

Phakomatosis Pigmentovascularis Presenting with Sturge-Weber Syndrome and Klippel-Trenaunay Syndrome

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype ‘a’ present only with cutaneous form and subtype ‘b’ also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.     

Extensive Infection of Face by Mycobacterium chelonae: An Unusual Presentation

Mycobacterium chelonae is a rapidly growing mycobacteria, causes cutaneous, soft tissue, and rarely lung infections. Here we present a rare case of extensive infection of face at multiple sites by Mycobacterium chelonae, with an unusual presentation, diagnosed by using conventional methods.     

A clinico-epidemiological study of macular amyloidosis from north India

Background:

Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly over the trunk and extremities. MA has a high incidence in Asia, Middle East, and South America. Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated.

Aim:

This study attempts to evaluate the epidemiology and risk factors in the etiology of MA.

Materials and: Methods:

Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Skin biopsies of 26 randomly selected patients were studied for the deposition of amyloid.

Results:

We observed a characteristic female preponderance (88%) with a female to male ratio of 7.3:1, with a mean age of onset of MA being earlier in females. Upper back was involved in 80% of patients and sun-exposed sites were involved in 64% cases. Incidence of MA was high in patients with skin phototype III. Role of friction was inconclusive

Conclusion:

Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. Histopathology is not specific and amyloid deposits can be demonstrated only in a small number of patients. For want of the requisite information on the natural course and definitive etiology, the disease MA remains an enigma and a source of concern for the suffering patients.     

Localized primary cutaneous nodular amyloidosis: case report

Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.     

Primary Cutaneous Plasmacytosis: Masquerading as Hidradenitis Suppurativa

Isolated cutaneous plasmacytosis (CP) is a rare entity with few cases reported in world literature. CP masquerading as hidradenitis suppurativa like presentation is a unique case with some features differentiating it clinically from it which were further confirmed by histopathology and immunostaining. Our case showed hyperplasia of mature plasma cells and polyclonal hypergammaglobulinemia, immunostaining for CD138 positivity and kappa: lambda ratio more than 3:1. Extensive clinical and laboratory investigations failed to reveal any underlying pathology, presence of any underlying disease accompanying the hypergammaglobulinemia and/or plasma cell proliferation.     

Disseminated cutaneous histoplasmosis in an immunocompetent adult

Histoplasmosis, a systemic mycosis caused by the dimorphic fungus Histoplasma capsulatum var capsulatum and Histoplasma capsulatum var duboisii is endemic to many parts of the world. The clinical manifestations range from acute or chronic pulmonary infection to a progressive disseminated disease. After initial exposure to the fungus, the infection is self-limited and restricted to the lungs in 99% of healthy individuals. The remaining 1%, however, progress to either disseminated or chronic disease involving the lungs, liver, spleen, lymph nodes, bone marrow or rarely, the skin and mucous membranes. Mucocutaneous histoplasmosis is frequently reported in patients with acquired immune deficiency syndrome (AIDS), but it is rare in immunocompetent hosts. A 60-year-old male presented with asymptomatic swelling of the hard palate and crusted papules and nodules over the extremities, face and trunk. Clinically, the diagnoses of cutaneous cryptococcosis versus histoplasmosis was considered in this patient. A chest X-ray revealed hilar lymphadenopathy. Enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was nonreactive. Skin biopsy revealed multiple tiny intracellular round yeast forms with a halo in the mid-dermis. Culture of the skin biopsy in Sabouraud's dextrose agar showed colonies of Histoplasma capsulatum. Despite an investigation including no evidence of underlying immunosuppression was found, he was started on IV amphotericin-B (0.5 mg/kg/day). However, the patient succumbed to his disease 2 days after presentation. We report a rare case of disseminated cutaneous histoplasmosis in an immunocompetent individual.     

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.     

ALOPECIA AREATA AND AUTOIMMUNITY: A CLINICAL STUDY

Alopecia areata (AA) frequently occur in association with other autoimmune diseases such as thyroid disorders, anemias and other skin disorders with autoimmune etiology. Despite numerous studies related to individual disease associations in alopecia areata, there is paucity of literature regarding comprehensive studies on concomitant cutaneous and systemic diseases. The present study has been designed to determine if there is a significant association between alopecia areata and other autoimmune diseases. This study covers 71 patients with the diagnosis of alopecia areata as the case group and 71 patients with no evidence of alopecia areata as the control group. Among the cutaneous diseases associated with AA, atopic dermatitis (AD) showed maximum frequency with an O/E ratio of 2.5, which indicates that it is two to three times more common in patients with alopecia areata. In our study, thyroid disorders showed the highest frequency with on O/E ratio of 3.2 and a P value of 0.01, which is statistically highly significant. Among the thyroid disorders, hypothyroidism was the most frequent association (14.1%) in our study. Since systemic involvement is not infrequent in patients with alopecia areata, it is imperative to screen these patients for associated disorders, particularly atopy, thyroid diseases, anemias and other autoimmune disorders, especially if alopecia areata is chronic, recurrent and extensive.     

Congenital lupus erythematosus

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.     

Myeloma-associated systemic amyloidosis presenting with acquired digital cutis laxa-like changes

A 59-year-old woman presented with a 6-year history of lax skin on the distal fingers of both hands, as well as a recent increase in the size of her tongue. Histopathology of skin from her distal finger showed amyloid deposition and bone marrow biopsy revealed an underlying plasma cell dyscrasia. Initial treatment with cyclophosphamide, vincristine, adriamycin and methylprednisolone has produced a significant reduction in the swelling of both her hands and tongue. Acquired digital cutis laxa-like changes are a rare cutaneous manifestation of systemic amyloidosis.