Distinctive Dermatosis Research Articles

Etiopathological Study of 50 Patients of Cutaneous Lichen Planus in Ayurveda

Lichen planus is a distinctive dermatosis characterized by pruritic violaceous flat topped papule or plaque with a network of white lines called the Wickham’s striae. First introduced by Erasmus Wilson in 1869, it an idiopathic inflammatory, autoimmune disorder affecting the skin, the mucous membrane, hairs and nails. Sites involved in skin are the flexor surface of wrists and forearms, dorsal surface of hands, anterior aspect of lower legs, neck and pre sacral area. In Ayurveda, though there is no direct reference of cutaneous lichen planus like diseases, there are certain skin lesions mentioned under Kusthas like Charmakustha, Kitibha Kustha, Vicarchika and Alasa having signs and symptoms similar to cutaneous lichen planus. An attempt has been made to study the aetiopathogenesis of cutaneous lichen planus in Ayurveda and to evaluate the common Nidanas mentioned under Kustha, which are commonly responsible for causation of the disease. For this purpose, a study was done on 50 patients suffering from cutaneous lichen planus. The patients were selected randomly from OPD & IPD; Govt. Ayurvedic College and Hospital, Guwahati, Assam. Proper history was taken in a specially designed proforma to evaluate the most common Nidanas. From this study it was found that there is a definite role of Aharaja, Viharaja and Manasik Nidanas in cutaneous Lichen planus. The Nidanas were mostly Pitta-Kapha Vardhak Ahar-Viharas like Gramya Anupa Udaka Mamsa, Ati Lavana Sevana, Diwaswapna, Vegdharana, etc. This study can be beneficial in preventing the occurrence and recurrence of lichen planus on adopting Nidana Parivarjanam.

Annular Lichenoid Dermatitis (of Youth).

About 20 years after its first description, Annular Lichenoid Dermatitis of Youth (ALDY) is recognized as a distinctive lichenoid dermatosis with specific clinical and histological features. The disease occurs mostly in young persons all over the world, runs a chronic course, and has an obscure etiopathogenesis. Clinically, lesions consist of persistent, asymptomatic erythematous macules and round-oval annular patches with a red-violaceous non-scaling border and central hypopigmentation, mostly localized on the groin and flanks. Histology shows a peculiar lichenoid dermatitis characterized by irregular epidermal hyperplasia with an alternation of thinned and quadrangular rete ridges and a dense band-like lichenoid infiltrate of lymphocytes in the papillary dermis. Typically, there is infiltration of lymphocytes into the lower epidermal layers with massive necrosis/apoptosis of keratinocytes, which is limited to the tips of rete ridges. Dermal lymphocytes are usually CD3+, CD4+, while most of the intraepidermal T cells are CD8+. Analysis of TCR-γ-chain gene rearrangement displayed polyclonality in all cases examined. Differential diagnosis mainly includes morphea, mycosis fungoides, annular erythemas and inflammatory lesions of vitiligo. Topical corticosteroids and topical tacrolimus represent the most effective drugs for ALDY treatment.

Lupus miliaris disseminatus faciei: A rare case report

Lupus miliaris disseminatus faciei (LMDF), initially thought to be a tuberculid, is an uncommon independent clinicopathological entity characterized by asymptomatic erythematous papules over the face, usually encountered in young individuals and rarely in the extremes of age group. It was initially thought to be a tuberculid due to similar histopathological features between the two entities. LMDF has a spontaneously resolving course; however, due to its ability to cosmetically debilitate an individual, patients often seek a dermatologist's opinion. Various treatment options, such as oral tetracyclines, steroids, and retinoids, have been tried with varying results. Around 200 cases have been reported till date, and further studies are needed to understand the etiopathogenesis and management of this distinct dermatosis. We report a case of LMDF in a young lady who was started on oral minocycline and topical tacrolimus but was lost to follow-up. We hereby report this case for its rare occurrence. The present paper highlights the clinical importance of LMDF mimicking granulomatous facial dermatitis and the need for further laboratory investigations to rule out tuberculosis. This report reviews the available literature and presents a patient with LMDF. Biopsy done on one of the lesions over face showed epidermal atrophy, dermal perivascular and periadnexal inflammatory infiltrate, focal granulomatous inflammation with multinucleated giant cells, with the special stain for acid-fast bacilli being negative. These findings suggested LMDF. LMDF is a distinct granulomatous inflammatory dermatosis not associated with cutaneous tuberculosis.

Juvenile plantar dermatosis: A barrier disease beyond eczema: An open prospective uncontrolled study in a tertiary care hospital of South India

Background: Juvenile plantar dermatosis (JPD), also known as “wet and dry foot syndrome,” is a skin disorder of the feet that commonly affects children from ages 3–14. JPD is frequently seen in children with eczema, but it is not a requirement for diagnosis. Forefoot eczema (FE) is synonymous with JPD is a condition characterized by dry fissured dermatitis of the plantar surface of the feet. Aims: To study the clinical patterns of FE and its associated risk factors. Methods: Twenty-five patients were recorded in the study during the period from April 2013 to March 2014. Fungal scrapings, patch testing, and biopsy for histopathological examination were done wherever required. Results: In our study, the most common site affected was the plantar surface of the toes in 8 (32%) patients. Hand involvement, with fissuring and soreness of the fingertips and palm, was seen in two patients (8%). Four patients (16%) had a personal history of atopy whereas a family history of atopy was present in 3 (12%). Nine patients (36%) had associated risk factors with reported aggravation of itching with plastic, rubber, or leather footwear, and with prolonged contact with water and detergents. Negative fungal scrapings and culture in all patients ruled out a dermatophyte infection. Patch testing with Indian Standard Series was performed in all patients and was positive in three. Conclusions: This study concluded FE as a distinctive dermatosis of the first and second decade, predominantly in males, with a multifactorial etiology and confirming the concept of FE as a barrier disease beyond eczema.

Poikiloderma vasculare atrophicans: a distinct clinical entity?

This paper describes a typical case of poikiloderma vasculare atrophicans (PVA) in a 48-year-old female. Histologically, the features were suggestive of PVA with the absence of Pautrier's microabscess or atypical lymphoid cells. The biopsy specimen was positive for cluster of differentiation (CD) 8 on immunohistochemical staining. Its exact pathogenesis remains obscure, and it remains unclear whether PVA actually is mycosis fungoides (MF), a forme fruste of MF, or a distinct and benign dermatosis with CD8+ phenotype that can perhaps be labeled as PVA. However, it has a long benign clinical course without progression to tumor stage of MF in most cases, and its status within the spectrum of cutaneous T-cell lymphoma remains poorly understood. Yet it is imperative to distinguish PVA from poikilodermic MF.

Perforating collagenosis with cutaneous neurofibroma

Perforating collagenosis is an uncommon distinct dermatosis characterized by transepidermal elimination of altered collagen through the epidermis. It was first described by Mehergan et al in 1967 and till date etiology of this disease is unknown. Herein, we describe a case of acquired perforating collagenosis in a 16 year old male who presented with multiple discrete pruritic and hyperkeratotic umblicated lesions on trunk, upper and lower extrmities and scalp for more than three years. Skin biopsy revealed a typical hyperkeratotic papule with a central plug, surrounding epidermal hyperplasia, inflammatory cells and vertically oriented fibers of collagen. On Masson's trichrome staining, perforating bundles of collagen were seen extending to the surface. A diagnosis of perforating collagenosis was made. The patient during follow up subsequently developed solitary nodule over the anterior chest wall which on histopathological examination revealed well circumscribed spindle shaped neoplasm in the dermis. These cells exibited wavy nuclei in a collagenous stroma along with proliferation of Schwann cells and fibroblasts. In view of these findings, the diagnosis was modified to reactive perforating collagenosis with cutaneous neurofibroma.

Aquagenic palmar wrinkling in two Indian patients with special reference to its dermoscopic pattern Sudip Kumar

Aquagenic palmar wrinkling (APW), synonymously known as aquagenic syringeal acrokeratoderma, transient aquagenic palmar hyperwrinkling, aquagenic palmoplantar keratoderma, or transient reactive papulotranslucent acrokeratoderma, is a distinctive dermatosis characterized by whitish papules, excessive wrinkling, and possible desquamation of the palms and/or soles after immersion into water for a short time[1-5]. We describe herein two cases of aquagenic palmar wrinkling in Indian patients with special reference to its dermoscopic pattern. Since its initial description, only a few cases of APW have been described in literature. To the best of our knowledge, APW is a hitherto unreported condition in Indian population.

Neutrophilic eccrine hidradenitis: A new culprit-carbamazepine

Neutrophilic eccrine hidradenitis (NEH) is a distinctive dermatosis occurring in patients with malignancy or undergoing chemotherapy. This disorder is characterized by a neutrophilic infiltrate around the eccrine glands and secretory coils, and is associated with necrosis. It must be distinguished from infections, drug eruptions, malignancies or other forms of skin diseases. As it is a self-limiting condition, establishing the diagnosis will avoid unnecessary investigations and treatment. Here we report a case of NEH in a 40-year-old woman suspected to be due to carbamazepine.

Immunohistochemical Detection of Parvovirus B19 in “Gloves and Socks” Papular Purpuric Syndrome: Direct Evidence for Viral Endothelial Involvement. Report of Three Cases and Review of the Literature

Papular-purpuric "gloves and socks" syndrome is a distinctive dermatosis featuring acral pruritus, edema, and petechiae. It has been attributed in most--but not all--reported cases to Parvovirus B19 infection, on the grounds of serological proof of recent infection or detection of viral DNA by polymerase chain reaction in patient serum or biopsies. We report the immunohistochemical detection of Parvovirus B19 VP2 structural protein in the endothelial lining of dermal blood vessels in 3 examples of Papular-purpuric "gloves and socks" syndrome and review previously described immunohistochemical investigations in cutaneous involvement by this infection.

Clinical profile of forefoot eczema: A study of 42 cases

Forefoot eczema (FE) is characterized by dry fissured dermatitis of the plantar surface of the feet. To study the clinical profile of FE and the possible etiological factors. Forty-two patients with FE were included in the study. A detailed history was recorded and examination done. Fungal scrapings and patch test with Indian Standard Series (ISS) were performed in all patients. The most common site affected was the plantar surface of the great toe in 16 (38.09%) patients. Hand involvement, with fissuring and soreness of the fingertips and palm, was seen in four patients (9.5%). Seven patients (16.6%) had a personal history of atopy whereas family history of atopy was present in six (14.2%). Seven patients (16.6%) reported aggravation of itching with plastic, rubber or leather footwear, and 13 (30.9%), with detergents and prolonged contact with water. Negative fungal scrapings in all patients ruled out a dermatophyte infection. Patch testing with ISS was performed in 19 patients and was positive in five. FE is a distinctive dermatosis of the second and third decade, predominantly in females, with a multifactorial etiology, possible factors being chronic irritation, atopy, footwear and seasonal influence.

Keratosis lichenoides chronica mimicking verrucous secondary syphilis

Keratosis lichenoides chronica is a rare dermatosis of unknown etiology with a wide variety of cutaneous manifestations. We present a 47-year-old male with a history of progressive and recalcitrant hyperkeratotic and warty plaques, mimicking verrucous secondary syphilis both clinically and microscopically. We review the clinical manifestations, microscopic features, and treatment modalities for this rare and distinctive dermatosis.

ALDY: Annular Lichenoid Dermatitis of Youth

Italian researchers describe what they conclude to be a distinctive inflammatory dermatosis: annular lichenoid dermatitis of youth (ALDY). The authors evaluated the cases of 23 young patients (age range, 5-22 years; median, 10) who presented with annular lesions that were initially suspected to be annular erythema, inflammatory morphea, or patch- or plaque-stage mycosis …

PAPULAR‐PURPURIC GLOVES‐AND‐SOCKS SYNDROME

Papular-purpuric gloves-and-socks syndrome (PPGSS) is a recently described dermatosis in which human parvovirus B19 (HPV B19) has been implicated as etiologic agent; however, it is suspected that PPGSS may be caused by various agents. This study was designed to survey the general characteristics of PPGSS and to determine the role of HPV B19 in its etiology. We analyzed data from 21 patient and examined serum samples from three new cases for various viruses. The PPGSS displays a striking uniform clinical pattern. Histologic and immunofluorescence findings are non-specific. Seroconversion of HPV B19 was reported in six cases and confirmed in two of our patients. In only one case was a possible causative role of Coxsackie virus B6 suggested consistently. The PPGSS represents a distinctive dermatosis and a manifestation of HPV B19 infection. Unlike erythema infectiosum, anti-HPV B19 antibodies seem to develop later after onset of the skin eruption and while viremia is still present.

Eosinophilic Pustular Folliculitis (Ofuji's Disease)

Eosinophilic pustular folliculitis is a distinctive dermatosis that was first described in Japan. Although the histopathologic feature of eosinophilic pustular folliculitis is characterized by follicular infiltrates with numerous eosinophils, its pathophysiology remains unclear. The lesional skin of five patients with eosinophilic pustular folliculitis was examined using several monoclonal antibodies including a variety of anti-leukocyte adhesion molecules: endothelial cell adhesion molecule 1, vascular cell adhesion molecule 1, and intercellular adhesion molecule 1, by means of immunohistochemical techniques. Intercellular adhesion molecule 1 expression by keratinocytes was observed on follicular epithelium, but not on epidermis. The migration of eosinophils and lymphocytes, which were intensely positive for antilymphocyte function-associated antigen 1, was limited to follicular epithelium. Endothelial cell adhesion molecule 1 and vascular cell adhesion molecule 1 expression of vascular endothelium was more often observed around hair follicles. There was no reactivity for interleukin 8 in follicular epithelium. These findings may explain the selective migration of eosinophils and lymphocytes to the hair follicles in eosinophilic pustular folliculitis.

Neutrophilic Eccrine Hidradenitis Heralding the Onset of Acute Myelogenous Leukemia

To the Editor.— Neutrophilic eccrine hidradenitis (NEH) was originally described as a distinctive dermatosis that occurred in patients undergoing chemotherapy for acute myelogenous leukemia (AML). 1,2 Although NEH has since been reported in several other settings, 3-6 most theories regarding its pathogenesis have focused on the role of the implicated therapeutic agents. We report the first case of NEH arising several weeks prior to the onset of fulminant AML. This suggests that NEH may be a paraneoplastic phenomenon, as was proposed in the initial reports on this entity. 1,2 Report of a Case.— A 44-year-old woman had a 2-month history of a nonpruritic rash that she associated with an insect bite reaction. Fever, arthralgias, decreased appetite, and weight loss developed several weeks later. Acetaminophen failed to control her symptoms. A course of methylprednisolone and erythromycin led to resolution of the rash and systemic symptoms, only to recur 1 week later.

Large-cell lymphoma presenting with a distinctive inflammatory dermatosis

Severe, progressively enlarging inflammatory plaques of the upper trunk and neck developed in a 12-year-old girl. Histologic examination revealed a unique inflammatory cell infiltrate that consisted of chronic inflammation in the dermis subtending spongiosis with a diffuse neutrophilic epidermal infiltrate. A tumor in the right axilla was found by computed tomography. Tissue obtained at open biopsy examination demonstrated a large-cell lymphoma of B-cell origin. The tumor and dermatosis responded dramatically to chemotherapy. The distinctive histologic pattern and clinical course suggest that this dermatosis may represent an unusual paraneoplastic presentation of large-cell lymphoma.

Acrokeratosis paraneoplastica of Bazex-a case in the UK

Since the original description of a distinctive dermatosis associated with internal malignancy, acrodermatitis paraneoplastica (APN), by Bazex et al. in 1965,1 there have been numerous reports in the French literature and further cases have been reported from mainland Europe, North America and Australasia, but as far as we are aware, no case has been reported from the United Kingdom.

Pruritic Urticarial Plaques and Papules of Pregnancy and Skin Distention

To the Editor.— Pruritic urticarial plaques and papules of pregnancy (PUPPP) is a distinct dermatosis of pregnancy that has recently been associated with abdominal skin distention.1We describe two cases, a triplet pregnancy and a case of polyhydramnios, that support this association. These are the first reported cases of PUPPP associated with in vitro fertilization. Report of Cases.—Case 1.— A 36-year-old woman (gravida 1, para 0) carrying triplets who were the result of in vitro fertilization developed an intensely pruritic rash over her abdomen during the 35th week of pregnancy. The rash started in the striae of her abdomen and spread to cover her inner arms, ankles, and wrists with urticarial plaques. The rash was treated with topical steroids. She went into labor the day of her dermatology consultation and was delivered of healthy triplets by cesarean section. The infants had no skin lesions. A skin biopsy

Wells' syndrome is a distinctive disease entity and not a histologic diagnosis

Wells' syndrome is a distinctive dermatosis clinically resembling acute cellulitis with solid edema; it resolves spontaneously after weeks or months without residues. Recurrences over many years are common. Light microscopy is characteristic for the disease, with diffuse tissue eosinophilia and marked edema, fibrinoid "flame figures," and palisading microgranuloma. Vasculitis is never found. Eosinophilia of the peripheral blood is a frequent feature. Etiology and pathogenesis are unknown, but the disease has been found to be associated with hematologic disorders in several cases, and recurrences can often be related to infections, arthropod bites, drug administration, or surgery. The diagnosis of Wells' syndrome should be based on the typical clinical picture and the course of the disease with its recurrences and histopathology. Flame figures in histologic sections are an important diagnostic feature but not diagnostic per se for the disease because they represent a reaction pattern that can occur in other conditions. A dilution of Wells' syndrome by making flame figures the central criterion of diagnosis and by lumping all flame figure-positive skin reactions together is therefore unjustified.

Acrokeratosis Paraneoplastica (Bazex' Syndrome)

• Acrokeratosis paraneoplastica (Bazex' syndrome) is a rare but clinically distinctive dermatosis that has been associated in all reported cases, to our knowledge, with either a primary malignant neoplasm of the upper aerodigestive tract or metastatic cancer to the lymph nodes of the neck. Acrokeratosis paraneoplastica was found in a 53-year-old black man with squamous cell carcinoma of the tonsil. A distinctive series of changes was found on histopathologic examination of biopsy specimens taken from his skin lesions, and direct immunofluorescence microscopy of both lesional and nonlesional skin specimens showed immunoglobulin and complement deposition on the epidermal basement membrane. The skin lesions largely resolved following radiation therapy of the neoplasm and of the presumably involved lymph nodes. (<i>Arch Dermatol</i>1983;119:820-826)