Macular hypopigmentation, hair loss and follicular spongiosis: A distinct clinicopathological entity.

BACKGROUND: Hypopigmented macules are seen in a variety of disorders and the diagnosis rests on clinicopathological correlation. However, some cases are difficult to classify and pose a diagnostic challenge. AIM: To describe the clinical and histopathological features of patients with hypopigmented macules and follicular spongiosis on histopathology. MATERIALS AND METHODS: We undertook a retrospective analysis of clinical and histopathological findings in 12 patients who presented with clinically nondiagnostic hypopigmented macules and showed follicular spongiosis on skin biopsy, at All India Institute of Medical Sciences, New Delhi, India between January 2015 and October 2016. The findings were compared with 12 patients with "unclassified" hypopigmented macules, who did not show follicular spongiosis on skin biopsy. RESULTS: A total of 12 patients with hypopigmented macules showed spongiosis affecting the follicular epithelium on histopathology. There were eight men and four women, most in their second decade (mean age 19.1 ± 8.05 years), presenting with hypopigmented macules most commonly on the upper limbs, for a mean duration of 6.33 ± 5.10 months. Clinically evident lesional hair loss was seen in all patients, and follicular prominences in seven (58%) patients. Histological features suggestive of other diagnosis, namely leprosy, mycosis fungoides or sarcoidosis were not seen in any biopsy. Alcian blue stain revealed an minimal amount of mucin in one biopsy. Clinically apparent hair loss and follicular prominences were found to be statistically significantly associated with histological evidence of follicular spongiosis (P < 0.001 and 0.003, respectively). LIMITATIONS: Our study is limited by its retrospective design and small sample size. CONCLUSIONS: Patients with hypopigmented macules and follicular spongiosis on histopathology may represent a distinct clinicopathological entity that is associated with lesional hair loss and follicular prominences. It is probably a variant of an endogenous dermatitis similar to pityriasis alba.

Tumor of follicular infundibulum: an unsuspected cause of macular hypopigmentation.

We present three cases of a rare eruptive variant of tumor of follicular infundibulum. Two patients presented with hypopigmented macules. The clinical differential diagnoses considered in these two cases were vitiligo, lichen sclerosus et atrophicus, and idiopathic guttate hypomelanosis. In the third case, the lesions were hypopigmented flat topped maculo-papules diagnosed clinically as verruca plana. In all three cases, the histopathological features of plate like growth of pale keratinocytes connected to the epidermis and peritumoral condensation of elastic fibers were diagnostic. Although no satisfactory treatment is available, the exclusion of other clinical differential diagnosis particularly vitiligo with its psychosocial implications underscores the importance of skin biopsy.

Clinicopathological correlation of acquired hypopigmentary disorders.

Acquired hypopigmentary disorders comprise a significant group of disorders that affect Indians and Asians. The pigment disturbance in darker skin individuals can be very distressing to the patient and the family. These disorders cover a wide array of pathologies including infections, autoimmune processes, lymphoproliferative disorders, and sclerosing diseases. Histological diagnosis is particularly important because treatments for these diseases are varied and specific. This review will focus on histopathological diagnosis based on clinicopathological correlation for commonly encountered disorders such as leprosy, vitiligo, lichen sclerosus, pityriasis alba (PA), and pityriasis versicolor (PV). Atypical or uncommon clinical presentation of classic diseases such as hypopigmented mycosis fungoides (HMF) and hypopigmented sarcoidosis are also included.

Familial progressive hypo- and hyperpigmentation: a variant case.

Familial progressive hyper- and hypopigmentation (FPHH) is characterized by diffuse hyperpigmentation with variable intensity. Cafe'-au-lait macules and larger hypopigmented ash-leaf macules are also present. Herein, we reported a variant case of FPHH. The patient was a two-year-old Chinese girl showing diffuse hyper- and hypopigmented lesions, longitudinal melanonychia in both thumbs, and infantile seizures, without any lentigines.

Clinicopathologic profile of hypopigmented mycosis fungoides in India.

BACKGROUND: Hypopigmented mycosis fungoides (HMF) is an under recognized disease in India, which is often mistaken for Hansen disease or vitiligo, resulting in delayed diagnosis and treatment. AIM: To describe the clinical, histopathologic and immunohistochemical features of HMF in Indian patients. MATERIALS AND METHODS: All cases presenting as hypopigmented lesions that were signed out as MF between 2001 and 2009 (15 cases) were included. Clinical data and histopathology slides were reviewed. Immunostains for CD4, CD8, and CD1a were done, where tissue was available. RESULTS: The age ranged from 14 to 38 years with a male preponderance. The commonest presentation was multiple hypopigmented patches on limbs and trunk with the duration of the lesions varying from 4 months to 14 years. All cases showed a psoriasiform/lichenoid epidermal pattern, disproportionate epidermotropism, basilar tagging of lymphocytes, monomorphous lymphocytes, haloed lymphocytes, and wiry dermal collagen. Other important findings were infiltration of hair follicles, larger epidermal lymphocytes, atypia of dermal lymphocytes, and stuffed dermal papillae. Dermal edema was absent in all cases. Immunohistochemistry done on 10 cases showed a CD8 phenotype in 6 cases and CD4 phenotype in the remaining 4 cases. CONCLUSIONS: Histopathology supplemented by immunohistochemistry is reliable in making a diagnosis of HMF. It is important to be aware of this uncommon, yet significant disease.

A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is cutaneous lymphoma of the T-cell lineage. Hypopigmented MF is a clinical variant of MF, described mainly in Asians. This is a retrospective clinicopathologic analysis of hypopigmented MF at a tertiary care center. AIMS: To describe the clinicopathologic profile of hypopigmented MF. METHODS: Records of clinicopathologic notes over a 5-year period ranging from January 2005 up to December 2009 were reviewed over a period of 3 months, of which 15 cases were diagnosed with hypopigmented MF based on clinicopathologic correlation. RESULTS: Hypopigmented MF was found to be more common in males, and between second and fourth decades of life. The latent period between onset and diagnosis was around 3.83 years. Most of the patients were asymptomatic 80% (12/15), with skin changes of subtle atrophy in 46.66% (7/15), scaling in 20% (3/15) and focal changes of poikiloderma in 26.66% (4/15) patients. Most common sites of distribution of the lesions were the trunk and extremities. Many of the cases had been clinically mistaken for Hansen's disease prior to correct diagnosis. Marked epidermotropism and tagging of epidermis by large lymphocytes characterizes the condition histopathologically. Of the 15 cases, immunohistochemistry was possible in 10 cases, of which 8 showed predominant CD8 positive epidermotropic infiltrates and two cases showed absence of CD8 positive and CD4 positive lymphocytic infiltrate in the epidermis. CONCLUSION: Hypopigmented MF presents as hypopigmented asymptomatic patches without any erythema or infiltration in its early stage and mimics Hansen's disease. Skin biopsy clinches the diagnosis.

Adult onset, hypopigmented solitary mastocytoma: report of two cases.

Solitary mastocytoma is known to occur predominantly in children below 2 years of age and onset in adulthood is rare. Lesions are hyperpigmented in the majority of cases owing to the stimulation of melanin synthesis by mast cell growth factor. We hereby report two patients with adult onset solitary mastocytoma presenting as hypopigmented plaque. The first case was a 24-year-old man who presented with a plaque on the back of the neck of 5 years duration. The second case was a 30-year-old man who had a well-defined solitary, oval 3 x 2.5 cm plaque on the nape of the neck. Stroking of lesion resulted in a wheal with flare (Darier's sign) in both cases. Systemic examination was within normal limits in both cases. Histopathology revealed a dense toluidine blue-positive infiltrate of mast cells in the upper dermis in both cases.

Clinical manifestations, diagnosis and classification of leprosy.

Mycobacterium leprae, the causative organism of leprosy is slow-growing and the reason is its long incubation period of 2-4 years. Males are predominantly affected and deformity is produced in less than 2% of people affected with the disease. The disease manifests in the skin as macules, papules, nodules, plaques or infiltration. Hypopigmented or erythematous skin patches with definite sensory deficit is one of the clinical cardinal signs by which one can make a definite diagnosis. Demonstration of bacilli in the slit skin smear is the bacteriological cardinal sign used to make definite diagnosis of leprosy. Involvement of common cutaneous nerves with thickening and/or tenderness with its dysfunction is the second clinical cardinal sign used to diagnose leprosy. Diagnosis can be made by eliciting definite sensory deficit in the skin lesions (other than nodules and infiltration). In the absence of two clinical cardinal signs and when there is a strong suspicion of leprosy, slit skin smear should be taken from both ear lobes and one of the lesions for demonstration of acid-test bacilli. Clinical classification is based on characteristics like number of lesions, their margin, sensory deficit, satellite lesions, symmetry of lesions, central healing and scaling. Up to 5 lesions are grouped under paucibacillary and six and more are grouped under multibacillary leprosy.

Poor correlation of systemic immunological parameters with clinical features in macular leprosy.

On the basis of clinical features and bacteriological status, macular skin lesions of nine cases of leprosy were classified as falling within a spectrum between the tuberculoid at one end and the lepromatous at the other. While histologic correlation was seen in 60% of cases, humoral and cellular systemic immunologic features were found to be uncharacteristic. It is suggested that macular lesions form an early stage in the development of leprosy where the systemic immunological response is yet to set in fully.

A clinical and histopathological study of macular type of post-kala-azar dermal leishmaniasis.

Post-kala-azar dermal leishmaniasis (PKDL) is an uncommon sequel seen in patients with a previous attack of kala-azar (KA). It is characterized by hypopigmented macules and erythematous eruptions leading to the formation of papules, plaques and nodules. Little attention has been paid to the rare group of patients who present with only hypopigmented macules. The present study has described the distribution of lesions in macular PKDL and their histopathology.

[Creole dyschromia or idiopathic macular hypomelanosis of the melanodermic halfcast of Guillet-Hélénon]. / Dyschromie créole ou hypomélanose maculeuse idiopathique du métis mélanoderme de Guillet-Hélénon.

Melanodermic halfcasts may develop an original cutaneous dyschromia known as "progressive and extensive hypomelanosis" (Guillet-Helenon 1988). This disease is characterized by hypochromic and coalescent macules on the back and abdomen with possible spontaneous improvement within five years, favoured by UV exposure. The disease is not restricted to a limited geographic group: eight observations were collected in melanodermic patients leaving in temperate area. The pathogenesis of the disorder involves a variation in melanosome size and distribution with decrease in production of type IV melanosomes featuring a change of ultrastructural phenotype of melanogenesis. Since it may be misdiagnosed as fungal disease, leprosy or achromic eczema leading to useless laboratory examinations, this specific and frequent disease deserves to be known and recognized.