Hypopigmented Patches Research Articles

Hypopigmented Patches in a Caucasian Male: A Quiz

Hypopigmented Patches in a Caucasian Male: A Quiz

Chemical Leukoderma Improved by Low-dose Steroid Pulse Therapy

Chemical leukoderma occurs due to the toxic effect of a specific chemical preceding allergic contact dermatitis. The mechanism is either destruction or inhibition of melanocytes by the offending substance. Clinicohistopathologically, no absolute criteria can differentiate chemical leukoderma from vitiligo. However, chemical leukoderma can be diagnosed clinically by a history of repeated exposure to a known or suspected depigmenting agent at the primary site. There is no agreed treatment guideline for chemical leukoderma. We report a healthy 51-year-old man who had multiple hypopigmented macules and patches on his face, neck, arms and legs after exposure to occupationally related chemicals. The lesions were recalcitrant to topical corticosteroids, but they showed much improvement after 3 cycles of systemic steroid pulse therapy. We suggest this therapy may be a good treatment option for chemical leukoderma.

Mycosis fungoides and CD30+ cutaneous T-cell lymphoma simulating pyoderma gangrenosum in a patient with ulcerative colitis

Pyoderma gangrenosum is estimated to occur in 5% to 12% of ulcerative colitis patients. Primary CD30+ cutaneous large cell lymphoma is the second most common cutaneous lymphoma. It may coexists with mycosis fungoides. We report a 38-years-old female patient with 12 year history of ulcerative colitis, treated previously with 5-aminosalicylic acid and systemic corticosteroids. The disease has been in remission for the past 1.5 years, with no maintenance treatment. The patient then developed a rapidly progressing ulcerated lesion clinically simulating pyoderma gangrenosum. Physical examination revealed also a small number of hairless hypopigmented patches on the upper and lower limbs which she claimed to have appeared 4 years ago. Surprisingly a histological evaluation of the ulcer revealed a CD30+ primary cutaneous large cell lymphoma, while histology of hypopigmented lesions revealed mycosis fungoides, patch stage. T-cell receptor gene rearrangement from the two lesions didn't reveal the same T Cell clonalitiy and the patients lymphoma was stable. Our case presents the rare coexistence of primary mycosis fungoides and primary CD30+ cutaneous large cell lymphoma, rather than the CD30+ cutaneous large cell lymphoma developing from mycosis fungoides. This case also presents the development of a pyoderma gangrenosum-like lesion of CD30+ cutaneous large cell lymphoma in a patient with ulcerative colitis. An observation that emphasizes the need for a high index of suspicion in cases diagnosed as pyoderma gangrenosum based solely on clinical appearance.

Two patients with leprosy and the sudden appearance of inflammation in the skin and new sensory loss.

Leprosy is a chronic infection caused by Mycobacterium leprae that affects the peripheral nerves, skin, and potentially other organs [1]–[5]. Although the worldwide prevalence of leprosy has decreased in the era of multi-drug therapy (MDT), the global detection of new cases of leprosy remains a concern, with more than 250,000 new cases of leprosy reported in 2007 [3]. The precise mechanism of transmission of leprosy has not been conclusively defined; however, it is likely that this occurs through respiratory secretions by untreated borderline lepromatous and polar lepromatous cases [1],[5]. Target cells of infection are macrophages, histiocytes in the skin, and the nonmyelinating and myelinating Schwann cells in the peripheral nerve, leading to axonal dysfunction and demyelination [6]. Nerve injury plays a central role in the pathogenesis of leprosy, leading to functional impairment and deformity of hands and feet and the eyes [1],[6]. Leprosy is diagnosed by definite loss of sensation in a hypopigmented or reddish skin patch, a thickened peripheral nerve with loss of sensation and muscle weakness in the affected nerve, and presence of acid-fast bacilli on skin smear or biopsy [1],[4]. The immunological response to M. leprae mounted by the host will determine the different potential clinical states. The Ridley-Joplin system uses clinical and histopathological features and the bacteriologic index and includes the polar categories (lepromatous [LL] and tuberculoid [TT]) and the borderline states (borderline tuberculoid [BT], borderline borderline [BB], and borderline lepromatous [BL]) [1] (Table 1). In the polar tuberculoid category, a Th1 type cell–mediated immune response with a low bacterial load is seen. Lepromatous states are characterized by low cell-mediated immunity and a higher bacterial load [5] (Table 1). Clinically, patients with tuberculoid leprosy have a single or very few hypopigmented macules or plaques with a raised edge; they are dry, scaly, hairless, and have reduced sensation; and only a few peripheral nerves are commonly enlarged [1]. Lepromatous leprosy is characterized by widely and symmetrically distributed skin macules, nodules, erythematous papules, and diffuse skin infiltration; thickened peripheral nerves are more frequently identified. Borderline states represent a mixture of signs and symptoms of the polar categories [1]. Table 1 World Health Organization System and Ridley-Joplin Classification and Type of Reaction.

Superficial morphea: 20‐year follow up in a patient with concomitant psoriasis vulgaris

Superficial morphea, a newly described variant of morphea, manifests clinically with hypopigmented or hyperpigmented patches that lack induration. The lesions show on biopsy dermal sclerosis in the superficial to mid-reticular dermis. So far, all cases reported in the literature were skin limited. In this report, we describe a 24-year-old female patient who developed at the age of 4 years plaques characteristic, both clinically and histologically, of superficial morphea. The patient developed later on psoriasis vulgaris. Although the association of scleroderma and psoriasis is rarely reported in the literature, most reports describe a progressive systemic course of scleroderma whenever the two diseases co-exist. This is the first report to describe superficial morphea in association with psoriasis and to provide a 20-year follow-up period during which the superficial morphea remained relatively stable with no evidence of systemic involvement.

Juvenile-Onset Hypopigmented Mycosis Fungoides Mimicking Vitiligo

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma and typically affects older adults. It is estimated that less than 5% of MF cases are of juvenile onset. An uncommon hypopigmented variant of MF exists, which has been more commonly observed in dark-skinned individuals and predominantly in juvenile-onset cases. We describe an 8-year-old otherwise healthy Hispanic male who, by 6 months of age, had developed asymptomatic hypopigmented patches on the lower legs, thighs, and buttocks, which have evolved over the past 7 years. This condition had previously been misdiagnosed as vitiligo. Recent immunohistologic and molecular biology studies are consistent with MF. Given that hypopigmented MF is an uncommon condition, it may not be clinically suspected in the pediatric population. Histopathologic, immunophenotypic, and/or molecular biologic studies are sometimes equivocal, with findings similar to inflammatory dermatoses or autoimmune vitiligo, which may initially lead to a misdiagnosis, as in this patient's case.

Variations in Leprosy Manifestations among HIV-Positive Patients, Manaus, Brazil

Variations in Leprosy Manifestations among HIV-Positive Patients, Manaus, Brazil

Pallister–Killian syndrome in a girl with mild developmental delay and mosaicism for hexasomy 12p

We report on a 5-year-old girl with Pallister-Killian syndrome (OMIM #601803) due to mosaicism of two supernumerary isochromosomes (hexasomy 12p). Hexasomy 12p was found in 20% of the cells by chromosome analysis of cultured skin fibroblasts and confirmed by FISH- and arrayCGH analysis. The girl has woolly and sparse hair, absence of lateral eyebrows, dry skin, brittle nails, hypopigmented patches, frontal bossing, hearing loss, hypertrophic dilated cardiomyopathy, polydactyly, and mild developmental delay. This is the second live case of mosaicism for hexasomy 12p to be reported. A gene-dosage hypothesis has previously suggested that cases with hexasomy 12p would have a worse phenotype than cases with tetrasomy 12p. The relatively mild symptoms found in the current girl with hexasomy 12p may to contradict this hypothesis.

Infantile Pityriasis Alba and Comorbid Disorders

Pityriasis alba is a common cutaneous disorder characterized by asymptomatic hypopigmented patches on the face, neck, trunk and proximal extremities of children and young adults. Its pathogenesis has not been definitely clarified. Pityriasis alba is considered to be a low-grade eczematous dermatitis and is regarded as a minor feature of atopic dermatitis. Possible comorbid disorders described in association with pityriasis alba are nutritional deficiency, anemia and parasitic infestations; however, these are exclusively observed in children of poor socioeconomic conditions. In the past, a pathogenic implication of several microorganisms, in particular Gram-positive bacteria, has repeatedly been proposed but never confirmed. Pityriasis alba has no reliable treatment, but it usually resolves with time. Emollients, mild topical steroids and pimecrolimus may aid to accelerate the repigmentation.

A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy

Syringotropic cutaneous T-cell lymphoma (SCTCL) is a variant of mycosis fungoides that is characterized by the presence of lymphocytic infiltration of eccrine glandular structures and the absence of the classic features of MF, such as epidermotropism and Pautrier's microabscesses. We report a patient with SCTCL who presented with multiple hypopigmented patches and localized alopecia who was treated with local radiotherapy with excellent local control. In our experience, local electron radiation offers local control in early stage SCTCL, and it may be possible to reserve systemic therapy for more advanced stages of the disease.

Eccrine Nevus Presenting as a Hypopigmented Patch

A 14-month-old Caucasian male presented with a hypopigmented patch associated with increased perspiration on the right side of his back, flank, and abdomen. Starch iodide test and biopsy revealed an eccrine nevus. The truncal presentation and light color both were unique presentations of this rare hamartoma.

Vitiligo-like Primary Melanoma

Depigmented or hypopigmented macules and patches have been described in association to primary and metastatic melanoma. Primary melanoma presenting at the onset as a vitiliginous patch without histopathological evidence of regression seems to be a rare event. We report 2 patients with melanoma in situ that expressed clinically as vitiligo-like patches and review the possible immunologic mechanisms responsible for this unusual presentation.

Lipodystrophia centrifugalis abdominalis infantilis in a Caucasian girl: report of a case with perieccrine inflammation

Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare, self-limiting disease typically affecting Asian children around the age of three. In classic cases, patients present with a hypopigmented patch with central atrophy and an erythematous border in the groin area. We present a case of LCAI affecting a female Caucasian toddler that shows striking perieccrine inflammation, a finding not previously reported in this entity. LCAI has been rarely reported in Caucasian children; as a result, there is sparse literature on the histopathological findings in this subset. This case illustrates how significant deep dermal and perieccrine inflammation can be seen in this condition, thus broadening the histopathological spectrum of this disorder.

Case 2: Seizures as a clue to…

A four-month-old girl with a history of right leg hemihypertrophy and hypopigmented skin patches presented to the emergency room with irritability and a four-day history of jerky movements. These were described as symmetrical flexion of both upper extremities, occurring in clusters lasting 5 min to 10 min. She was afebrile with stable vital signs and no obvious dysmorphic features. Her weight was 6.7 kg (75th percentile). Several white spots were noted on her right cheek and both legs. She had right thigh hemihypertrophy. There was mildly decreased central tone with head lag and normal peripheral tone. There were no abdominal masses. She was born to a 33-year-old G3P1A1 mother at 40 weeks’ gestation by vaginal delivery. Her birth weight was 3.46 kg (between the 50th and 75th percentiles). The pregnancy was uncomplicated and her prenatal ultrasound was normal. On the first day of life, she was noted to have right leg hemihypertrophy and was seen by the genetics department. Her family history was negative for overgrowth syndromes. Her mother had one hypopigmented skin lesion on her left leg. There was no consanguinity. Her hemoglobin, blood gas and glucose levels were normal. Her calcium level was mildly elevated at 2.95 mmol/L (normal 2.17 mmol/L to 2.44 mmol/L). The remainder of her electrolyte levels, including phosphate and magnesium, were normal. Further investigations revealed her diagnosis.

Glomerulocystic kidney disease in hypomelanosis of Ito

Hypomelanosis of Ito (incontinentia pigmenti type I) was first described as a disorder characterized by unusual unilateral or bilateral cutaneous macular hypopigmented whorls, streaks and patches. Subsequently, neurologic, skeletal and ocular involvement were described. Kidney involvement has been reported only exceptionally. Here, we describe the case of a male infant with hypomelanosis of Ito with a prenatal diagnosis of bilateral enlargement of the kidneys, decreased corticomedullary differentiation and cysts located in the cortical and subcapsular regions. These findings were confirmed postnatally. The skin examination showed hypopigmented linear and round diffuse lesions located on the right leg and the arms. Ophthalmological examination showed anterior capsular and posterior subcapsular cataract of the left eye. Renal biopsy was characteristic of glomerulocystic kidney disease, whereas the skin biopsy confirmed the clinical diagnosis of hypomelanosis of Ito. Four other cases of kidney disease in hypomelanosis of Ito have been reported, including two cases characterized by cystic renal changes, indicating that gene abnormalities that cause hypomelanosis of Ito may also impair normal renal development, causing renal cystic changes.

Lentigines within Nevus Depigmentosus: A Rare Collateral Effect of UVB Therapy?

Nevus depigmentosus is an uncommon hypopigmented macule or patch that is congenital and stable in its relative size and distribution throughout life; it occurs sporadically and may be localized, segmental or, less often, systematized. We report the case of a 17-year-old girl with a segmental achromic nevus of the left leg and a patch on the lower back with late age of onset who developed lentigines after prolonged intense ultraviolet B exposure as a consequence of an incorrect diagnosis of segmental vitiligo.

Linear Morphea Presenting as Acquired Unilateral Edema

We describe a 2-year-old African-American boy with a 4-month history of gradually worsening unilateral edema that was initially noted on his left hand and then approximately 2 weeks later on his left lower extremity. In addition, linear hypopigmented patches were noted along the left forearm and leg, with no appreciable scarring or induration. The edema on the left-hand side of his body progressed so that he developed tense bullae on his left hand. Two months later, the hypopigmented patches were indurated and bound-down, especially over the left groin and thigh. A biopsy specimen from this area showed features characteristic of morphea. In this patient, dilated lymphatic channels secondary to the sclerosis of the morphea caused the bullae. Bullous morphea is a rare condition. We were unable to find any reports its occurrence in children under 18 with associated lymphedema. This entity should be included in the differential for acquired unilateral edema in children.

A bullous dermatosis in an infant

Conflict of interest: there are no conflicts of interest. A 7‐month‐old Indian girl was referred by her general practitioner with a 4‐month history of blistering lesions on her trunk and scalp. These were intensely itchy, keeping the infant awake at night. Resolution occurred with healing of superficial erosions leaving postinflammatory hypopigmented patches, which repigmented slowly. The infant also exhibited frequent facial flushing. Examination of the skin revealed numerous intact and ruptured vesicles and bullae arising on an urticated base on the trunk, on a background of Fitzpatrick type V skin (Fig. 1). There was also extensive involvement of the scalp with weeping vesicles and associated crusting. Darier's sign was negative. The skin was not thickened or lichenified at any site, and had no pachydermia or skin folding. ... Skin biopsy from an affected area of the lower back revealed a moderately dense perivascular and interstitial infiltrate of mast cells filling the papillary dermis and marked oedema of the dermal papillae (Fig 2). There were scattered eosinophils and a few neutrophils. The overlying epidermis showed mild elongation of the rete ridges, basal pigmentation and serum in the stratum corneum.

Multiple sclerosis associated with systemic sclerosis

Multiple sclerosis (MS) has been increasingly reported in association with other autoimmune diseases not primary affected the nervous system. The coexistence of MS and systemic sclerosis (SSc) has been rarely described. We report here the case of a 46-year-old female patient with longstanding MS since the age of 26, who developed SSc 12 years later. Her MS was of the relapsing-remitting type, but the definite diagnosis was not made until the age of 33. MS diagnosis was based on medical history, magnetic resonance imaging (MRI) studies and positive cerebrospinal fluid analysis. One year after the diagnosis of MS, she developed Raynaud's phenomenon, skin tightness and hypopigmented patches, suggestive of scleroderma. Further investigation with laboratory studies, including serology, hand and chest X-rays, and chest computerized tomography scan confirmed the SSc diagnosis. Our report highlights the interesting association between MS and SSc that may be due to an overlapping pathogenetic mechanism for both processes.

Mycosis Fungoides Presenting as Pigmented Purpuric Dermatitis

Mycosis fungoides, a cutaneous T-cell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling and over time can evolve into tumor stage with tumor nodules. Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. We report Mycosis fungoides in a 14-year-old boy presenting as pigmented purpuric dermatitis and review the relevant literature. This is a rare presentation of a condition that is uncommon in the pediatric population. In our patient, histologic features were typical of Mycosis fungoides presenting as pigmented purpuric dermatitis. The clinical features, pathology, molecular biology, and the relationship between these two entities are discussed.